Materiały źródłowe

• #1 Desmoplastic Small Round Cell Tumor: A Review of Main Molecular Abnormalities and Emerging Therapy

  https://www.mdpi.com/2072-6694/13/3/498

  Desmoplastic small round cell tumor (DSRCT) is an extremely rare, aggressive sarcoma affecting adolescents and young adults with male predominance. Generally, it originates from the serosal surface of the abdominal cavity. The hallmark characteristic of DSRCT is the EWSR1–WT1 gene fusion. This translocation up-regulates the expression of PDGFRα, VEGF and other proteins related to tumor and vascular cell proliferation. Current management of DSRCT includes a combination of chemotherapy, radiation and aggressive cytoreductive surgery plus intra-peritoneal hyperthermic chemotherapy (HIPEC). Despite advances in multimodal therapy, outcomes remain poor since the majority of patients present disease recurrence and die within three years. The dismal survival makes DSRCT an orphan disease with an urgent need for new drugs.

• #2 Desmoplastic Small Round Cell Tumor : EMA

  https://www.webpathology.com/images/soft-tissue/uncertain-histogenesis/desmoplastic-small-round-cell-tumor/45143

  Desmoplastic small round cell tumors (DSRCT) are characterized by co-expression of epithelial, mesenchymal, and neural markers. Desmoplastic small round cell tumor (DSRCT) is a rare highly aggressive tumor of adolescent and young adults with a strong male predominance. It usually involves abdominal or pelvic peritoneum. DSRCT shows chromosomal translocation t(11:22) (p13; q12) involving the EWSR1 gene (22q12) and WT1 gene (11p13). The prognosis is dismal with a 5-yr survival rate of less than 15%.

• #3 Incidence and Outcomes of Desmoplastic Small Round Cell Tumor: Results from the Surveillance, Epidemiology, and End Results Database

  https://pmc.ncbi.nlm.nih.gov/articles/PMC4238280/

  Desmoplastic small round cell tumor (DSRCT) is a rare but highly fatal malignancy. […] Due to the rarity of this neoplasm, no large population based studies exist. […] A total of 192 cases of DSRCT were identified. […] Peak incidence age was between 20 and 24 years. […] Age-adjusted incidence rate for blacks was 0.5 cases/million and for whites was 0.2 cases/million (P = 0.037). […] DSRCT is more common in males and in people of African-American descent. […] The age-adjusted incidence rate of DSRCT was 0.3 cases/million, with a peak incidence of 0.74 cases/million in persons 20-24 years of age. […] Age-adjusted incidence rates for males and females were 0.4 and 0.1 cases/million, respectively (P 0.001). […] Age-adjusted incidence rates for Caucasians, African-Americans, American Indians, and Asian Pacific Islanders were 0.2, 0.5, 0.3, and 0.1 cases/million, respectively, with a statistically significant difference between Caucasians and African-Americans (P = 0.037).

• #4 Desmoplastic Small Round Cell Tumor: A Review of Main Molecular Abnormalities and Emerging Therapy

  https://www.mdpi.com/2072-6694/13/3/498

  In a study published in 2014, a total of 192 cases of DSRCT were identified in the SEER database between 1973 to 2007. The age-adjusted incidence rate based on this analysis was 0.3 cases/million, with a peak incidence of 0.74 in individuals 20–24 years-old. There is a predominance of DSRCT in males. The age-adjusted incidence rates for males and females were 0.4 and 0.1 cases/million, respectively (p < 0.001). There is predominance in African–American individuals and it is more common in males. The age-adjusted incidence rates are higher among African-Americans as compared to Caucasians (0.5 × 0.2, p = 0.037, respectively). Out of 192 cases, the common primary sites of disease were the peritoneum or soft tissue of abdomen and pelvis (42%) and less common primary sites included the ovary/fallopian tube (6 cases), orbit (1 case), cerebellum (1 case), and cerebral ventricle (1 case).

• #5 Incidence and Outcomes of Desmoplastic Small Round Cell Tumor: Results from the Surveillance, Epidemiology, and End Results Database

  https://pmc.ncbi.nlm.nih.gov/articles/PMC4238280/

  Desmoplastic small round cell tumor (DSRCT) is a rare but highly fatal malignancy. […] Due to the rarity of this neoplasm, no large population based studies exist. […] A total of 192 cases of DSRCT were identified. […] Peak incidence age was between 20 and 24 years. […] Age-adjusted incidence rate for blacks was 0.5 cases/million and for whites was 0.2 cases/million (P = 0.037). […] DSRCT is more common in males and in people of African-American descent. […] The age-adjusted incidence rate of DSRCT was 0.3 cases/million, with a peak incidence of 0.74 cases/million in persons 20-24 years of age. […] Age-adjusted incidence rates for males and females were 0.4 and 0.1 cases/million, respectively (P 0.001). […] Age-adjusted incidence rates for Caucasians, African-Americans, American Indians, and Asian Pacific Islanders were 0.2, 0.5, 0.3, and 0.1 cases/million, respectively, with a statistically significant difference between Caucasians and African-Americans (P = 0.037).

• #6 Incidence and outcomes of desmoplastic small round cell tumor: results from the surveillance, epidemiology, and end results database – PubMed

  https://pubmed.ncbi.nlm.nih.gov/25431592/

  Desmoplastic small round cell tumor (DSRCT) is a rare but highly fatal malignancy. Due to the rarity of this neoplasm, no large population based studies exist. A total of 192 cases of DSRCT were identified. Peak incidence age was between 20 and 24 years. Age-adjusted incidence rate for blacks was 0.5 cases/million and for whites was 0.2 cases/million (P = 0.037). DSRCT is more common in males and in people of African-American descent. Although overall survival remains poor, radiation therapy following surgery seems to improve outcome in these patients. […] There was no statistically significant difference in survival based on gender or ethnicity. When adjusted for age, there was no statistically significant difference in survival amongst patients who received radiation therapy compared to those who did not (HRadj = 0.73; 95% CI 0.49, 1.11). There was a statistically significant survival advantage for patients who received radiation after surgery compared to those who did not (HR 0.49; 95% CI 0.30, 0.79).

• #7 Desmoplastic small round cell tumor of the liver: diagnosing a rare case on liver biopsy | Diagnostic Pathology | Full Text

  https://diagnosticpathology.biomedcentral.com/articles/10.1186/s13000-023-01373-1

  Desmoplastic small round cell tumors (DSRCT) are rare and highly invasive malignant soft tissue tumors that mainly occur in children and young people, with an incidence of 0.0002-0.0005. […] Primary DSRCT of the liver is extremely rare, with fewer than 10 reported cases. […] DSRCT is a rare neoplasm that accounts for less than 1% of soft tissue sarcomas with extremely aggressive behavior. […] The age of onset of DSRCT is 3-52 years old, about 80% of the patients are 20-30 years old, and the ratio of male to female is 3:5:1. […] DSRCT is mainly located in the serosa of the abdominal cavity and pelvis, and usually metastasizes to the surrounding lymph nodes, liver, lung, and so on. […] Currently, there is a lack of effective treatments for patients with DSRCT. […] But the prognosis of DSRCT is poor and the survival time is short, with an overall median survival of 2 years and a 5-year survival rate approaching 15%. […] In summary, primary DSRCT of the liver are extremely rare and can be challenging for core biopsy specimens.

• #8 Desmoplastic Small Round-cell Tumor: Retrospective Review of Institutional Data and Literature Review | Anticancer Research

  https://ar.iiarjournals.org/content/41/8/3859

  Desmoplastic small round-cell tumor (DSCRT) in adults is an extremely rare (age-adjusted incidence 0.3 per million) and aggressive sarcoma with limited data for optimal management. […] Desmoplastic small round-cell tumor (DSCRT) in adults is a rare sarcoma predominantly affecting young adults, with an age-adjusted incidence of 0.3 per million. […] While aggressive multimodality treatment is always warranted for DSCRT, the options are limited by the multicentric presentation, short-lived initial response and lack of established subsequent therapy portending a poor prognosis. […] As a result, 60-70% of patients succumb to DSCRT within 2-3 years despite best care. […] The findings of our study are consistent with historical data namely that the disease predominantly affects young adults with a male predilection.

• #9 Desmoplastic small round cell tumor of the liver: diagnosing a rare case on liver biopsy | Diagnostic Pathology | Full Text

  https://diagnosticpathology.biomedcentral.com/articles/10.1186/s13000-023-01373-1

  Desmoplastic small round cell tumors (DSRCT) are rare and highly invasive malignant soft tissue tumors that mainly occur in children and young people, with an incidence of 0.0002-0.0005. […] Primary DSRCT of the liver is extremely rare, with fewer than 10 reported cases. […] DSRCT is a rare neoplasm that accounts for less than 1% of soft tissue sarcomas with extremely aggressive behavior. […] The age of onset of DSRCT is 3-52 years old, about 80% of the patients are 20-30 years old, and the ratio of male to female is 3:5:1. […] DSRCT is mainly located in the serosa of the abdominal cavity and pelvis, and usually metastasizes to the surrounding lymph nodes, liver, lung, and so on. […] Currently, there is a lack of effective treatments for patients with DSRCT. […] But the prognosis of DSRCT is poor and the survival time is short, with an overall median survival of 2 years and a 5-year survival rate approaching 15%. […] In summary, primary DSRCT of the liver are extremely rare and can be challenging for core biopsy specimens.

