Materiały źródłowe

• #1 Desmoplastic small round cell tumors – Symptoms and causes – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/dsrct/symptoms-causes/syc-20355405

  Desmoplastic small round cell tumor symptoms vary depending on where the cancer begins. Most often it begins in the abdomen. […] Signs and symptoms of desmoplastic small round cell tumors in the abdomen include: Swelling of the abdomen, Abdominal pain, Constipation, Difficulty urinating. […] Make an appointment with your healthcare professional if you have any persistent signs and symptoms that worry you.

• #2 8 Top Desmoplastic Small Round Cell Tumors Symptoms & Signs | MD Anderson Cancer Center

  https://www.mdanderson.org/cancer-types/desmoplastic-small-round-cell-tumors/desmoplastic-small-round-cell-tumors-symptoms.html

  Desmoplastic small round cell tumors (DSRCT) usually have no early symptoms. In most cases, the first sign is a hard, round mass in the abdomen, which can grow very large before it is found. The child may feel pain in the abdomen. Because DSRCT is rare, it is often misdiagnosed by pediatricians and family doctors. […] Desmoplastic small round cell tumor (DSRCT) symptoms include: Swelling (distention) of abdomen, Back pain, Gastrointestinal blockage, Lack of appetite and weight loss, Fatigue, Fluid in the abdomen (ascites), Anemia, Thyroid or hormone problems. […] DSRCT is rare. If your child has any of these symptoms, they are most likely caused by something less serious. However, its a good idea to talk to your childs doctor, as they may signal other health problems.

• #3 Desmoplastic Small Round Cell Tumors (DSRCT)

  https://my.clevelandclinic.org/health/diseases/dsrct

  Desmoplastic small round cell tumor (DSRCT) is a rare condition where fast-growing tumors develop in the membrane lining your abdomen. […] You may not develop symptoms until tumors get large enough to affect your abdomen and digestive system. Common DSRCT symptoms include: Abdominal pain. Constipation. Diarrhea. Distended (swollen) belly. Nausea and vomiting. Unexplained weight loss. […] Treatment may put the condition into remission, meaning you dont have symptoms and tests dont find signs of disease. But remission often doesnt last, and youll probably need new or different treatment for recurring tumors. […] DSRCT often comes back. Your healthcare provider may recommend that you have imaging tests, like CT scans, every three to six months to check for new tumors in the lining of your abdomen or other areas of your body.

• #4 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

  Many people with DSRCT do not have symptoms when the cancer first starts. Later, when the tumors get larger, symptoms can include: Pain, Nausea and vomiting, Diarrhea, Constipation, Swelling in the abdomen. […] 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.

• #5 Desmoplastic small-round-cell tumor – Wikipedia

  https://en.wikipedia.org/wiki/Desmoplastic_small-round-cell_tumor

  Desmoplastic small-round-cell tumor (DSRCT) is an aggressive and rare cancer that primarily occurs as masses in the abdomen. Other areas affected may include the lymph nodes, the lining of the abdomen, diaphragm, spleen, liver, chest wall, skull, spinal cord, large intestine, small intestine, bladder, brain, lungs, testicles, ovaries, and the pelvis. […] There are few early warning signs that a patient has a DSRCT. Patients are often young and healthy as the tumors grow and spread uninhibited within the abdominal cavity. These are rare tumors and symptoms are often misdiagnosed by physicians. The abdominal masses can grow to enormous size before being noticed by the patient. The tumors can be felt as hard, round masses by palpating the abdomen. […] First symptoms of the disease often include abdominal distention, abdominal mass, abdominal or back pain, gastrointestinal obstruction, lack of appetite, ascites, anemia, and cachexia.

