Materiały źródłowe

• #1 The Role of Biomarkers in Adrenocortical Carcinoma: A Review of Current Evidence and Future Perspectives

  https://www.mdpi.com/2227-9059/9/2/174

  Adrenocortical carcinoma (ACC) is a rare endocrine malignancy arising from the adrenal cortex often with unexpected biological behavior. […] Despite these achievements, the 5-year mortality rate still accounts for approximately 75% to 90%, ACC is frequently diagnosed in advanced stages and therapeutic options are unfortunately limited. […] ACCs usually show aggressive biological behavior and in 40%–60% of patients there are symptoms and signs of hormonal hyperproduction. […] However, their clinical utility has not been broadly integrated and ACCs still have poor prognosis with a 5-year mortality rate of approximately 75% to 90%. […] By the time of diagnosis, most patients have loco-regional or distant advanced disease often with hormonal hypersecretion that increases morbidity or develop local recurrence and distant metastasis after surgical treatment.

• #2 Adrenal Cancer | Treatment & Management | Point of Care

  https://www.statpearls.com/point-of-care/17222

  The prognosis of adrenocortical carcinoma was previously quite poor. In the past, an average overall survival in patients with adrenocortical carcinoma was 14.5 months with a 5-year mortality rate of about 75 to 90%. Median survival for metastatic disease (stage IV) at the time of diagnosis is reported to be less than one year. Of those who had undergone treatment, about 30 to 60% survived 5-years. Furthermore, it is worth noting that only about 30% of ACC cases are curable at early stages, which further implies an overall poor prognosis of ACC. […] More recently, different studies have shown that adrenocortical carcinoma clinical presentation, survival, and prognosis is quite heterogeneous, ranging from short term survival to more long-term survival. […] Based on the ENSAT staging system, the 5-year survival rates for ACC are as follows: 66 to 82% in patients with stage I, 58 to 64% in patients with stage II, 24 to 50% in patients with stage III, and 0 to 17% in patients with stage IV.

• #3 The Role of Biomarkers in Adrenocortical Carcinoma: A Review of Current Evidence and Future Perspectives

  https://www.mdpi.com/2227-9059/9/2/174

  Adrenocortical carcinoma (ACC) is a rare endocrine malignancy arising from the adrenal cortex often with unexpected biological behavior. […] Despite these achievements, the 5-year mortality rate still accounts for approximately 75% to 90%, ACC is frequently diagnosed in advanced stages and therapeutic options are unfortunately limited. […] ACCs usually show aggressive biological behavior and in 40%–60% of patients there are symptoms and signs of hormonal hyperproduction. […] However, their clinical utility has not been broadly integrated and ACCs still have poor prognosis with a 5-year mortality rate of approximately 75% to 90%. […] By the time of diagnosis, most patients have loco-regional or distant advanced disease often with hormonal hypersecretion that increases morbidity or develop local recurrence and distant metastasis after surgical treatment.

• #4 Adrenal Cancer | Treatment & Management | Point of Care

  https://www.statpearls.com/point-of-care/17222

  The prognosis of adrenocortical carcinoma was previously quite poor. In the past, an average overall survival in patients with adrenocortical carcinoma was 14.5 months with a 5-year mortality rate of about 75 to 90%. Median survival for metastatic disease (stage IV) at the time of diagnosis is reported to be less than one year. Of those who had undergone treatment, about 30 to 60% survived 5-years. Furthermore, it is worth noting that only about 30% of ACC cases are curable at early stages, which further implies an overall poor prognosis of ACC. […] More recently, different studies have shown that adrenocortical carcinoma clinical presentation, survival, and prognosis is quite heterogeneous, ranging from short term survival to more long-term survival. […] Based on the ENSAT staging system, the 5-year survival rates for ACC are as follows: 66 to 82% in patients with stage I, 58 to 64% in patients with stage II, 24 to 50% in patients with stage III, and 0 to 17% in patients with stage IV.