• #10 Incidence and Outcomes of Desmoplastic Small Round Cell Tumor: Results from the Surveillance, Epidemiology, and End Results Database

  https://pmc.ncbi.nlm.nih.gov/articles/PMC4238280/

  Desmoplastic small round cell tumor (DSRCT) is a rare but highly fatal malignancy. […] Due to the rarity of this neoplasm, no large population based studies exist. […] A total of 192 cases of DSRCT were identified. […] Peak incidence age was between 20 and 24 years. […] Age-adjusted incidence rate for blacks was 0.5 cases/million and for whites was 0.2 cases/million (P = 0.037). […] DSRCT is more common in males and in people of African-American descent. […] The age-adjusted incidence rate of DSRCT was 0.3 cases/million, with a peak incidence of 0.74 cases/million in persons 20-24 years of age. […] Age-adjusted incidence rates for males and females were 0.4 and 0.1 cases/million, respectively (P 0.001). […] Age-adjusted incidence rates for Caucasians, African-Americans, American Indians, and Asian Pacific Islanders were 0.2, 0.5, 0.3, and 0.1 cases/million, respectively, with a statistically significant difference between Caucasians and African-Americans (P = 0.037).

• #11 Desmoplastic Small Round Cell Tumor: A Review of Main Molecular Abnormalities and Emerging Therapy

  https://www.mdpi.com/2072-6694/13/3/498

  In a study published in 2014, a total of 192 cases of DSRCT were identified in the SEER database between 1973 to 2007. The age-adjusted incidence rate based on this analysis was 0.3 cases/million, with a peak incidence of 0.74 in individuals 20–24 years-old. There is a predominance of DSRCT in males. The age-adjusted incidence rates for males and females were 0.4 and 0.1 cases/million, respectively (p < 0.001). There is predominance in African–American individuals and it is more common in males. The age-adjusted incidence rates are higher among African-Americans as compared to Caucasians (0.5 × 0.2, p = 0.037, respectively). Out of 192 cases, the common primary sites of disease were the peritoneum or soft tissue of abdomen and pelvis (42%) and less common primary sites included the ovary/fallopian tube (6 cases), orbit (1 case), cerebellum (1 case), and cerebral ventricle (1 case).

• #12 Desmoplastic small round cell tumor of the liver: diagnosing a rare case on liver biopsy | Diagnostic Pathology | Full Text

  https://diagnosticpathology.biomedcentral.com/articles/10.1186/s13000-023-01373-1

  Desmoplastic small round cell tumors (DSRCT) are rare and highly invasive malignant soft tissue tumors that mainly occur in children and young people, with an incidence of 0.0002-0.0005. […] Primary DSRCT of the liver is extremely rare, with fewer than 10 reported cases. […] DSRCT is a rare neoplasm that accounts for less than 1% of soft tissue sarcomas with extremely aggressive behavior. […] The age of onset of DSRCT is 3-52 years old, about 80% of the patients are 20-30 years old, and the ratio of male to female is 3:5:1. […] DSRCT is mainly located in the serosa of the abdominal cavity and pelvis, and usually metastasizes to the surrounding lymph nodes, liver, lung, and so on. […] Currently, there is a lack of effective treatments for patients with DSRCT. […] But the prognosis of DSRCT is poor and the survival time is short, with an overall median survival of 2 years and a 5-year survival rate approaching 15%. […] In summary, primary DSRCT of the liver are extremely rare and can be challenging for core biopsy specimens.

• #13 Orphanet: Desmoplastic small round cell tumor

  https://www.orpha.net/en/disease/detail/83469

  DSRCT is extremely rare. Only a few hundred cases have been reported worldwide since the first description in 1989. It usually affects males, during adolescence or young adulthood, with a male-to-female ratio of 4:1. […] Prognosis is poor. Median overall survival is 17 months and less than 20% of patients live more than 5 years after diagnosis.

• #14 Desmoplastic small round cell tumor: the report of two cases and literature analysis review of the radiological findings – Chen – Quantitative Imaging in Medicine and Surgery

  https://qims.amegroups.org/article/view/114164/html

  Desmoplastic small round cell tumor (DSRCT) is a rare, unique, and highly malignant tumor that tends to occur in children and adolescents. There is an obvious gender preference, with a male to female ratio reaching 5 to 1. It occurs predominantly in the abdominal pelvic cavity, spreads along the peritoneal surface, and very rarely appears in the lungs, salivary glands, heart, colon, bones and gonads. DSRCT has an occult onset and rapid disease progression and is susceptible to implantable spread and blood and lymphatic metastasis, which most often occurs in the liver, followed by the lungs, lymph nodes, and bones. In recent years, a small number of studies have compared adolescents and adults (18 years old) with DSRCT, but no significant differences in clinical manifestations, treatment methods, or prognoses between the 2 groups were found. DSRCT often occurs in adolescents and young men, the peritoneal cavity is the most common site, and usually spreads along the peritoneal surface. Clinical symptoms are nonspecific, mainly manifest as abdominal pain, bloating, or palpable abdominal masses. Most (~90%) cases of DSRCT are associated with diffuse or multifocal large omental and peritoneal lesions. Diaphragmatic involvement and retroperitoneal involvement are seen in 40% to 50% of cases, mainly in the form of tumor implantation and lymph node metastases, with additional liver metastases being relatively common (30%). The primary renal DSRCT was first reported in 2004 by Su et al. Thus far, 19 cases of primary renal DSRCT have been reported in the literature, so we report our case 1 as the 20th case. When it occurs in visceral organs (such as the kidney), it is radiologically and pathologically similar to other malignancies, making its diagnosis markedly challenging. The characteristic imaging finding of DSRCT is multiple soft tissue masses in the abdomen and pelvis involving the omentum or mesentery without an exact organ of origin. The multiple larger nodular or lobulated masses often appear with heterogeneous density, similar to necrosis, cystic changes, and hemorrhage, displaying areas of nonenhancement. PET-CT usually shows multiple intraperitoneal hypermetabolic masses with a variable SUVmax of the masses, often up to 12, indicating a strong hypermetabolic trend. The diagnosis of DSRCT relies on pathology and immunohistochemistry. Histopathological examination demonstrates nests, cords, and sheets of small round cells embedded in the desmoplastic stroma. The differential diagnosis of DSRCT is challenging, as diffusely spreading tumors such as desmoid tumors, malignant peritoneal mesothelioma, lymphoma, peritoneal sarcoma, gastrointestinal mesenchymal tumors, and Castleman disease can mimic DSRCT. At present, there is no standard treatment protocol for DSRCT, and complete surgical resection of DSRCT is crucial when technically feasible. For most patients in advanced stages, effective systemic chemotherapy helps in systemic control. Despite advances in multimodal therapy, patients with DSRCT have a poor prognosis, with about 60-70% dying from the disease within 2-3 years.

• #15 Incidence and Outcomes of Desmoplastic Small Round Cell Tumor: Results from the Surveillance, Epidemiology, and End Results Database

  https://pmc.ncbi.nlm.nih.gov/articles/PMC4238280/

  Desmoplastic small round cell tumor (DSRCT) is a rare but highly fatal malignancy. […] Due to the rarity of this neoplasm, no large population based studies exist. […] A total of 192 cases of DSRCT were identified. […] Peak incidence age was between 20 and 24 years. […] Age-adjusted incidence rate for blacks was 0.5 cases/million and for whites was 0.2 cases/million (P = 0.037). […] DSRCT is more common in males and in people of African-American descent. […] The age-adjusted incidence rate of DSRCT was 0.3 cases/million, with a peak incidence of 0.74 cases/million in persons 20-24 years of age. […] Age-adjusted incidence rates for males and females were 0.4 and 0.1 cases/million, respectively (P 0.001). […] Age-adjusted incidence rates for Caucasians, African-Americans, American Indians, and Asian Pacific Islanders were 0.2, 0.5, 0.3, and 0.1 cases/million, respectively, with a statistically significant difference between Caucasians and African-Americans (P = 0.037).

• #16 Incidence and Outcomes of Desmoplastic Small Round Cell Tumor: Results from the Surveillance, Epidemiology, and End Results Database

  https://pmc.ncbi.nlm.nih.gov/articles/PMC4238280/

  Desmoplastic small round cell tumor (DSRCT) is a rare but highly fatal malignancy. […] Due to the rarity of this neoplasm, no large population based studies exist. […] A total of 192 cases of DSRCT were identified. […] Peak incidence age was between 20 and 24 years. […] Age-adjusted incidence rate for blacks was 0.5 cases/million and for whites was 0.2 cases/million (P = 0.037). […] DSRCT is more common in males and in people of African-American descent. […] The age-adjusted incidence rate of DSRCT was 0.3 cases/million, with a peak incidence of 0.74 cases/million in persons 20-24 years of age. […] Age-adjusted incidence rates for males and females were 0.4 and 0.1 cases/million, respectively (P 0.001). […] Age-adjusted incidence rates for Caucasians, African-Americans, American Indians, and Asian Pacific Islanders were 0.2, 0.5, 0.3, and 0.1 cases/million, respectively, with a statistically significant difference between Caucasians and African-Americans (P = 0.037).