• #6 Desmoplastic Small Round Cell Tumor Treatment | St. Jude Care & Treatment

  https://www.stjude.org/care-treatment/treatment/childhood-cancer/solid-tumors/desmoplastic-small-round-cell-tumor.html

  Signs and symptoms of desmoplastic small round cell tumor include: […] Lump or swelling in the abdomen […] Belly pain or cramping […] Nausea […] Vomiting […] Diarrhea […] Constipation or trouble passing gas. […] DSRCT in the abdomen may not be found until the tumors have grown large. By then, the disease has often spread to the liver, lymph nodes, lungs, or bones. […] The prognosis for DSRCT depends on where the tumor is located, if the cancer has spread, and how well it can be treated with surgery. DSRCT tumors that are outside the abdomen and have not spread to other organs respond better to treatment. DSRCT in the abdomen or DSRCT that has spread to other parts of the body does not respond as well. […] The survival rate is about 15% survival at 5 years. Patients with DSRCT often experience relapse (the cancer comes back after being successfully treated).

• #7 Desmoplastic Small Round Cell Tumors | Symptoms, Diagnosis & Treatment

  https://www.cincinnatichildrens.org/health/d/desmoplastic-small-round-cell-tumors

  Desmoplastic small round cell tumors (DSRCT) are a type of rare, soft tissue sarcoma. This type of cancer usually begins in the abdomen or pelvis, and does not respond well to treatment. Most patients (90%) are Caucasian males between the ages of 10 and 30. […] DSRCT has few early warning signs. The cancer affects people who are otherwise healthy. The tumor can grow to a large size and coat the inside surface of the abdomen before a person notices it. […] The main symptom of DSRCT is a solid mass in the abdomen. Other symptoms include: Belly cramps, Continued belly pain, Constipation, Unexplained weight loss, A painful lump in the belly button. […] DSRCT almost always begins in the abdomen. In more than 40% of patients, it can spread to the liver, lymph nodes, lungs, bones and other parts of the body.

• #8 Desmoplastic small round cell tumour | Sarcoma UK

  https://sarcoma.org.uk/about-sarcoma/what-is-sarcoma/types-of-sarcoma/desmoplastic-small-round-cell-tumour/

  Desmoplastic small round cell tumour is a rare type of soft tissue sarcoma, which is a type of cancer. […] Symptoms of desmoplastic small round cell tumour can vary depending on the size and location of your tumour. […] Before you are diagnosed, you may experience all, some or none of these symptoms: A lump or swelling in the body, almost always in the abdomen. This lump can grow very quickly, and is often painful. Abdominal pain and swelling. Unexplained weight gain. Nausea. Difficulty urinating or constipation. […] After treating a previous tumour, desmoplastic small round cell tumour can come back in the same place. This is known as a local recurrence. […] A recurrence of desmoplastic small round cell tumour may be accompanied by cancer in other parts of the body. This is called metastasis or secondary cancer. Some people are diagnosed with sarcoma because their tumour has spread before the main tumour is found. Some patients may develop secondary cancers in the lungs. […] Secondary cancers may also appear in the liver and bones. If you have advanced cancer, your treatment may involve surgery, radiotherapy, or other therapies. Doctors will evaluate your treatment on a case-by-case basis.

• #9 Desmoplastic Small Round Cell Tumor Symptoms Guide – Acibadem Health Point – ACIBADEM Hospitals – Acibadem Health Group

  https://www.acibademhealthpoint.com/desmoplastic-small-round-cell-tumor-symptoms-guide/?amp=1

  The common symptoms of desmoplastic small round cell tumor include: Persistent abdominal pain; Abdominal distension or swelling; Unexplained weight loss; Nausea and vomiting; Feeling of fullness even after small meals; Fatigue and weakness; Difficulty breathing; Swollen lymph nodes; Reduced appetite; Changes in bowel habits. […] Recognizing these symptoms and seeking medical attention promptly can significantly improve the prognosis for individuals with desmoplastic small round cell tumor.