• #5 Adrenal Cancer – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK546580/

  The prognosis of adrenocortical carcinoma was previously quite poor. In the past, an average overall survival in patients with adrenocortical carcinoma was 14.5 months with a 5-year mortality rate of about 75 to 90%. Median survival for metastatic disease (stage IV) at the time of diagnosis is reported to be less than one year. Of those who had undergone treatment, about 30 to 60% survived 5-years. Furthermore, it is worth noting that only about 30% of ACC cases are curable at early stages, which further implies an overall poor prognosis of ACC. […] Based on the ENSAT staging system, the 5-year survival rates for ACC are as follows: 66 to 82% in patients with stage I, 58 to 64% in patients with stage II, 24 to 50% in patients with stage III, and 0 to 17% in patients with stage IV. […] Older age older than 45 years old, distant metastasis, and incomplete resection have shown to associate with poor survival.

• #6 Adrenal Cancer | Treatment & Management | Point of Care

  https://www.statpearls.com/point-of-care/17222

  The prognosis of adrenocortical carcinoma was previously quite poor. In the past, an average overall survival in patients with adrenocortical carcinoma was 14.5 months with a 5-year mortality rate of about 75 to 90%. Median survival for metastatic disease (stage IV) at the time of diagnosis is reported to be less than one year. Of those who had undergone treatment, about 30 to 60% survived 5-years. Furthermore, it is worth noting that only about 30% of ACC cases are curable at early stages, which further implies an overall poor prognosis of ACC. […] More recently, different studies have shown that adrenocortical carcinoma clinical presentation, survival, and prognosis is quite heterogeneous, ranging from short term survival to more long-term survival. […] Based on the ENSAT staging system, the 5-year survival rates for ACC are as follows: 66 to 82% in patients with stage I, 58 to 64% in patients with stage II, 24 to 50% in patients with stage III, and 0 to 17% in patients with stage IV.

• #7

  https://link.springer.com/article/10.1007/s12020-019-01918-9

  Adrenocortical carcinoma (ACC) is a rare endocrine carcinoma with poor 5-year survival rates of 40%. […] Overall 5-year survival was 67%, and 96% vs. 26% for ENSAT stage III vs. IIIIV (p<0.0001). [...] The 5-year survival of 96% in ENSAT stage IIII compares favourably to previous studies. [...] In the present cohort, 5-year survival rates were 100%, 93%, 60% for ENSAT I, II and III, respectively. [...] However, for ENSAT stage IV, 5-year survival remains poor (11%). [...] ENSAT stage and Ki6719% predicted survival and, as previously reported, mitotane therapy was associated with better survival. [...] Mitotane therapy associates with better survival, it is thus important to tailor the treatment according to best practice, including drug concentration monitoring and adequate replacement with rather high hydrocortisone doses, as well as thyroxine and testosterone. [...] Malignancy cannot be ruled out by small tumour size only.

• #8 Efficacy and safety of radiation therapy in advanced adrenocortical carcinoma | British Journal of Cancer

  https://www.nature.com/articles/s41416-022-02082-0

  Adrenocortical carcinoma (ACC) is a rare malignancy with poor prognosis and 5-year overall survival ranging from about 80 % in patients with ACC stage I to less than 20 % in stage IV disease. […] However, also in stage IV some patients survive many years and even complete responses have been reported. […] The results in these 57 patients were quite heterogeneous, but one has to acknowledge that the majority of these patients have been treated before 2000 and the used techniques are not really comparable anymore with methods offered to patients nowadays. […] Overall, in only 11% of lesions immediate progression was diagnosed. […] Our data indicate that RT (especially when applied in adequate dosage) is of benefit for selected patients confirming what previous small studies suggested and what the recent guideline panels concluded mainly based on expert opinion. […] Overall survival in comparison to cRT20-49Gy was significantly longer for cRT50-60Gy, but not for SBRT and BT group. […] Our study clearly suggests that RT is associated with beneficial effects on clinical outcome in selected patients with advanced ACC when applied in adequate dosage.