• #17 Incidence and outcomes of desmoplastic small round cell tumor: results from the surveillance, epidemiology, and end results database – PubMed

  https://pubmed.ncbi.nlm.nih.gov/25431592/

  Desmoplastic small round cell tumor (DSRCT) is a rare but highly fatal malignancy. Due to the rarity of this neoplasm, no large population based studies exist. A total of 192 cases of DSRCT were identified. Peak incidence age was between 20 and 24 years. Age-adjusted incidence rate for blacks was 0.5 cases/million and for whites was 0.2 cases/million (P = 0.037). DSRCT is more common in males and in people of African-American descent. Although overall survival remains poor, radiation therapy following surgery seems to improve outcome in these patients. […] There was no statistically significant difference in survival based on gender or ethnicity. When adjusted for age, there was no statistically significant difference in survival amongst patients who received radiation therapy compared to those who did not (HRadj = 0.73; 95% CI 0.49, 1.11). There was a statistically significant survival advantage for patients who received radiation after surgery compared to those who did not (HR 0.49; 95% CI 0.30, 0.79).

• #18 Incidence and Outcomes of Desmoplastic Small Round Cell Tumor: Results from the Surveillance, Epidemiology, and End Results Database

  https://pmc.ncbi.nlm.nih.gov/articles/PMC4238280/

  Desmoplastic small round cell tumor (DSRCT) is a rare but highly fatal malignancy. […] Due to the rarity of this neoplasm, no large population based studies exist. […] A total of 192 cases of DSRCT were identified. […] Peak incidence age was between 20 and 24 years. […] Age-adjusted incidence rate for blacks was 0.5 cases/million and for whites was 0.2 cases/million (P = 0.037). […] DSRCT is more common in males and in people of African-American descent. […] The age-adjusted incidence rate of DSRCT was 0.3 cases/million, with a peak incidence of 0.74 cases/million in persons 20-24 years of age. […] Age-adjusted incidence rates for males and females were 0.4 and 0.1 cases/million, respectively (P 0.001). […] Age-adjusted incidence rates for Caucasians, African-Americans, American Indians, and Asian Pacific Islanders were 0.2, 0.5, 0.3, and 0.1 cases/million, respectively, with a statistically significant difference between Caucasians and African-Americans (P = 0.037).

• #19 Desmoplastic Small Round Cell Tumor: A Review of Main Molecular Abnormalities and Emerging Therapy

  https://www.mdpi.com/2072-6694/13/3/498

  In a study published in 2014, a total of 192 cases of DSRCT were identified in the SEER database between 1973 to 2007. The age-adjusted incidence rate based on this analysis was 0.3 cases/million, with a peak incidence of 0.74 in individuals 20–24 years-old. There is a predominance of DSRCT in males. The age-adjusted incidence rates for males and females were 0.4 and 0.1 cases/million, respectively (p < 0.001). There is predominance in African–American individuals and it is more common in males. The age-adjusted incidence rates are higher among African-Americans as compared to Caucasians (0.5 × 0.2, p = 0.037, respectively). Out of 192 cases, the common primary sites of disease were the peritoneum or soft tissue of abdomen and pelvis (42%) and less common primary sites included the ovary/fallopian tube (6 cases), orbit (1 case), cerebellum (1 case), and cerebral ventricle (1 case).

• #20 Desmoplastic Small Round Cell Tumor: A Review of Main Molecular Abnormalities and Emerging Therapy

  https://www.mdpi.com/2072-6694/13/3/498

  In a study published in 2014, a total of 192 cases of DSRCT were identified in the SEER database between 1973 to 2007. The age-adjusted incidence rate based on this analysis was 0.3 cases/million, with a peak incidence of 0.74 in individuals 20–24 years-old. There is a predominance of DSRCT in males. The age-adjusted incidence rates for males and females were 0.4 and 0.1 cases/million, respectively (p < 0.001). There is predominance in African–American individuals and it is more common in males. The age-adjusted incidence rates are higher among African-Americans as compared to Caucasians (0.5 × 0.2, p = 0.037, respectively). Out of 192 cases, the common primary sites of disease were the peritoneum or soft tissue of abdomen and pelvis (42%) and less common primary sites included the ovary/fallopian tube (6 cases), orbit (1 case), cerebellum (1 case), and cerebral ventricle (1 case).

• #21 Desmoplastic Small Round Cell Tumor: A Review of Main Molecular Abnormalities and Emerging Therapy

  https://www.mdpi.com/2072-6694/13/3/498

  In a study published in 2014, a total of 192 cases of DSRCT were identified in the SEER database between 1973 to 2007. The age-adjusted incidence rate based on this analysis was 0.3 cases/million, with a peak incidence of 0.74 in individuals 20–24 years-old. There is a predominance of DSRCT in males. The age-adjusted incidence rates for males and females were 0.4 and 0.1 cases/million, respectively (p < 0.001). There is predominance in African–American individuals and it is more common in males. The age-adjusted incidence rates are higher among African-Americans as compared to Caucasians (0.5 × 0.2, p = 0.037, respectively). Out of 192 cases, the common primary sites of disease were the peritoneum or soft tissue of abdomen and pelvis (42%) and less common primary sites included the ovary/fallopian tube (6 cases), orbit (1 case), cerebellum (1 case), and cerebral ventricle (1 case).

• #22 Desmoplastic small round cell tumor: the report of two cases and literature analysis review of the radiological findings – Chen – Quantitative Imaging in Medicine and Surgery

  https://qims.amegroups.org/article/view/114164/html

  Desmoplastic small round cell tumor (DSRCT) is a rare, unique, and highly malignant tumor that tends to occur in children and adolescents. There is an obvious gender preference, with a male to female ratio reaching 5 to 1. It occurs predominantly in the abdominal pelvic cavity, spreads along the peritoneal surface, and very rarely appears in the lungs, salivary glands, heart, colon, bones and gonads. DSRCT has an occult onset and rapid disease progression and is susceptible to implantable spread and blood and lymphatic metastasis, which most often occurs in the liver, followed by the lungs, lymph nodes, and bones. In recent years, a small number of studies have compared adolescents and adults (18 years old) with DSRCT, but no significant differences in clinical manifestations, treatment methods, or prognoses between the 2 groups were found. DSRCT often occurs in adolescents and young men, the peritoneal cavity is the most common site, and usually spreads along the peritoneal surface. Clinical symptoms are nonspecific, mainly manifest as abdominal pain, bloating, or palpable abdominal masses. Most (~90%) cases of DSRCT are associated with diffuse or multifocal large omental and peritoneal lesions. Diaphragmatic involvement and retroperitoneal involvement are seen in 40% to 50% of cases, mainly in the form of tumor implantation and lymph node metastases, with additional liver metastases being relatively common (30%). The primary renal DSRCT was first reported in 2004 by Su et al. Thus far, 19 cases of primary renal DSRCT have been reported in the literature, so we report our case 1 as the 20th case. When it occurs in visceral organs (such as the kidney), it is radiologically and pathologically similar to other malignancies, making its diagnosis markedly challenging. The characteristic imaging finding of DSRCT is multiple soft tissue masses in the abdomen and pelvis involving the omentum or mesentery without an exact organ of origin. The multiple larger nodular or lobulated masses often appear with heterogeneous density, similar to necrosis, cystic changes, and hemorrhage, displaying areas of nonenhancement. PET-CT usually shows multiple intraperitoneal hypermetabolic masses with a variable SUVmax of the masses, often up to 12, indicating a strong hypermetabolic trend. The diagnosis of DSRCT relies on pathology and immunohistochemistry. Histopathological examination demonstrates nests, cords, and sheets of small round cells embedded in the desmoplastic stroma. The differential diagnosis of DSRCT is challenging, as diffusely spreading tumors such as desmoid tumors, malignant peritoneal mesothelioma, lymphoma, peritoneal sarcoma, gastrointestinal mesenchymal tumors, and Castleman disease can mimic DSRCT. At present, there is no standard treatment protocol for DSRCT, and complete surgical resection of DSRCT is crucial when technically feasible. For most patients in advanced stages, effective systemic chemotherapy helps in systemic control. Despite advances in multimodal therapy, patients with DSRCT have a poor prognosis, with about 60-70% dying from the disease within 2-3 years.