• #10 Desmoplastic small round cell tumor | About the Disease | GARD

  https://rarediseases.info.nih.gov/diseases/6265/desmoplastic-small-round-cell-tumor

  Desmoplastic small round cell tumor (DSRCT) is a rare type of soft tissue cancer (sarcoma) that usually begins in the abdomen. It primarily affects children and young adults and is more common in males. Symptoms may include abdominal pain and a feeling of fullness, abdominal mass, and symptoms of gastrointestinal obstruction, such as constipation. […] Symptoms of this disease may start to appear as a Teenager and as an Adult. […] The types of symptoms experienced, and their intensity, may vary among people with this disease. […] Symptoms may start to appear as a Teenager and as an Adult. […] The following describes the symptom(s) associated with this disease along with the corresponding body system(s), description, synonyms, and frequency: Abdominal Distention, Abdominal Pain, Anemia, Ascites, Cachexia, Hepatomegaly, Ileus, Lymphadenopathy, Nausea and Vomiting, Weight Loss.

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

  https://pmc.ncbi.nlm.nih.gov/articles/PMC7865637/

  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. […] 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. […] Patients can be asymptomatic for long periods of time until symptoms of pain, ascites, constipation, weight loss, distension and jaundice. […] In most cases, patients with an abdominal disease are diagnosed in an advanced stage, with large masses and/or extensive seeding in the visceral and parietal peritoneum. Symptoms, which are related to the tumor burden and location of the lesions, motivate investigation by image exams.

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

  https://pmc.ncbi.nlm.nih.gov/articles/PMC7865637/

  The disease predominantly originates from the peritoneum or retroperitoneum and can invade the omentum with multiple peritoneal implants involving the diaphragm, splenic hilum, mesentery of the small and large bowel, and the pelvic peritoneum. […] 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. […] DSRCT is characterized by poor response to conventional chemotherapy and early relapse after radical surgery. Second-line treatment is ineffective in most cases.

• #13 Desmoplastic Small Round Cell Tumors (DSRCT) Diagnosis

  https://tamc.co.il/en/article/desmoplastic-small-round-cell-tumors-dsrct-diagnosis/

  Desmoplastic small cell tumour is a rare disease affecting predominantly young adults and male children. Usually, a tumour occurs in the abdominal (more often) or thoracic cavity, affects the omega, gastrointestinal tract, bladder and other organs and systems. It is distinguished from other small round cell tumors by characteristic histology a cluster of blue small round or ovoid cells in a desmoplastic stroma, which is differentiated by epithelial, mesenchymal, myogenic, neural mitosis. […] The most common symptoms are abdominal pain, bloating, a sense of severity and hepatomegaly. Less common signs are retroperitoneal lymphadenopathy, hydronephrosis, bulobestruction, calcification and thickening of the nodal peritoneal lymph nodes. These symptoms are not typical and do not serve as a basis for diagnosis. Therefore, it is important to consider DSRCT as a possible diagnosis when a young person has nonspecific abdominal symptoms and there are radiological signs of disseminated intraperitoneal malignancy.

• #14 Desmoplastic_small_round_cell_tumor

  https://www.bionity.com/en/encyclopedia/Desmoplastic_small_round_cell_tumor.html

  First symptoms of the disease often include abdominal distention, abdominal mass, abdominal or back pain, gastrointestinal obstruction, lack of appetite, ascites, anemia, and/or cachexia. […] Other reported symptoms include unknown lumps, thyroid conditions, hormonal conditions, blood clotting, kidney or urological problems, testicle, breast, uterine, vaginal, or ovarian masses. […] The prognosis for desmoplastic small round cell tumor remains poor with less than 20% surviving beyond two to three years. Prognosis often depends upon the stage of the cancer and the grade of the tumor. Because the disease can be misdiagnosed or remain undetected tumors frequently grow large within the abdomen and metastasized or seed to other parts of the body. […] There is no known organ or area of origin. Desmoplastic small round cell tumor can metastasize through lymph nodes or the blood stream. Sites of metastatis include the spleen, diaphragm, liver, large and small intestine, lungs, central nervous system, bones, uterus, bladder, genitals, abdominal cavity, and the brain.