• #9 Survival Rates for Adrenal Cancer | American Cancer Society

  https://www.cancer.org/cancer/types/adrenal-cancer/detection-diagnosis-staging/survival-by-stage.html

  Survival rates can give you an idea of what percentage of people with the same type and stage of cancer are still alive a certain amount of time (usually 5 years) after they were diagnosed. […] Keep in mind that survival rates are estimates and are often based on previous outcomes of large numbers of people who had a specific cancer, but they cant predict what will happen in any particular persons case. […] A relative survival rate compares people with the same type and stage of cancer to people in the overall population. […] The SEER database tracks 5-year relative survival rates for adrenal cancer in the United States, based on how far the cancer has spread. […] These numbers apply only to the stage of the cancer when it is first diagnosed. […] People now being diagnosed with adrenal cancer may have a better outlook than these numbers show.

• #10 Adrenal Cancer Stages and Survival Rate

  https://www.cancercenter.com/cancer-types/adrenal-cancer/stages

  Following an adrenal cancer diagnosis, the care team reviews the patient’s pathology to confirm the diagnosis and determine the stage of disease. […] Identifying the stage of cancer not only helps the care team determine a treatment plan, it also helps predict a potential prognosis. […] The five-year relative survival rate for localized adrenal cancer is about 73 percent. […] The five-year relative survival rate for regional adrenal cancer is about 53 percent. […] The five-year relative survival rate for distant adrenal cancer is about 38 percent. […] The overall five-year relative survival rate for adrenal cancer is 50 percent, according to the ACS. […] Keep in mind that the survival rate for adrenal cancer depends on a variety of factors, including the patients age, overall health and the extent of the disease, so always talk to the care team about the patients individual prognosis.

• #11 Adrenal cancer prognosis

  https://www.medicalnewstoday.com/articles/adrenal-cancer-prognosis

  Adrenal cancer is a rare cancer that originates in the adrenal glands. The prognosis varies based on the cancers stage at diagnosis. […] Survival rates vary based on the cancers aggressiveness and stage at diagnosis and can change as diagnosis treatment strategies develop. […] Adrenal cancer diagnosis usually occurs in advanced stages, which can result in a poor prognosis. The median survival is about 4 years. […] Survival rates depend on the cancers stage at diagnosis, and estimates can vary. The American Cancer Society (ACS) offers the following 5-year survival rates based on people with a diagnosis between 2012 and 2018: localized (limited to the adrenal glands): 73%, regionalized (advanced to nearby areas): 53%, distant (advanced to distant areas): 38%. […] There is a chance for a cure, but 50-80% of people, who have their tumor surgically removed, develop metastatic or recurrent disease.

• #12 Adrenal Cancer: Symptoms, Prognosis & Treatment

  https://my.clevelandclinic.org/health/diseases/25236-adrenal-cancer

  Adrenal cancer prognosis depends on the location and size of the tumor and whether it has spread to other areas of your body. […] The five-year survival rate for early-stage adrenal cancer is between 50% and 60%. This means that up to 60% of people with this disease will still be alive five years after their diagnosis. Once the cancer spreads to other areas of your body, the five-year survival rate drops to 10% to 20%.

• #13 Adrenal Cancer | Treatment & Management | Point of Care

  https://www.statpearls.com/point-of-care/17222

  In these latest studies, resection status and tumor grade have been shown to play a key role in the disease-free survival of localized ACC cases. […] European studies of ACC patients have shown that the proliferation index, Ki67, has been the single most important factor in determining the recurrence of ACC in patients following complete resection of a localized ACC tumor (R0) for stage I-III ACC patients. Further research suggests that stage I through III, younger age, and low Ki67 are positive predictors of recurrence-free survival. […] Similarly, another large European study showed that tumor grading as measured by Ki67 and Weiss score were considered important prognostic markers of overall survival in stage IV ACC patients, which further confirmed data shown by mitotic count in French studies.