• #23 Desmoplastic small round cell tumor: the report of two cases and literature analysis review of the radiological findings – Chen – Quantitative Imaging in Medicine and Surgery

  https://qims.amegroups.org/article/view/114164/html

  Desmoplastic small round cell tumor (DSRCT) is a rare, unique, and highly malignant tumor that tends to occur in children and adolescents. There is an obvious gender preference, with a male to female ratio reaching 5 to 1. It occurs predominantly in the abdominal pelvic cavity, spreads along the peritoneal surface, and very rarely appears in the lungs, salivary glands, heart, colon, bones and gonads. DSRCT has an occult onset and rapid disease progression and is susceptible to implantable spread and blood and lymphatic metastasis, which most often occurs in the liver, followed by the lungs, lymph nodes, and bones. In recent years, a small number of studies have compared adolescents and adults (18 years old) with DSRCT, but no significant differences in clinical manifestations, treatment methods, or prognoses between the 2 groups were found. DSRCT often occurs in adolescents and young men, the peritoneal cavity is the most common site, and usually spreads along the peritoneal surface. Clinical symptoms are nonspecific, mainly manifest as abdominal pain, bloating, or palpable abdominal masses. Most (~90%) cases of DSRCT are associated with diffuse or multifocal large omental and peritoneal lesions. Diaphragmatic involvement and retroperitoneal involvement are seen in 40% to 50% of cases, mainly in the form of tumor implantation and lymph node metastases, with additional liver metastases being relatively common (30%). The primary renal DSRCT was first reported in 2004 by Su et al. Thus far, 19 cases of primary renal DSRCT have been reported in the literature, so we report our case 1 as the 20th case. When it occurs in visceral organs (such as the kidney), it is radiologically and pathologically similar to other malignancies, making its diagnosis markedly challenging. The characteristic imaging finding of DSRCT is multiple soft tissue masses in the abdomen and pelvis involving the omentum or mesentery without an exact organ of origin. The multiple larger nodular or lobulated masses often appear with heterogeneous density, similar to necrosis, cystic changes, and hemorrhage, displaying areas of nonenhancement. PET-CT usually shows multiple intraperitoneal hypermetabolic masses with a variable SUVmax of the masses, often up to 12, indicating a strong hypermetabolic trend. The diagnosis of DSRCT relies on pathology and immunohistochemistry. Histopathological examination demonstrates nests, cords, and sheets of small round cells embedded in the desmoplastic stroma. The differential diagnosis of DSRCT is challenging, as diffusely spreading tumors such as desmoid tumors, malignant peritoneal mesothelioma, lymphoma, peritoneal sarcoma, gastrointestinal mesenchymal tumors, and Castleman disease can mimic DSRCT. At present, there is no standard treatment protocol for DSRCT, and complete surgical resection of DSRCT is crucial when technically feasible. For most patients in advanced stages, effective systemic chemotherapy helps in systemic control. Despite advances in multimodal therapy, patients with DSRCT have a poor prognosis, with about 60-70% dying from the disease within 2-3 years.

• #24

  https://journals.lww.com/md-journal/fulltext/2020/07240/a_nationwide_analysis_of_desmoplastic_small_round.78.aspx

  This study aim is to enhance the understanding, diagnosis and treatment of desmoplastic small round cell tumor (DSRCT) and to determine what factors can affect survival of the disease in China. […] The prognosis of DSRCT patients is very poor, overall survival is approximately 30% to 55% despite chemotherapy, radiotherapy, and aggressive surgical resection. […] DSRCT has a predilection for children and adolescents, and primarily involves the abdomen and pelvis. The tumor is more common among men than among women. […] The main primary tumor site was the abdomen and/or pelvis in 92/104 patients (88.5%). Only 25% of patients had localized disease. […] Multivariate analysis revealed that Metastatic status (HR: 2.327, 95% CI: 1.1364.768, P = .021), Surgical patterns (HR: 0.673, 95% CI: 0.4870.928, P = .016), and Adjuvant chemotherapy (HR: 0.337, 95% CI: 0.1670.678, P = .002) were significant independent prognostic factors for longer overall survival. […] The survival rates are lower in patients with metastases or no surgery or incomplete surgery or no chemotherapy, suggesting the importance of early diagnosis, early treatment, complete surgery and adjuvant chemotherapy.

• #25 Desmoplastic small round cell tumor: the report of two cases and literature analysis review of the radiological findings – Chen – Quantitative Imaging in Medicine and Surgery

  https://qims.amegroups.org/article/view/114164/html

  Desmoplastic small round cell tumor (DSRCT) is a rare, unique, and highly malignant tumor that tends to occur in children and adolescents. There is an obvious gender preference, with a male to female ratio reaching 5 to 1. It occurs predominantly in the abdominal pelvic cavity, spreads along the peritoneal surface, and very rarely appears in the lungs, salivary glands, heart, colon, bones and gonads. DSRCT has an occult onset and rapid disease progression and is susceptible to implantable spread and blood and lymphatic metastasis, which most often occurs in the liver, followed by the lungs, lymph nodes, and bones. In recent years, a small number of studies have compared adolescents and adults (18 years old) with DSRCT, but no significant differences in clinical manifestations, treatment methods, or prognoses between the 2 groups were found. DSRCT often occurs in adolescents and young men, the peritoneal cavity is the most common site, and usually spreads along the peritoneal surface. Clinical symptoms are nonspecific, mainly manifest as abdominal pain, bloating, or palpable abdominal masses. Most (~90%) cases of DSRCT are associated with diffuse or multifocal large omental and peritoneal lesions. Diaphragmatic involvement and retroperitoneal involvement are seen in 40% to 50% of cases, mainly in the form of tumor implantation and lymph node metastases, with additional liver metastases being relatively common (30%). The primary renal DSRCT was first reported in 2004 by Su et al. Thus far, 19 cases of primary renal DSRCT have been reported in the literature, so we report our case 1 as the 20th case. When it occurs in visceral organs (such as the kidney), it is radiologically and pathologically similar to other malignancies, making its diagnosis markedly challenging. The characteristic imaging finding of DSRCT is multiple soft tissue masses in the abdomen and pelvis involving the omentum or mesentery without an exact organ of origin. The multiple larger nodular or lobulated masses often appear with heterogeneous density, similar to necrosis, cystic changes, and hemorrhage, displaying areas of nonenhancement. PET-CT usually shows multiple intraperitoneal hypermetabolic masses with a variable SUVmax of the masses, often up to 12, indicating a strong hypermetabolic trend. The diagnosis of DSRCT relies on pathology and immunohistochemistry. Histopathological examination demonstrates nests, cords, and sheets of small round cells embedded in the desmoplastic stroma. The differential diagnosis of DSRCT is challenging, as diffusely spreading tumors such as desmoid tumors, malignant peritoneal mesothelioma, lymphoma, peritoneal sarcoma, gastrointestinal mesenchymal tumors, and Castleman disease can mimic DSRCT. At present, there is no standard treatment protocol for DSRCT, and complete surgical resection of DSRCT is crucial when technically feasible. For most patients in advanced stages, effective systemic chemotherapy helps in systemic control. Despite advances in multimodal therapy, patients with DSRCT have a poor prognosis, with about 60-70% dying from the disease within 2-3 years.

• #26

  http://waocp.com/journal/index.php/apjcc/article/view/1022

  Desmoplastic small round cell tumor (DSRCT) is a rare and aggressive malignancy that predominantly affects males. It typically arises in the abdominal cavity and is typically diagnosed through histopathological examination. Most cases of DSRCT exhibit a characteristic EWSR1-WT1 gene fusion. […] DSRCT mainly develops in adolescents and young adults; the mean age at diagnosis is approximately 22 years, and the male-to-female ratio is 4: 1. […] CT or Magnetic Resonance Imaging (MRI) showing multiple peritoneal implants increases the suspicion of DSRCT. The most common site of initial organ metastasis is the liver. The lungs, pleura, and mediastinum are the next most common locations. […] Proper consensus about treatment has not yet been established. DSRCT still remains a clinical challenge for oncologists because of the lack of standard guidelines.

• #27 Incidence and Outcomes of Desmoplastic Small Round Cell Tumor: Results from the Surveillance, Epidemiology, and End Results Database

  https://pmc.ncbi.nlm.nih.gov/articles/PMC4238280/

  This is the first SEER database analysis evaluating both incidence and survival data among patients with DSRCT. […] The majority of published data on DSRCT are single institution studies analyzing small numbers of cases given heterogeneous treatment. […] The patients described in the literature with the longest survival tend to have received multimodality therapy including aggressive surgery, multiagent chemotherapy including hyperthermic peritoneal perfusion, and radiation therapy. […] This review of SEER data supports inclusion of multimodality therapy in treatment for DSRCT, with data revealing improvement in survival by 32% compared to those without radiation treatment.

• #28 Incidence and Outcomes of Desmoplastic Small Round Cell Tumor: Results from the Surveillance, Epidemiology, and End Results Database

  https://pmc.ncbi.nlm.nih.gov/articles/PMC4238280/

  Desmoplastic small round cell tumor (DSRCT) is a rare but highly fatal malignancy. […] Due to the rarity of this neoplasm, no large population based studies exist. […] A total of 192 cases of DSRCT were identified. […] Peak incidence age was between 20 and 24 years. […] Age-adjusted incidence rate for blacks was 0.5 cases/million and for whites was 0.2 cases/million (P = 0.037). […] DSRCT is more common in males and in people of African-American descent. […] The age-adjusted incidence rate of DSRCT was 0.3 cases/million, with a peak incidence of 0.74 cases/million in persons 20-24 years of age. […] Age-adjusted incidence rates for males and females were 0.4 and 0.1 cases/million, respectively (P 0.001). […] Age-adjusted incidence rates for Caucasians, African-Americans, American Indians, and Asian Pacific Islanders were 0.2, 0.5, 0.3, and 0.1 cases/million, respectively, with a statistically significant difference between Caucasians and African-Americans (P = 0.037).

• #29 Incidence and outcomes of desmoplastic small round cell tumor: results from the surveillance, epidemiology, and end results database. – Document – Gale Academic OneFile

  https://go.gale.com/ps/i.do?id=GALE%7CA420324814&sid=googleScholar&v=2.1&it=r&linkaccess=abs&issn=16878558&p=AONE&sw=w

  Desmoplastic small round cell tumor (DSRCT) is a rare but highly fatal malignancy. Due to the rarity of this neoplasm, no large population based studies exist. A total of 192 cases of DSRCT were identified. Peak incidence age was between 20 and 24 years. Age-adjusted incidence rate for blacks was 0.5 cases/million and for whites was 0.2 cases/million (P = 0.037). DSRCT is more common in males and in people of African-American descent. Although overall survival remains poor, radiation therapy following surgery seems to improve outcome in these patients.