• #15 Desmoplastic Small Round Cell Tumors (DSRCT): Causes, Symptoms and Treatment Options – cancerGO

  https://cancergo.org/blog/desmoplastic-small-round-cell-tumors-dsrct-causes-symptoms-and-treatment-options/

  Desmoplastic small round cell tumors (DSRCTs) are a rare and aggressive type of soft tissue sarcoma. The tumors typically develop in the abdomen and can spread to other body parts, such as the lungs, liver, and lymph nodes. DSRCTs are often difficult to diagnose and treat and have a poor prognosis. […] The symptoms of Desmoplastic small round cell tumors (DSRCTs) can vary depending on the location and size of the tumor, but some common symptoms include: Abdominal pain or swelling, Loss of appetite, Fatigue, Nausea and vomiting, Constipation or diarrhea, Abdominal mass or lump, Blood in stool or urine, Shortness of breath (if the tumor has spread to the lungs), Jaundice (if the tumor has spread to the liver), Swelling of the legs or arms (if the tumor has spread to the lymph nodes). […] The treatment of DSRCTs is often complex and can involve a combination of surgery, chemotherapy, and radiation therapy. Surgery is typically the first treatment used, and it is used to remove as much of the tumor as possible. However, due to the aggressive nature of the tumors, complete surgical removal may not be possible, and the tumors can come back after surgery.

• #16 Desmoplastic Small Round Cell Tumor Symptoms Guide – Acibadem Health Point – ACIBADEM Hospitals – Acibadem Health Group

  https://www.acibademhealthpoint.com/desmoplastic-small-round-cell-tumor-symptoms-guide/?amp=1

  Desmoplastic small round cell tumor is a rare and aggressive cancer that primarily affects young adults and adolescents. Recognizing the symptoms of DSRCT is crucial for timely intervention and better prognosis. By understanding the common symptoms associated with this condition, you can take proactive steps towards early detection and management. […] Signs of desmoplastic small round cell tumor may include: Persistent and unexplained abdominal pain, often accompanied by swelling; A palpable lump or mass in the abdomen; Unintentional weight loss and loss of appetite; Fluid buildup in the abdomen or other areas of the body, causing swelling; Constipation, diarrhea, or changes in bowel movements; Shortness of breath or difficulty breathing due to tumor pressing on the diaphragm. […] Individuals with desmoplastic small round cell tumor (DSRCT) may experience a range of symptoms that can vary depending on the location and stage of the tumor. Early detection of these symptoms is crucial for timely diagnosis and treatment.

• #17 Hamish’s Rare Desmoplastic Small Round Cell Tumors Story

  https://thepatientstory.com/patient-stories/rare/desmoplastic-small-round-cell-tumor-dsrct/hamish-s/

  At 25, Hamish was diagnosed with desmoplastic small round cell tumors (DSRCT), a rare and aggressive sarcoma (cancer that starts in the body’s soft tissues or bones, like muscles, fat, or blood vessels). His diagnosis followed months of fatigue, weight loss, and nausea, initially dismissed as long COVID or dietary issues, until he discovered a lump in his abdomen. […] Symptoms: Persistent fatigue, nausea, unexplained weight loss, discovery of a hard abdominal lump. […] His most recent PET scan showed no evidence of disease, a moment of relief and encouragement.

• #18 What are Desmoplastic Small Round Cell Tumors (DSRCT)? Symptoms, Risk Factors & Treatments | MD Anderson Cancer Center

  https://www.mdanderson.org/cancer-types/desmoplastic-small-round-cell-tumors.html

  Desmoplastic small round cell tumors (DSRCT) are a type of soft-tissue sarcoma. DSRCT causes multiple tumors to form in the abdomen and pelvis area, but exactly where it begins usually is not known. […] Since DSRCT often is not diagnosed until the cancer is advanced, tumors grow large and spread through the lymph system or blood stream to other parts of the body. They can spread to many areas of the body, including the lymph nodes, lungs, bone and liver. Other types of cancer, including rhabdomyosarcoma, may be in the abdomen as well. […] DSRCT is a dangerous type of cancer that often is resistant to treatment and frequently comes back after treatment.