• #14 Adrenal Cancer | Treatment & Management | Point of Care

  https://www.statpearls.com/point-of-care/17222

  By comparison, in adults, cortisol production, higher tumor stage (advanced stage III/stage IV) and more disordered tumor grade have been identified as negative prognostic factors in ACC with regards to overall survival and recurrence of the disease. […] Moreover, older age older than 45 years old, distant metastasis, and incomplete resection have shown to associate with poor survival. […] Recently, transcriptome analysis revealed unraveled two distinct groups of adrenocortical carcinoma, reflecting tumor proliferation (measured by mitotic counts and cell cycle genes): one group of aggressive proliferating tumors (C1A), associated with a poor prognosis and one group of less aggressive tumors (C1B), associated with a better outcome. […] The miRNA sequence, miR-195, and miR483-5p have been shown in serum to serve as a biomarker of aggressive ACC, associated with poor prognosis.

• #15 Frontiers | Identification of prognostic factors for predicting survival of patients with malignant adrenal tumors: A population-based study

  https://www.frontiersin.org/journals/oncology/articles/10.3389/fonc.2022.930473/full

  Identification of prognostic factors for predicting survival of patients with malignant adrenal tumors: A population-based study. This study aimed to identify the prognostic factors for overall survival (OS) and cancer-specific survival (CSS) in patients with malignant adrenal tumors and establish a predictive nomogram for patient survival. It was determined that gender, age, marital status, histological type, tumor size, SEER stage, surgery, and chemotherapy were prognostic factors that affected patient survival. The OS prediction nomogram contained all the risk factors, while gender was excluded in the CSS prediction nomogram. The C-index of nomogram for OS and CSS prediction was 0.773 (95% confidence interval [CI]: 0.761–0.785) and 0.689 (95% CI: 0.675–0.703) in the training set. The following clinical variables were identified as prognostic factors: age, marital status, histological type, tumor size, SEER stage, surgery, and chemotherapy. The nomogram for patients with malignant adrenal tumors contained the accurate predictive performance of OS and CSS, contributing to optimizing individualized clinical treatments. Malignant adrenal tumors are aggressive and commonly result in a poor prognosis. The worldwide estimated incidence of adult ACC is between 0.5 and 2.0 per million people annually, and that of pheochromocytoma is 1 to 2 cases per million. However, the mortality rate of ACC accounts for 0.02% to 0.2% of all cancer-related deaths, and the 5-year survival rate is less than 40% for pheochromocytoma patients. Accurate predictions for patient survival would contribute to the personalized therapy management, which prolongs survival time. Establishing an effective prognostic prediction model is necessary to assist clinicians in optimizing individualized treatments. The successful establishment of nomograms would contribute to optimizing personalized treatment and extending survival time in patients with malignant adrenal tumors.

• #16 Adrenocortical Cancer | CancerIndex

  http://www.cancerindex.org/Adrenocortical

  Adrenocortical carcinoma (ACC) is a rare and aggressive malignancy with limited therapeutic options beyond surgical resection. The prognosis is poor, particularly for metastatic disease. […] Surgery for ACC can offer a 1 in 4 chance of actual 5-year survival and a 1 in 15 chance of actual 10-year survival. Long-term survival was often achieved with repeat resection for local or distant recurrence, further underscoring the important role of surgery in managing patients with ACC. […] The prognosis was generally poor and macroscopically positive resection margins resulted in a worse prognosis. However, some patients were still alive many years following primary surgery with no sign of residual disease. […] Tumor size, Ki-67 index, stage, and resection status are important prognostic indicators of survival in ACC patients.

• #17 Adrenal Cancer – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK546580/

  High-grade adrenocortical carcinomas are enriched for mutations of TP53 and/or CTNNB1, and these mutations tend to be mutually exclusive. […] The presence of abnormal nuclear staining of TP53 correlates well with TP53 mutations and could serve as a diagnostic tool. Somatic mutations of TP53 are associated with aggressive tumors and poor outcomes. […] The prognosis for most patients is excellent. However, the late-stage disease has a much poorer prognosis.