• #30 Desmoplastic small round cell tumor: an update of current management practices | Journal of the Egyptian National Cancer Institute | Full Text

  https://jenci.springeropen.com/articles/10.1186/s43046-025-00276-0

  Desmoplastic small round cell tumor (DSRCT) poses a diagnostic challenge, initiating with imaging techniques like ultrasound, CT, MRI, and PET scans, with CT being the primary choice for abdominal tumor visualization. […] The prognosis of DSRCT patients is very poor, overall survival is approximately 30% to 55% despite chemotherapy, radiotherapy, and aggressive surgical resection. […] A retrospective cohort study conducted in 2022 evaluated the incidence and survival rates of patients diagnosed with DSRCT in the USA. Data was obtained from the Surveillance, Epidemiology, and End Results (SEER) 9 Cancer Registry which reported 154 cases of DSRCT from 1975 to 2018, with 94% of the cases occurring after the year 2000. […] Therefore, the clinical presentation of DSRCT occurs predominantly in males around 30 years old and consists of a large intra-abdominal, pelvic, or retroperitoneal mass with metastasis to the lungs, lymph nodes, and liver.

• #31 Desmoplastic Small Round Cell Tumor: A Review of Main Molecular Abnormalities and Emerging Therapy

  https://www.mdpi.com/2072-6694/13/3/498

  Desmoplastic small round cell tumor is a very rare subtype of sarcoma. For research purposes, DSRCT cases can be searched using the histology and behavior (malignant) classification code 8806/3. There is no uniform definition of rare sarcoma, however, the burden of rare cancer in our current days is great. The US Orphan Drug Act of 1983 defined rare diseases as those affecting less than 200,000 people in the United States. In 2010, Greenlee et al. described the US burden of rare cancers according to the National Cancer Institute definition as those cancers with fewer than 15 cases per 100,000 people per year. More recently, a consortium from the European Union, Surveillance of Rare Cancer in Europe (RARECARE), described a new definition of rare cancer in Europe as those with fewer than 6 cases per 100,000 people per year.

• #32 Desmoplastic Small Round Cell Tumors (dsrcts) – Market Insight, Epidemiology and Market Forecast – 2034

  https://www.researchandmarkets.com/reports/5524502/desmoplastic-small-round-cell-tumors-dsrcts?srsltid=AfmBOop2rVGowxUnxmGV9LKYWrcn1YE8Y7o7w7ZcS6VzA7yP62tpjyeB

  The Desmoplastic Small Round Cell Tumors (dsrcts) epidemiology division provide insights about historical and current Desmoplastic Small Round Cell Tumors (dsrcts) patient pool and forecasted trend for every seven major countries. It helps to recognize the causes of current and forecasted trends by exploring numerous studies and views of key opinion leaders. This part of The report also provides the diagnosed patient pool and their trends along with assumptions undertaken. […] The disease epidemiology covered in the report provides historical as well as forecasted Desmoplastic Small Round Cell Tumors (dsrcts) epidemiology scenario in the 7MM covering the United States, EU5 countries (Germany, Spain, Italy, France, and the United Kingdom), and Japan from 2020 to 2034. […] The epidemiology segment also provides the Desmoplastic Small Round Cell Tumors (dsrcts) epidemiology data and findings across the United States, EU5 (Germany, France, Italy, Spain, and the United Kingdom), and Japan.

• #33 Desmoplastic Small Round Cell Tumor – Epidemiology Forecast – 2032

  https://www.researchandmarkets.com/reports/5525599/desmoplastic-small-round-cell-tumor?srsltid=AfmBOopD4BdElDZgKIoYqGHOIbte60V8UQ_2BN7p-IRWrabJEqTP1ZEy

  The „Desmoplastic Small Round Cell Tumor – Epidemiology Forecast to 2032” report delivers an in-depth understanding of the disease, historical and forecasted Desmoplastic Small Round Cell Tumor epidemiology in the 7MM, i.e., the United States, EU5 (Germany, Spain, Italy, France, and the United Kingdom), and Japan. […] The Desmoplastic Small Round Cell Tumor epidemiology report gives a thorough understanding of the Desmoplastic Small Round Cell Tumor by including details such as disease definition, symptoms, causes, pathophysiology, and diagnosis. It also provides treatment algorithms and treatment guidelines for Desmoplastic Small Round Cell Tumor in the US, Europe, and Japan. The report covers the detailed information of the Desmoplastic Small Round Cell Tumor epidemiology scenario in seven major countries (US, EU5, and Japan).

• #34 Incidence and Outcomes of Desmoplastic Small Round Cell Tumor: Results from the Surveillance, Epidemiology, and End Results Database

  https://pmc.ncbi.nlm.nih.gov/articles/PMC4238280/

  This is the first SEER database analysis evaluating both incidence and survival data among patients with DSRCT. […] The majority of published data on DSRCT are single institution studies analyzing small numbers of cases given heterogeneous treatment. […] The patients described in the literature with the longest survival tend to have received multimodality therapy including aggressive surgery, multiagent chemotherapy including hyperthermic peritoneal perfusion, and radiation therapy. […] This review of SEER data supports inclusion of multimodality therapy in treatment for DSRCT, with data revealing improvement in survival by 32% compared to those without radiation treatment.

• #35 Desmoplastic Small Round Cell Tumor: A Review of Main Molecular Abnormalities and Emerging Therapy

  https://www.mdpi.com/2072-6694/13/3/498

  Despite multimodal treatment, DSRCT has a poor prognosis, and approximately 60–70% of patients die due to disease progression usually within 3 years after diagnosis. The median overall survival varies between 28–60 months, with a median disease-free survival between 10–15.5 months. One study proposed that the absence of extra-peritoneal metastasis, complete surgical resection and postoperative WAP-RT are factors predictive of 3-year overall survival. The multimodality treatment combining chemotherapy, cytoreductive surgery, HIPEC and WAP-RT can warrant local control of the disease, but patients will still present distant metastasis during follow-up, meaning that more effective chemotherapy is necessary to improve long-term outcomes. […] DSRCT is characterized by poor response to conventional chemotherapy and early relapse after radical surgery. Second-line treatment is ineffective in most cases. In our cohort, out of 19 patients treated with first-line chemotherapy, 13 received the second-line and the progression-free survival was only 3.9 months. This short survival time highlights the aggressiveness of this disease and the challenge in developing new therapeutic strategies to treat these young patients. Despite the development of new regimens for Ewing Sarcoma and other soft tissue and bone sarcoma in recent years, DSRCT is underrepresented or were not included in the trials that lead to the drug approval. As a result, the evidence to use second-line therapy is very limited. It is paramount to develop active cooperative groups to quickly collect data and propose new strategies for the treatment of DSRCT.

• #36 Desmoplastic Small Round Cell Tumor: A Review of Main Molecular Abnormalities and Emerging Therapy

  https://www.mdpi.com/2072-6694/13/3/498

  Despite multimodal treatment, DSRCT has a poor prognosis, and approximately 60–70% of patients die due to disease progression usually within 3 years after diagnosis. The median overall survival varies between 28–60 months, with a median disease-free survival between 10–15.5 months. One study proposed that the absence of extra-peritoneal metastasis, complete surgical resection and postoperative WAP-RT are factors predictive of 3-year overall survival. The multimodality treatment combining chemotherapy, cytoreductive surgery, HIPEC and WAP-RT can warrant local control of the disease, but patients will still present distant metastasis during follow-up, meaning that more effective chemotherapy is necessary to improve long-term outcomes. […] DSRCT is characterized by poor response to conventional chemotherapy and early relapse after radical surgery. Second-line treatment is ineffective in most cases. In our cohort, out of 19 patients treated with first-line chemotherapy, 13 received the second-line and the progression-free survival was only 3.9 months. This short survival time highlights the aggressiveness of this disease and the challenge in developing new therapeutic strategies to treat these young patients. Despite the development of new regimens for Ewing Sarcoma and other soft tissue and bone sarcoma in recent years, DSRCT is underrepresented or were not included in the trials that lead to the drug approval. As a result, the evidence to use second-line therapy is very limited. It is paramount to develop active cooperative groups to quickly collect data and propose new strategies for the treatment of DSRCT.

• #37 Desmoplastic small round cell tumor: an update of current management practices | Journal of the Egyptian National Cancer Institute | Full Text

  https://jenci.springeropen.com/articles/10.1186/s43046-025-00276-0

  The diagnostic process lacks a universally standardized staging system designed specifically for DSRCT. […] Systemic chemotherapy remains the cornerstone of DSRCT management. […] The most commonly used first-line regimen, the P6 protocol, combines multiple agents and has shown promising results. […] The potential benefits of hyperthermic intraperitoneal chemotherapy (HIPEC) are introduced, but the effectiveness of combining it with cytoreductive surgery is an area of ongoing investigation. […] Whole abdomen radiotherapy (WART) is discussed as an adjuvant treatment modality, typically administered after chemotherapy, surgical debulking, and HIPEC. […] Immunotherapy represents an exciting frontier in cancer treatment, and potential targets for DSRCT have been identified. […] Recent research highlights the potential of targeting AR, particularly given the elevated testosterone levels in males. […] The promising results and ongoing research in DSRCT management provide clear guidance for future directions in the field.