• #19 Desmoplastic small-round-cell tumor – Wikipedia

  https://en.wikipedia.org/wiki/Desmoplastic_small-round-cell_tumor

  The prognosis for DSRCT remains poor. Prognosis depends upon the stage of the cancer. Because the disease can be misdiagnosed or remain undetected, tumors frequently grow large within the abdomen and metastasize or seed to other parts of the body. […] DSRCT can metastasize through lymph nodes or the blood stream. Sites of metastasis include the spleen, diaphragm, liver, large and small intestine, lungs, central nervous system, bones, uterus, bladder, genitals, abdominal cavity, and the brain. […] A multi-modality approach of high-dose chemotherapy, aggressive surgical resection, radiation, and stem cell rescue improves survival for some patients. Reports have indicated that patients will initially respond to first line chemotherapy and treatment but that relapse is common. […] Some patients in remission or with inoperable tumor seem to benefit from long term low dose chemotherapy, turning DSRCT into a chronic disease.

• #20 Desmoplastic small round cell tumour: characteristics and prognostic factors of 41 patients and review of the literature | Clinical Sarcoma Research | Full Text

  https://clinicalsarcomaresearch.biomedcentral.com/articles/10.1186/2045-3329-3-14

  Desmoplastic small round cell tumour (DSRCT) is a rare but frequently fatal sarcoma, and many of its characteristics still require further clarification. The median age of presentation was 27 years (range 16 to 45 years), with a male-to-female ratio of 3:1. Ninety percent of patients had disease in the abdomen. The median size of the presenting tumour was 13 cm (range 3.5 to 23 cm), and 80% had metastatic disease at diagnosis, mainly in the liver (33%) and lungs (21%). Time-to-progression (TTP) was 3.9, 2.3 and 1.1 months after first-, second- and third-line chemotherapy, respectively. First-line treatment with VIDE chemotherapy appeared to confer the longest TTP (median 14.6 months). Ifosfamide and doxorubicin resulted in TTP of 3.8 months when used in any-line setting. After a median follow-up of 14 months, the overall median survival (MS) was 16 months. Patients with extra-abdominal disease survived longer compared to those with tumours in the abdomen (all still alive vs MS of 15 months; P= 0.0246). Patients with non-metastatic intra-abdominal disease who underwent surgery had an MS of 47 months (16 months for those who did not have surgery; P=0.0235). Radiotherapy for locoregional control in patients with metastatic intra-abdominal DSRCT was associated with longer survival (MS of 47 vs 14 months; P=0.0147). DSRCT is a rare but often fatal disease that mainly affects younger male patients. Those with intra-abdominal DSRCT have a poorer prognosis, although surgical resection for localised disease and radiotherapy in the metastatic setting are associated with improved survival.

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

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

  Patients can be asymptomatic for long periods of time until symptoms of pain, ascites, constipation, weight loss, distension and jaundice. Other sites of the primary tumor are described in the literature as the thoracic cavity, testicle, head and neck, intracranial, thigh, axilla/shoulder, intraosseous, uterine corpus, ovary, skull, middle ear, and others. About one-half of the patients will present extra-peritoneal metastasis at the time of the diagnosis, although this percentage was lower in the report of Stiles et al. The liver and lung are the two most common sites for distant metastasis. […] In most cases, patients with an abdominal disease are diagnosed in an advanced stage, with large masses and/or extensive seeding in the visceral and parietal peritoneum. Symptoms, which are related to the tumor burden and location of the lesions, motivate investigation by image exams. The most common imaging finding is multiple, lobulated, low-attenuated, heterogeneous peritoneal, omental and serosal soft tissue masses usually discrete, round or ovoid, without an apparent primary organ of origin. Almost all patients will present a dominant mass, mainly in the retrovesical or recto-uterine location, peritoneal or omental.