• #18 S-GRAS score for prognostic classification of adrenocortical carcinoma: an international, multicenter ENSAT study

  https://pmc.ncbi.nlm.nih.gov/articles/PMC8679848/

  Adrenocortical carcinoma (ACC) has an aggressive but variable clinical course. Prognostic stratification based on the European Network for the Study of Adrenal Tumours stage and Ki67 index is limited. We aimed to demonstrate the prognostic role of a points-based score (S-GRAS) in a large cohort of patients with ACC. The S-GRAS score showed superior prognostic performance for both progression-free survival (PFS) and disease-specific survival (DSS), with best discrimination obtained using the individual scores (09) (C-index=0.73, R2D = 0.30, and C-index=0.79, R2D = 0.45, respectively, all P 0.01vs each component). The superiority of S-GRAS score remained when comparing patients treated or not with adjuvant mitotane. In particular, the risk of recurrence was significantly reduced as a result of adjuvant mitotane only in patients with S-GRAS 45. The prognostic performance of S-GRAS is superior to tumour stage and Ki67 in operated ACC patients, independently from adjuvant mitotane. S-GRAS score provides a new important guide for personalised management of ACC (i.e. radiological surveillance and adjuvant treatment). The 5-year survival ranges from 10 to 60%, mostly reflecting the primary tumour stage and the resection status. However, up to 60% of patients with localised tumours experience disease recurrence after radical surgery and 15% die within 2 years. Reliable prognostication after ACC resection is critical to guide frequency of follow-up, adjuvant treatment, and to more accurately counsel patients regarding long-term outcomes. The S-GRAS score provides a new important guide for personalised management of ACC (i.e. radiological surveillance and adjuvant treatment).

• #19 Prediction of Adrenocortical Carcinoma Relapse and Prognosis with a Set of Novel Multigene Panels

  https://www.mdpi.com/2072-6694/14/11/2805

  Adrenocortical carcinoma (ACC) is a rare but aggressive cancer with a high rate of fatality. Accurate prediction of cancer relapse following therapy and prognosis (fatality) is essential to improve patient management. This research aims to significantly increase this prediction capacity. We produced four multigene sets: Sig27var25, SigIQvar8, SigCmbnvar5, and SigCmbn_B. These panels have not been studied in ACC and are thus novel. Importantly, they predict ACC’s relapse and death risk with impressively high levels of accuracy. […] Effective assessment of adrenocortical carcinoma (ACC) prognosis is critical in patient management. We report four novel and robust prognostic multigene panels. Sig27var25, SigIQvar8, SigCmbnvar5, and SigCmbn_B predict ACC relapse at area under the curve (AUC) of 0.89, 0.79, 0.78, and 0.80, respectively, and fatality at AUC of 0.91, 0.88, 0.85, and 0.87, respectively.

• #20 Prediction of Adrenocortical Carcinoma Relapse and Prognosis with a Set of Novel Multigene Panels

  https://www.mdpi.com/2072-6694/14/11/2805

  Effective prediction of ACC prognosis or its clinical behavior at the time of diagnosis is critical for patient management. […] Collectively, we report novel multigene panels that stratify ACC’s relapse and fatality risks with high levels of certainty; these panels have great clinical potential in the management of ACC.

• #21 Frontiers | Cancer Stemness Associated With Prognosis and the Efficacy of Immunotherapy in Adrenocortical Carcinoma

  https://www.frontiersin.org/journals/oncology/articles/10.3389/fonc.2021.651622/full

  Cancer stemness is significantly associated with prognosis, clinical characteristics, immune cells infiltration, and the biomarkers of ICIs. […] High-mRNAsi predicted shorter overall survival (OS) and a higher metastatic trend in adrenocortical carcinoma (ACC) patients. […] The results of Kaplan-Meier survival analysis suggested that the patients in the high-mRNAsi group had shorter OS than those in the low-mRNAsi group (P < 0.01). [...] The above analyses proved that stemness-related hub genes and mRNAsi were significantly related to prognosis in ACC patients. [...] Our results indicate that the patients in low-mRNAsi groups have higher PD-L1, CTLA4, and TIGHT expression, which means the patients with low-mRNAsi are more likely to benefit from immune checkpoint inhibitors.