• #38 Desmoplastic Small Round Cell Tumor: A Review of Main Molecular Abnormalities and Emerging Therapy

  https://www.mdpi.com/2072-6694/13/3/498

  Desmoplastic small round cell tumor is a very rare subtype of sarcoma. For research purposes, DSRCT cases can be searched using the histology and behavior (malignant) classification code 8806/3. There is no uniform definition of rare sarcoma, however, the burden of rare cancer in our current days is great. The US Orphan Drug Act of 1983 defined rare diseases as those affecting less than 200,000 people in the United States. In 2010, Greenlee et al. described the US burden of rare cancers according to the National Cancer Institute definition as those cancers with fewer than 15 cases per 100,000 people per year. More recently, a consortium from the European Union, Surveillance of Rare Cancer in Europe (RARECARE), described a new definition of rare cancer in Europe as those with fewer than 6 cases per 100,000 people per year.

• #39 Desmoplastic Small Round-cell Tumor: Retrospective Review of Institutional Data and Literature Review | Anticancer Research

  https://ar.iiarjournals.org/content/41/8/3859

  Desmoplastic small round-cell tumor (DSCRT) in adults is an extremely rare (age-adjusted incidence 0.3 per million) and aggressive sarcoma with limited data for optimal management. […] Desmoplastic small round-cell tumor (DSCRT) in adults is a rare sarcoma predominantly affecting young adults, with an age-adjusted incidence of 0.3 per million. […] While aggressive multimodality treatment is always warranted for DSCRT, the options are limited by the multicentric presentation, short-lived initial response and lack of established subsequent therapy portending a poor prognosis. […] As a result, 60-70% of patients succumb to DSCRT within 2-3 years despite best care. […] The findings of our study are consistent with historical data namely that the disease predominantly affects young adults with a male predilection.

• #40 Desmoplastic Small Round Cell Tumor: A Review of Main Molecular Abnormalities and Emerging Therapy

  https://www.mdpi.com/2072-6694/13/3/498

  Despite multimodal treatment, DSRCT has a poor prognosis, and approximately 60–70% of patients die due to disease progression usually within 3 years after diagnosis. The median overall survival varies between 28–60 months, with a median disease-free survival between 10–15.5 months. One study proposed that the absence of extra-peritoneal metastasis, complete surgical resection and postoperative WAP-RT are factors predictive of 3-year overall survival. The multimodality treatment combining chemotherapy, cytoreductive surgery, HIPEC and WAP-RT can warrant local control of the disease, but patients will still present distant metastasis during follow-up, meaning that more effective chemotherapy is necessary to improve long-term outcomes. […] DSRCT is characterized by poor response to conventional chemotherapy and early relapse after radical surgery. Second-line treatment is ineffective in most cases. In our cohort, out of 19 patients treated with first-line chemotherapy, 13 received the second-line and the progression-free survival was only 3.9 months. This short survival time highlights the aggressiveness of this disease and the challenge in developing new therapeutic strategies to treat these young patients. Despite the development of new regimens for Ewing Sarcoma and other soft tissue and bone sarcoma in recent years, DSRCT is underrepresented or were not included in the trials that lead to the drug approval. As a result, the evidence to use second-line therapy is very limited. It is paramount to develop active cooperative groups to quickly collect data and propose new strategies for the treatment of DSRCT.

• #41 Orphanet: Desmoplastic small round cell tumor

  https://www.orpha.net/en/disease/detail/83469

  DSRCT is extremely rare. Only a few hundred cases have been reported worldwide since the first description in 1989. It usually affects males, during adolescence or young adulthood, with a male-to-female ratio of 4:1. […] Prognosis is poor. Median overall survival is 17 months and less than 20% of patients live more than 5 years after diagnosis.

• #42 Desmoplastic small round cell tumor (peritoneal) | Radiology Reference Article | Radiopaedia.org

  https://radiopaedia.org/articles/desmoplastic-small-round-cell-tumour-peritoneal?lang=us

  Desmoplastic small round cell tumors of the peritoneum are a rare and highly aggressive primary peritoneal malignancy. […] Desmoplastic small round cell tumor is usually seen in young adolescents and have a male predominance with a mean survival of 2-3 years.

• #43 Desmoplastic Small Round Cell Tumors (DSRCT) – NCI

  https://www.cancer.gov/pediatric-adult-rare-tumor/rare-tumors/rare-soft-tissue-tumors/desmoplastic-small-round-cell-tumors

  How common are desmoplastic small round cell tumors? DSRCT is very rare. DSRCT occurs most often in young white males between the ages of 10 and 30. Some reports say that only about 200 cases of DSRCT have been recorded since the cancer was first described in 1989. […] The five-year survival rate for DSRCT is 15%. Keep in mind that doctors estimate DSRCT survival rates by how groups of people with DSRCT have done in the past. Because there are so few people with DSRCT, these rates may not be very accurate. It is very important to work with a team of experts as soon as possible after diagnosis with DSRCT to improve your chances of survival.

• #44 Intra-Abdominal Desmoplastic Small Round Cell Tumor (DSRCT) and the Role of Hyperthermic Intraperitoneal Chemotherapy (HIPEC): A Review

  https://www.mdpi.com/1718-7729/30/4/299

  Aggressive multimodality treatment and improvements in therapeutics have translated to improved DSRCT patient outcomes. Currently, the results of the largest series of 187 patients with DSRCT have been published by the MDACC group. They have shown an improvement in 5-year OS from 5% before 2003 (without multimodal treatment) to a 5-year OS of 25% with multimodal treatment. However, the prognosis of DSRCT remains poor as patients who respond well to initial therapy eventually recur both intraperitoneally and extraperitoneally.

• #45

  https://journals.lww.com/md-journal/fulltext/2020/07240/a_nationwide_analysis_of_desmoplastic_small_round.78.aspx

  This study aim is to enhance the understanding, diagnosis and treatment of desmoplastic small round cell tumor (DSRCT) and to determine what factors can affect survival of the disease in China. […] The prognosis of DSRCT patients is very poor, overall survival is approximately 30% to 55% despite chemotherapy, radiotherapy, and aggressive surgical resection. […] DSRCT has a predilection for children and adolescents, and primarily involves the abdomen and pelvis. The tumor is more common among men than among women. […] The main primary tumor site was the abdomen and/or pelvis in 92/104 patients (88.5%). Only 25% of patients had localized disease. […] Multivariate analysis revealed that Metastatic status (HR: 2.327, 95% CI: 1.1364.768, P = .021), Surgical patterns (HR: 0.673, 95% CI: 0.4870.928, P = .016), and Adjuvant chemotherapy (HR: 0.337, 95% CI: 0.1670.678, P = .002) were significant independent prognostic factors for longer overall survival. […] The survival rates are lower in patients with metastases or no surgery or incomplete surgery or no chemotherapy, suggesting the importance of early diagnosis, early treatment, complete surgery and adjuvant chemotherapy.

• #46 Desmoplastic Small Round Cell Tumor: A Review of Main Molecular Abnormalities and Emerging Therapy

  https://www.mdpi.com/2072-6694/13/3/498

  Despite multimodal treatment, DSRCT has a poor prognosis, and approximately 60–70% of patients die due to disease progression usually within 3 years after diagnosis. The median overall survival varies between 28–60 months, with a median disease-free survival between 10–15.5 months. One study proposed that the absence of extra-peritoneal metastasis, complete surgical resection and postoperative WAP-RT are factors predictive of 3-year overall survival. The multimodality treatment combining chemotherapy, cytoreductive surgery, HIPEC and WAP-RT can warrant local control of the disease, but patients will still present distant metastasis during follow-up, meaning that more effective chemotherapy is necessary to improve long-term outcomes. […] DSRCT is characterized by poor response to conventional chemotherapy and early relapse after radical surgery. Second-line treatment is ineffective in most cases. In our cohort, out of 19 patients treated with first-line chemotherapy, 13 received the second-line and the progression-free survival was only 3.9 months. This short survival time highlights the aggressiveness of this disease and the challenge in developing new therapeutic strategies to treat these young patients. Despite the development of new regimens for Ewing Sarcoma and other soft tissue and bone sarcoma in recent years, DSRCT is underrepresented or were not included in the trials that lead to the drug approval. As a result, the evidence to use second-line therapy is very limited. It is paramount to develop active cooperative groups to quickly collect data and propose new strategies for the treatment of DSRCT.

• #47

  https://journals.lww.com/md-journal/fulltext/2020/07240/a_nationwide_analysis_of_desmoplastic_small_round.78.aspx

  This study aim is to enhance the understanding, diagnosis and treatment of desmoplastic small round cell tumor (DSRCT) and to determine what factors can affect survival of the disease in China. […] The prognosis of DSRCT patients is very poor, overall survival is approximately 30% to 55% despite chemotherapy, radiotherapy, and aggressive surgical resection. […] DSRCT has a predilection for children and adolescents, and primarily involves the abdomen and pelvis. The tumor is more common among men than among women. […] The main primary tumor site was the abdomen and/or pelvis in 92/104 patients (88.5%). Only 25% of patients had localized disease. […] Multivariate analysis revealed that Metastatic status (HR: 2.327, 95% CI: 1.1364.768, P = .021), Surgical patterns (HR: 0.673, 95% CI: 0.4870.928, P = .016), and Adjuvant chemotherapy (HR: 0.337, 95% CI: 0.1670.678, P = .002) were significant independent prognostic factors for longer overall survival. […] The survival rates are lower in patients with metastases or no surgery or incomplete surgery or no chemotherapy, suggesting the importance of early diagnosis, early treatment, complete surgery and adjuvant chemotherapy.