• #22 Desmoplastic Small Round Cell Tumors – Support finding a pediatric cancer cure

  https://sarcomafree.org/desmoplastic-small-round-cell-tumors/

  After initial treatment, desmoplastic small round cell tumor can reappear in the same location, known as “local recurrence.” If the cancer returns, it’s crucial to seek treatment promptly. […] Desmoplastic small round cell tumor can metastasize, meaning the cancer spreads to other parts of the body, known as “secondary cancer.” Some people receive a sarcoma diagnosis only after it has already spread. Secondary cancers often appear in the lungs, which is why chest x-rays are part of follow-up appointments. […] Secondary tumors can also occur in the liver and bones. If you have advanced cancer, treatment options may include surgery, radiotherapy, or other therapies, evaluated on a case-by-case basis by your medical team.

• #23 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.

• #24 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. 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. 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%). In case 2, the patient presented with multiple abdominal masses and combined abdominal lymph nodes and liver metastases, which are consistent with the literature mentioned above. Despite advances in multimodal therapy, patients with DSRCT have a poor prognosis, with about 60-70% dying from the disease within 2-3 years. […] 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.

• #25

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

  DSRCT still remains a clinical challenge for oncologists because of the lack of standard guidelines. […] 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. For advanced disease, symptom palliation is paramount. The reported Median Survival of DSRCT ranges from 17 to 25 months. […] 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. The current data and treatment protocols have not shown improved outcomes, thereby invoking the need for future clinical trials. Newer treatment approaches are the need of the hour in this disease with a bleak prognosis.

• #25

• #26 Orphanet: Desmoplastic small round cell tumor

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

  Clinical signs and symptoms of DSRCT are non-specific. DSRCT presents with abdominal pain, enlarged abdomen, dyspepsia, and/or vomiting and weight loss depending on the stage of the disease. Other signs can be observed such as a palpable abdominal mass, gastrointestinal occlusion, ascites, and hepatomegaly. […] Prognosis is poor. Median overall survival is 17 months and less than 20% of patients live more than 5 years after diagnosis.

• #27 Desmoplastic small round cell tumor (DSRCT) | EBSCO Research Starters

  https://www.ebsco.com/research-starters/health-and-medicine/desmoplastic-small-round-cell-tumor-dsrct

  Desmoplastic small round cell tumor (DSRCT) is a rare and aggressive type of soft tissue sarcoma that primarily affects adolescents and young adults, with a higher incidence in males. […] Common symptoms include abdominal pain, gastrointestinal issues, and weight loss, often leading to misdiagnosis, particularly in females where it may be mistaken for ovarian cancer. […] The most common symptoms include abdominal pain, abdominal mass, constipation, diarrhea, nausea, weight loss, and gastrointestinal obstruction. Most symptoms only occur after the tumors are large. […] Patients are often misdiagnosed at the onset of symptoms, and the rapid growth of the tumor cells can lead to an advanced stage, in which the cancer has spread to lymph nodes and other organs before being diagnosed. […] The prognosis of patients diagnosed with desmoplastic round cell tumors is very poor, with a five-year survival rate of 15 to 38 percent. Patients treated before the tumors spread have a much higher survival rate.

• #28

  https://link.springer.com/article/10.1007/BF02303860

  Desmoplastic small round cell tumors (DSRCT or DSCT) are rare aggressive cancers of adolescence and early adulthood. […] The primary site of disease in 97% of cases was the abdomen or pelvis. Twenty-nine patients (91%) had extensive disease involving peritoneal surfaces, lymph nodes, or discontinuous organs. […] Improved survival is correlated with a complete or very good partial response to multimodality therapy, surgical debulking of more than 90% either before or after chemotherapy, and use of the P6 protocol. […] DSRCT is an aggressive cancer that occurs predominantly in young males. Improved survival is correlated with intense chemotherapy and aggressive resection.