• #22 The Role of Biomarkers in Adrenocortical Carcinoma: A Review of Current Evidence and Future Perspectives

  https://www.mdpi.com/2227-9059/9/2/174

  The main obstacle is scarcity of effective and available treatment options. […] The most used drug for treatment of ACC is mitotane, an adrenolytic drug, a derivate from the insecticide dichlorodiphenyltrichloroethane, which was introduced in 1960 for this indication, both in an adjuvant setting and for advanced disease. […] In metastatic disease, first line systemic therapy consists of etoposide, doxorubicin, cisplatin and mitotane (EDP-M); in the future potentially liposomal EDP-M regimens with improved tolerability could be created. […] However, standard chemotherapy only has a 23% response rate. […] Despite the remarkable progresses made in understanding of the molecular signature in ACC, a major turning point in treatment success was not produced.

• #23 Adrenocortical Cancer | CancerIndex

  http://www.cancerindex.org/Adrenocortical

  Adrenocortical carcinoma is a rare cancer that often presents in an advanced stage. Not only systemic metastases but also local recurrence and peritoneal metastases prevent long-term survival in these patients. […] The clinical features suggesting a favorable outcome from this combined treatment are relative sparing of small bowel and its mesentery, absence of disease outside the abdomen, low-grade disease, or limited extent of high-grade disease. […] The 5-year survival rate for ACC was 28%. […] The characteristics of actual long-term survivors following surgical resection for ACC have not been previously reported. […] The prognosis is poor, particularly for metastatic disease.

• #24 The Role of Biomarkers in Adrenocortical Carcinoma: A Review of Current Evidence and Future Perspectives

  https://www.mdpi.com/2227-9059/9/2/174

  The aim of this review was to summarize well established and experimental biomarkers of adrenocortical cancer, including biochemical, pathohistological and molecular aspects of diseases, to analyze their utility in everyday clinical diagnostic and therapeutic practice and to discuss possible future implications. […] The ACC work-up requires a holistic multidisciplinary expert approach to a single patient since the diagnosis of ACC is not always obvious and represents a challenging task with the possibility of making severe mistakes. […] Despite novel discoveries and modern technologies, curative approaches are still limited and the unfavorable outcome has not improved over the past 40 years. […] The aim is to provide a snapshot of some of the most exciting work published in the various research areas of the journal.

• #25

  https://winshipcancer.emory.edu/cancer-types-and-treatments/adrenal-cancer/outcomes.php

  Winship Cancer Institute treats more cases of adrenal cancer than any other center in the state, and we have some of the nations best adrenal gland tumor survival rates. […] If youve been diagnosed with adrenal cancer, youre probably wondering about your adrenal cancer prognosis. After all, adrenal cancer survival rates vary widely, depending on how advanced the cancer is at the time of diagnosis. […] For example, the five-year relative survival rate for localized adrenal cancer (cancer that hasnt spread) is 74%, while the relative survival rate for adrenal cancer thats spread to distant parts of the body is 37%, according to the American Cancer Society. […] But its important to keep in mind that adrenal cancer survival rates dont tell the whole story. First, overall cancer survival rates are improving every day, thanks to new treatment options like targeted therapy. Second, adrenal cancer survival rates are highly individualized, so averages tend to be misleading. And for a rare cancer like adrenal cancer, survival rates can also vary from center to center, depending on how much experience physicians have with a particular type of cancer.

• #26

  https://winshipcancer.emory.edu/cancer-types-and-treatments/adrenal-cancer/outcomes.php

  As a result, our outcomes including survival rates, recurrence rates, and management of long-term side effects are better than most other cancer centers in the U.S. […] Research shows survival rates are up to 25% higher when starting treatment at an NCI-designated Comprehensive Cancer Center. […] When it comes to your adrenal cancer prognosis, its important to consider the side effects associated with the cancer itself as well as the treatment.

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