• #48 Desmoplastic Small Round Cell Tumor: A Review of Main Molecular Abnormalities and Emerging Therapy

  https://www.mdpi.com/2072-6694/13/3/498

  Despite multimodal treatment, DSRCT has a poor prognosis, and approximately 60–70% of patients die due to disease progression usually within 3 years after diagnosis. The median overall survival varies between 28–60 months, with a median disease-free survival between 10–15.5 months. One study proposed that the absence of extra-peritoneal metastasis, complete surgical resection and postoperative WAP-RT are factors predictive of 3-year overall survival. The multimodality treatment combining chemotherapy, cytoreductive surgery, HIPEC and WAP-RT can warrant local control of the disease, but patients will still present distant metastasis during follow-up, meaning that more effective chemotherapy is necessary to improve long-term outcomes. […] DSRCT is characterized by poor response to conventional chemotherapy and early relapse after radical surgery. Second-line treatment is ineffective in most cases. In our cohort, out of 19 patients treated with first-line chemotherapy, 13 received the second-line and the progression-free survival was only 3.9 months. This short survival time highlights the aggressiveness of this disease and the challenge in developing new therapeutic strategies to treat these young patients. Despite the development of new regimens for Ewing Sarcoma and other soft tissue and bone sarcoma in recent years, DSRCT is underrepresented or were not included in the trials that lead to the drug approval. As a result, the evidence to use second-line therapy is very limited. It is paramount to develop active cooperative groups to quickly collect data and propose new strategies for the treatment of DSRCT.

• #49 Incidence and outcomes of desmoplastic small round cell tumor: results from the surveillance, epidemiology, and end results database – PubMed

  https://pubmed.ncbi.nlm.nih.gov/25431592/

  Desmoplastic small round cell tumor (DSRCT) is a rare but highly fatal malignancy. Due to the rarity of this neoplasm, no large population based studies exist. A total of 192 cases of DSRCT were identified. Peak incidence age was between 20 and 24 years. Age-adjusted incidence rate for blacks was 0.5 cases/million and for whites was 0.2 cases/million (P = 0.037). DSRCT is more common in males and in people of African-American descent. Although overall survival remains poor, radiation therapy following surgery seems to improve outcome in these patients. […] There was no statistically significant difference in survival based on gender or ethnicity. When adjusted for age, there was no statistically significant difference in survival amongst patients who received radiation therapy compared to those who did not (HRadj = 0.73; 95% CI 0.49, 1.11). There was a statistically significant survival advantage for patients who received radiation after surgery compared to those who did not (HR 0.49; 95% CI 0.30, 0.79).

• #50 Incidence and Outcomes of Desmoplastic Small Round Cell Tumor: Results from the Surveillance, Epidemiology, and End Results Database

  https://pmc.ncbi.nlm.nih.gov/articles/PMC4238280/

  This is the first SEER database analysis evaluating both incidence and survival data among patients with DSRCT. […] The majority of published data on DSRCT are single institution studies analyzing small numbers of cases given heterogeneous treatment. […] The patients described in the literature with the longest survival tend to have received multimodality therapy including aggressive surgery, multiagent chemotherapy including hyperthermic peritoneal perfusion, and radiation therapy. […] This review of SEER data supports inclusion of multimodality therapy in treatment for DSRCT, with data revealing improvement in survival by 32% compared to those without radiation treatment.

• #51 Intra-Abdominal Desmoplastic Small Round Cell Tumor (DSRCT) and the Role of Hyperthermic Intraperitoneal Chemotherapy (HIPEC): A Review

  https://www.mdpi.com/1718-7729/30/4/299

  The American Joint Committee on Cancer (AJCC) staging system does not account well for DSRCT in view of the unknown primary origin and multifocal nature of disease. The MD Anderson Cancer Center (MDACC) hence developed staging criteria to illustrate disease burden, but it has yet to be validated. These staging criteria consist of a combination of PCI, the presence or absence of liver metastases, and extra-abdominal metastases. […] The Memorial Sloan Kettering Cancer Center (MSKCC) has more recently suggested an image-based risk stratification system, based on the presence of serosal or parenchymal liver lesions and/or ascites at the time of diagnosis. This risk stratification system assigns patients into three risk categories based on the presence of ascites and/or liver metastases at diagnosis.

• #52 Desmoplastic Small Round Cell Tumor: A Review of Main Molecular Abnormalities and Emerging Therapy

  https://www.mdpi.com/2072-6694/13/3/498

  Desmoplastic small round cell tumor (DSRCT) is an extremely rare, aggressive sarcoma affecting adolescents and young adults with male predominance. Generally, it originates from the serosal surface of the abdominal cavity. The hallmark characteristic of DSRCT is the EWSR1–WT1 gene fusion. This translocation up-regulates the expression of PDGFRα, VEGF and other proteins related to tumor and vascular cell proliferation. Current management of DSRCT includes a combination of chemotherapy, radiation and aggressive cytoreductive surgery plus intra-peritoneal hyperthermic chemotherapy (HIPEC). Despite advances in multimodal therapy, outcomes remain poor since the majority of patients present disease recurrence and die within three years. The dismal survival makes DSRCT an orphan disease with an urgent need for new drugs.

• #53 Desmoplastic Small Round Cell Tumor: A Review of Main Molecular Abnormalities and Emerging Therapy

  https://www.mdpi.com/2072-6694/13/3/498

  Despite multimodal treatment, DSRCT has a poor prognosis, and approximately 60–70% of patients die due to disease progression usually within 3 years after diagnosis. The median overall survival varies between 28–60 months, with a median disease-free survival between 10–15.5 months. One study proposed that the absence of extra-peritoneal metastasis, complete surgical resection and postoperative WAP-RT are factors predictive of 3-year overall survival. The multimodality treatment combining chemotherapy, cytoreductive surgery, HIPEC and WAP-RT can warrant local control of the disease, but patients will still present distant metastasis during follow-up, meaning that more effective chemotherapy is necessary to improve long-term outcomes. […] DSRCT is characterized by poor response to conventional chemotherapy and early relapse after radical surgery. Second-line treatment is ineffective in most cases. In our cohort, out of 19 patients treated with first-line chemotherapy, 13 received the second-line and the progression-free survival was only 3.9 months. This short survival time highlights the aggressiveness of this disease and the challenge in developing new therapeutic strategies to treat these young patients. Despite the development of new regimens for Ewing Sarcoma and other soft tissue and bone sarcoma in recent years, DSRCT is underrepresented or were not included in the trials that lead to the drug approval. As a result, the evidence to use second-line therapy is very limited. It is paramount to develop active cooperative groups to quickly collect data and propose new strategies for the treatment of DSRCT.

• #54 Desmoplastic Small Round Cell Tumor: A Review of Main Molecular Abnormalities and Emerging Therapy

  https://www.mdpi.com/2072-6694/13/3/498

  Despite multimodal treatment, DSRCT has a poor prognosis, and approximately 60–70% of patients die due to disease progression usually within 3 years after diagnosis. The median overall survival varies between 28–60 months, with a median disease-free survival between 10–15.5 months. One study proposed that the absence of extra-peritoneal metastasis, complete surgical resection and postoperative WAP-RT are factors predictive of 3-year overall survival. The multimodality treatment combining chemotherapy, cytoreductive surgery, HIPEC and WAP-RT can warrant local control of the disease, but patients will still present distant metastasis during follow-up, meaning that more effective chemotherapy is necessary to improve long-term outcomes. […] DSRCT is characterized by poor response to conventional chemotherapy and early relapse after radical surgery. Second-line treatment is ineffective in most cases. In our cohort, out of 19 patients treated with first-line chemotherapy, 13 received the second-line and the progression-free survival was only 3.9 months. This short survival time highlights the aggressiveness of this disease and the challenge in developing new therapeutic strategies to treat these young patients. Despite the development of new regimens for Ewing Sarcoma and other soft tissue and bone sarcoma in recent years, DSRCT is underrepresented or were not included in the trials that lead to the drug approval. As a result, the evidence to use second-line therapy is very limited. It is paramount to develop active cooperative groups to quickly collect data and propose new strategies for the treatment of DSRCT.

• #55

  http://waocp.com/journal/index.php/apjcc/article/view/1022

  Although complete cytoreductive surgery (CRS) is the standard therapy, the microscopic residual disease is often present. […] Looking at the real-world picture, most patients with DSRCT present in the advanced stage, and hence local therapies are rarely possible. DSRCT is known to be at least somewhat chemosensitive and radiosensitive. […] The reported Median Survival of DSRCT ranges from 17 to 25 months. […] Novel treatment methods using precision oncology are essential in managing this disease. […] In conclusion, DSRCT is a rare and aggressive disease and fatal for the majority of patients at this point in time. A definite treatment protocol has not been devised owing to the uncommonness of the disease.