• #29 Reddit – The heart of the internet

  https://www.reddit.com/r/cancer/comments/14olspi/diagnosed_with_ultra_rare_sarcoma_dscrt/

  Hey everybody, Im a 23 year old who is struggling to come to terms with everything thats going on. To give a little bit of backstory I noticed that my abdomen was becoming more and more distended and I went to the local hospital where I was they told me I was constipated, to me it didnt make very much sense however I complied and they prescribed some medication to help. As expected it did nothing and I sought help elsewhere. Along the way they discovered an enormous mass in my abdomen however it seemed to not be attached to any organs and there were no lesions anywhere else so it was assumed to be some sort of benign growth. They opted for surgery right away as it was pushing my stomach and liver around. Up until this point the only symptoms I experienced apart from bloating was a small pain in my rib when sitting down. After the surgery I was informed that it was removed in its entirety and the way the surgeons described it was it popped out on its own like a balloon barely attached anywhere and completely encapsulated in other words we were thrilled. They sent off the entire mass for analysis and the results were Desmoplastic Small Round Cell Tumor (DSCRT) of the abdomen a type of very aggressive sarcoma with no known protocol of treatment. Only about 200-300 documented cases around the world.

• #30 Desmoplastic_small_round_cell_tumor

  https://www.bionity.com/en/encyclopedia/Desmoplastic_small_round_cell_tumor.html

  Desmoplastic small round cell tumor is classified as a soft tissue sarcoma. It is an aggressive and rare tumor that primarily occurs as multiple masses in the abdomen. Other areas affected may include the lymph nodes, the lining of the abdomen, diaphragm, spleen, liver, chest wall, skull, spinal cord, large intestine, small intestine, bladder, brain, lungs, testicles, ovaries, and the pelvis. Reported sites of metatastic spread include the liver, lungs, lymph nodes, brain, skull, and bones. […] The disease rarely occurs in females, but when it does the tumors can be mistaken for Ovarian cancer. […] There are few early warning signs that a patient has a desmoplastic small round cell tumor. Patients are often young and healthy as the tumors grow and spread uninhibited within the abdominal cavity. These are rare tumors and symptoms are often misdiagnosed by family physicians. The abdominal masses can grow to enormous size before being noticed by the patient. The tumors can be felt as hard, round masses by palpating the abdomen.

• #31 SciELO Brazil – Desmoplastic Small Round Cell Tumor of the Ovary: A Case Report with a New Modality of Treatment and Review of the Literature Desmoplastic Small Round Cell Tumor of the Ovary: A Case Report with a New Modality of Treatment and Review of t

  https://www.scielo.br/j/rbgo/a/3Xxpmvtt8Pdt9x46cybVkwB/?lang=en

  Desmoplastic small round cell tumor (DSRCT) is a highly malignant, rare intraabdominal neoplasm of mesenchymal origin with an extremely poor prognosis. It primarily affects young men, with a reported male to female ratio of four to one. Desmoplastic small round cell tumor has a propensity for serosal surfaces, and the majority of the patients are in the late stages of the disease upon presentation. […] The presenting symptoms of DSRCT are usually related to the site of involvement, such as crampy abdominal pain, abdominal distension with ascites, palpable mass, constipation, anorexia, or weight loss. The most common primary location of DSRCT is the peritoneal cavity, but it can be found at other sites, such as the ovary, kidney, or retroperitoneal space. […] Although the tumor markers in our case were within the reference values range, -HCG, AFP, LDH, and CA-125 should be measured in all young women who present with a pelvic mass. Elevated serum tumor marker levels may serve as an adjunct in the initial diagnosis, therapy monitoring, and posttreatment surveillance.

• #32 Desmoplastic small round cell tumors (DSRCT): overview

  https://tamc.co.il/en/article/desmoplastic-small-round-cell-tumors-dsrct-overview/

  Knowing the clinical information and characteristic cytological and histological features of the tumor, pathologists and cytopathologists can make an accurate diagnosis of DSRCT. If problems arise in establishing the correct diagnosis, additional studies can be carried out, such as cytogenetic karyotypic analysis and molecular study.