• #56 A genomic case study of desmoplastic small round cell tumor: comprehensive analysis reveals insights into potential therapeutic targets and development of a monitoring tool for a rare and aggressive disease | Human Genomics | Full Text

  https://humgenomics.biomedcentral.com/articles/10.1186/s40246-016-0092-0

  This limited genomic information about DSRCT impairs new and more efficient therapeutic opportunities for the young patients affected with this rare tumor. […] Overall, our findings revealed genes with potential to be associated with risk assessment and tumorigenesis of this rare type of sarcoma. Additionally, we established a liquid biopsy approach for monitoring patient follow-up based on genomic information that can be similarly adopted for patients diagnosed with a rare tumor.

• #57 Enzalutamide induces cytotoxicity in desmoplastic small round cell tumor independent of the androgen receptor | Communications Biology

  https://www.nature.com/articles/s42003-024-06003-0

  With a 5-year survival rate of 15-25% and a high frequency of recurrence and metastasis, DSRCT is in urgent need of novel therapeutic strategies to improve patient outcomes. […] Our finding that AR is not necessary for DSRCT xenograft growth leads to questions about the target for enzalutamide in DSRCT cell lines and the reason behind DSRCT’s overwhelming male predominance. […] If androgens and AR are not needed for DSRCT growth, then androgen growth dependence cannot explain the strong male prevalence in DSRCT.

• #58 Desmoplastic Small Round Cell Tumor: A Review of Main Molecular Abnormalities and Emerging Therapy

  https://www.mdpi.com/2072-6694/13/3/498

  Desmoplastic small round cell tumor (DSRCT) is an extremely rare, aggressive sarcoma affecting adolescents and young adults with male predominance. Generally, it originates from the serosal surface of the abdominal cavity. The hallmark characteristic of DSRCT is the EWSR1–WT1 gene fusion. This translocation up-regulates the expression of PDGFRα, VEGF and other proteins related to tumor and vascular cell proliferation. Current management of DSRCT includes a combination of chemotherapy, radiation and aggressive cytoreductive surgery plus intra-peritoneal hyperthermic chemotherapy (HIPEC). Despite advances in multimodal therapy, outcomes remain poor since the majority of patients present disease recurrence and die within three years. The dismal survival makes DSRCT an orphan disease with an urgent need for new drugs.

• #59 CXCR4-Directed Imaging and Endoradiotherapy in Desmoplastic Small Round Cell Tumors | Journal of Nuclear Medicine

  https://jnm.snmjournals.org/content/64/9/1424

  Desmoplastic small round cell tumor (DSRCT) is a rare, radiosensitive, yet difficult-to-treat sarcoma subtype affecting predominantly male adolescents. […] Because of the lack of clinical trials in this orphan disease, with approximately 1,000 patients reported to date, no standard therapy has been established. […] Patients with DSRCT have been compiled in sarcoma studies, and systemic chemotherapy regimens are derived from protocols established primarily for Ewing and other soft-tissue sarcomas. […] However, patients experience early relapse and prognosis remains poor, with a median OS of 24-29 mo and 3-y and 5-y survival rates of 30%-35% and 4%, respectively. […] Functional imaging using [18F]FDG PET/CT is regarded as the most suitable imaging technique for DSRCT and helps to select patients with a metabolic response to induction chemotherapy for debulking surgery even in the absence of significant tumor shrinkage according to RECIST.

• #60 Incidence and Outcomes of Desmoplastic Small Round Cell Tumor: Results from the Surveillance, Epidemiology, and End Results Database

  https://pmc.ncbi.nlm.nih.gov/articles/PMC4238280/

  Desmoplastic small round cell tumor (DSRCT) is a rare but highly fatal malignancy. […] Due to the rarity of this neoplasm, no large population based studies exist. […] A total of 192 cases of DSRCT were identified. […] Peak incidence age was between 20 and 24 years. […] Age-adjusted incidence rate for blacks was 0.5 cases/million and for whites was 0.2 cases/million (P = 0.037). […] DSRCT is more common in males and in people of African-American descent. […] The age-adjusted incidence rate of DSRCT was 0.3 cases/million, with a peak incidence of 0.74 cases/million in persons 20-24 years of age. […] Age-adjusted incidence rates for males and females were 0.4 and 0.1 cases/million, respectively (P 0.001). […] Age-adjusted incidence rates for Caucasians, African-Americans, American Indians, and Asian Pacific Islanders were 0.2, 0.5, 0.3, and 0.1 cases/million, respectively, with a statistically significant difference between Caucasians and African-Americans (P = 0.037).

• #61 Desmoplastic Small Round Cell Tumor: A Review of Main Molecular Abnormalities and Emerging Therapy

  https://www.mdpi.com/2072-6694/13/3/498

  In a study published in 2014, a total of 192 cases of DSRCT were identified in the SEER database between 1973 to 2007. The age-adjusted incidence rate based on this analysis was 0.3 cases/million, with a peak incidence of 0.74 in individuals 20–24 years-old. There is a predominance of DSRCT in males. The age-adjusted incidence rates for males and females were 0.4 and 0.1 cases/million, respectively (p < 0.001). There is predominance in African–American individuals and it is more common in males. The age-adjusted incidence rates are higher among African-Americans as compared to Caucasians (0.5 × 0.2, p = 0.037, respectively). Out of 192 cases, the common primary sites of disease were the peritoneum or soft tissue of abdomen and pelvis (42%) and less common primary sites included the ovary/fallopian tube (6 cases), orbit (1 case), cerebellum (1 case), and cerebral ventricle (1 case).

• #62 Enzalutamide induces cytotoxicity in desmoplastic small round cell tumor independent of the androgen receptor | Communications Biology

  https://www.nature.com/articles/s42003-024-06003-0

  Desmoplastic Small Round Cell Tumor (DSRCT) is a rare, pediatric cancer caused by the EWSR1::WT1 fusion protein. DSRCT predominantly occurs in males, which comprise 80-90% of the patient population. While the reason for this male predominance remains unknown, one hypothesis is that the androgen receptor (AR) plays a critical role in DSRCT and elevated testosterone levels in males help drive tumor growth. […] DSRCT survival remains poor with a 5-year survival rate of 15-25% necessitating the development of novel therapeutic strategies. […] The reason for DSRCT’s male predominance remains unknown and has the potential to provide insight into DSRCT biology and lead to the development of novel therapies. […] This study is the first comprehensive evaluation of DSRCT xenograft tumor seeding and demonstrates that DSRCT xenografts can be reliably formed with far fewer cells than previously utilized.

• #63 Desmoplastic Small Round Cell Tumor: A Review of Main Molecular Abnormalities and Emerging Therapy

  https://www.mdpi.com/2072-6694/13/3/498

  Despite multimodal treatment, DSRCT has a poor prognosis, and approximately 60–70% of patients die due to disease progression usually within 3 years after diagnosis. The median overall survival varies between 28–60 months, with a median disease-free survival between 10–15.5 months. One study proposed that the absence of extra-peritoneal metastasis, complete surgical resection and postoperative WAP-RT are factors predictive of 3-year overall survival. The multimodality treatment combining chemotherapy, cytoreductive surgery, HIPEC and WAP-RT can warrant local control of the disease, but patients will still present distant metastasis during follow-up, meaning that more effective chemotherapy is necessary to improve long-term outcomes. […] DSRCT is characterized by poor response to conventional chemotherapy and early relapse after radical surgery. Second-line treatment is ineffective in most cases. In our cohort, out of 19 patients treated with first-line chemotherapy, 13 received the second-line and the progression-free survival was only 3.9 months. This short survival time highlights the aggressiveness of this disease and the challenge in developing new therapeutic strategies to treat these young patients. Despite the development of new regimens for Ewing Sarcoma and other soft tissue and bone sarcoma in recent years, DSRCT is underrepresented or were not included in the trials that lead to the drug approval. As a result, the evidence to use second-line therapy is very limited. It is paramount to develop active cooperative groups to quickly collect data and propose new strategies for the treatment of DSRCT.

• #64

  http://waocp.com/journal/index.php/apjcc/article/view/1022

  Although complete cytoreductive surgery (CRS) is the standard therapy, the microscopic residual disease is often present. […] Looking at the real-world picture, most patients with DSRCT present in the advanced stage, and hence local therapies are rarely possible. DSRCT is known to be at least somewhat chemosensitive and radiosensitive. […] The reported Median Survival of DSRCT ranges from 17 to 25 months. […] Novel treatment methods using precision oncology are essential in managing this disease. […] In conclusion, DSRCT is a rare and aggressive disease and fatal for the majority of patients at this point in time. A definite treatment protocol has not been devised owing to the uncommonness of the disease.

• #65 Desmoplastic Small Round Cell Tumors (DSRCTs) | Memorial Sloan Kettering Cancer Center

  https://www.mskcc.org/pediatrics/cancer-care/types/desmoplastic-small-round-cell-tumors-dsrcts

  Desmoplastic small round cell tumors (DSRCTs, sometimes also referred to as small blue round cell tumor) are a type of sarcoma that usually develops in the belly or pelvis. They are most often found in white teenagers and young men between age 10 and 30. DSRCTs may cause pain or a mass in the belly. They are very rare, affecting only about 20 young people in the United States each year. […] The MSK Kids team has cared for more than 100 patients with DSRCTs more than at any other hospital in the world. Our teams have published the largest reports on people with DSRCTs who have been treated with chemotherapy, surgery, radiation therapy, and immunotherapy.

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