• #33 Sinonasal desmoplastic small round cell tumor: a case report and review of the literature | BMC Cancer | Full Text

  https://bmccancer.biomedcentral.com/articles/10.1186/s12885-019-6076-4

  Desmoplastic small round cell tumor (DSRCT) is a rare malignancy with poor prognosis that generally involves the peritoneum. Its diagnosis can be achieved only by immunohistochemistry and cytogenetic studies. […] The clinical manifestations of DSRCT are not typical, and patients often experience abdominal or pelvic discomfort, typically including abdominal pain and/or bloating, ascites, constipation, and urinary tract disease. […] DSRCT, which occurs in the nasal cavity and sinuses, is extremely rare, and only two cases reported in the literature were retrieved on PubMed, their clinical features, treatment and outcomes were summarized in Table 1. Its clinical manifestations are complex and varied. Generally, local symptoms occur according to different parts of the body, without its own unique clinical manifestations. If it occurs in the abdominal cavity or pelvic cavity, clinical manifestations are usually abdominal pain, abdominal distension or abdominal mass, which can be accompanied by cachexia such as fever, anemia, emaciation, and prone to substantial organs and lymph node metastasis; while in the nasal cavity and paranasal sinuses, the primary symptoms are mainly sinusitis, nosebleeds and nasal congestion, local infiltration and cervical lymph node metastasis may occur, and small round cell malignant swelling is common in this area. The clinical manifestations of the tumors were not significantly different, and it was difficult to make a definite diagnosis in clinic.

• #34 Primary desmoplastic small-round-cell tumor of the ovary | Journal of the Egyptian National Cancer Institute | Full Text

  https://jenci.springeropen.com/articles/10.1186/s43046-019-0001-4

  Desmoplastic small-round-cell tumor (DSRCT) is an extremely rare and highly aggressive malignancy. It tends to have a predilection for adolescent males with an annual incidence of about 0.1 cases per million. It usually presents as intra-abdominal masses that might mimic gastrointestinal stromal tumors (GIST) or retroperitoneal sarcoma. Its early presentation is usually non-specific, with symptoms varying between vague abdominal pain, distension, and altered bowel habits. Such a tendency to late presentation accounts for its difficult management, as it often manifests in advanced disease stage. Radiologic findings usually describe abdominal masses of variable sizes in association with peritoneal deposits, omental cakes, and ascites, findings that are usually found in the advanced colon, ovarian, and gastric cancer, hence the encountered diagnostic difficulty. The patient had a cytoreductive surgery done in the form of total abdominal hysterectomy, bilateral salpingo-oophorectomy, omentectomy, pelvic peritonectomy, low para-aortic and bilateral iliac lymphadenectomy. Postoperative pathology examination revealed tumor tissue formed of malignant cells with intervening desmoplasia. The tumor cells were small with a marked degree of atypia and pleomorphism and high mitotic activity. The patient had an insignificant postoperative course. Adjuvant chemotherapy (VACA-IE) protocol: vincristine (Oncovin), doxorubicin (Adriamycin), cyclophosphamide (Cytoxan), doxorubicin (Adriamycin), ifosfamide (Ifex), and etoposide (Vepesid) respectively were ensued soon after full recuperation. Patient is still receiving the proposed chemotherapy protocol, and she is planned to receive adjuvant external beam radiation therapy. Prognosis is still dismal with 5-year survival barely exceeding 30%. […] Given the lack of clear guidelines, treatment should be offered based on the best available evidence and the collaborative effort of a multi-disciplinary team.

• #35 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 is the initial treatment upon DSRCT diagnosis, prescribed in conjunction with other treatment modalities such as surgical debulking or radiation therapy. […] Tumor responsiveness to chemotherapy indicates whether or not the patient is a solid candidate for surgery. […] The most common first-line chemotherapy option is the P6 regimen; a mixture of vincristine, doxorubicin, cyclophosphamide, ifosfamide, and etoposide. […] Surgical management of DSRCT is attempted through cytoreductive surgery, also known as debulking surgery. […] The goal of debulking surgery is to remove the highest percentage of the tumor possible since the remaining cells can metastasize.

• #36 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.

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