Desmoplastic small round cell tumor (DSRCT) is an aggressive soft tissue cancer that typically arises in serous lined surfaces of the abdominal or pelvic peritoneum, and spreads to the omentum, lymph ...
Comprehensive, easy-to-understand information about this condition
How we create this content →The documentation on desmoplastic small round cell tumor is limited due to its extreme rarity, affecting fewer than 1 in 1,000,000 individuals. This rarity restricts the scope of systematic clinical studies and comprehensive data collection. As a result, the understanding of its genetic basis and clinical management is still evolving, leaving many questions unanswered for patients and families.
The clinical features of desmoplastic small round cell tumor include lymphadenopathy (80-99%), abdominal pain (80-99%), and abdominal distention (80-99%). Other symptoms may include mediastinal lymphadenopathy (30-79%), weight loss (30-79%), nausea and vomiting (30-79%), hepatomegaly (30-79%), and ileus (30-79%). These symptoms reflect the aggressive nature of the tumor and its tendency to spread within the abdominal cavity.
To navigate your care for desmoplastic small round cell tumor, consider seeking a specialist in sarcomas or a medical oncologist with experience in treating rare tumors. Additionally, inquire about participation in clinical trials, as these may offer access to cutting-edge treatments. Resources such as the National Organization for Rare Disorders (NORD) at https://rarediseases.org can provide valuable information and support. While no patient organizations are currently identified for DSRCT, connecting with broader cancer support groups may also be beneficial.
Currently, there are 19 active clinical trials investigating various treatment approaches for desmoplastic small round cell tumor. These trials may explore novel therapies and combinations that could improve outcomes for patients. For more information on ongoing studies, you can search the ClinicalTrials.gov database at https://clinicaltrials.gov/search?cond=desmoplastic%20small%20round%20cell%20tumor.
Actionable guidance for navigating care for desmoplastic small round cell tumor
To navigate your care for desmoplastic small round cell tumor, consider seeking a specialist in sarcomas or a medical oncologist with experience in treating rare tumors. Additionally, inquire about participation in clinical trials, as these may offer access to cutting-edge treatments. Resources such as the National Organization for Rare Disorders (NORD) at https://rarediseases.org can provide valuable information and support. While no patient organizations are currently identified for DSRCT, connecting with broader cancer support groups may also be beneficial.
Consider asking your healthcare providers these condition-specific questions
Helpful links for rare disease information and support
The documentation on desmoplastic small round cell tumor is limited due to its extreme rarity, affecting fewer than 1 in 1,000,000 individuals. This rarity restricts the scope of systematic clinical studies and comprehensive data collection. As a result, the understanding of its genetic basis and clinical management is still evolving, leaving many questions unanswered for patients and families.
The clinical features of desmoplastic small round cell tumor include lymphadenopathy (80-99%), abdominal pain (80-99%), and abdominal distention (80-99%). Other symptoms may include mediastinal lymphadenopathy (30-79%), weight loss (30-79%), nausea and vomiting (30-79%), hepatomegaly (30-79%), and ileus (30-79%). These symptoms reflect the aggressive nature of the tumor and its tendency to spread within the abdominal cavity.
To navigate your care for desmoplastic small round cell tumor, consider seeking a specialist in sarcomas or a medical oncologist with experience in treating rare tumors. Additionally, inquire about participation in clinical trials, as these may offer access to cutting-edge treatments. Resources such as the National Organization for Rare Disorders (NORD) at https://rarediseases.org can provide valuable information and support. While no patient organizations are currently identified for DSRCT, connecting with broader cancer support groups may also be beneficial.
Currently, there are 19 active clinical trials investigating various treatment approaches for desmoplastic small round cell tumor. These trials may explore novel therapies and combinations that could improve outcomes for patients. For more information on ongoing studies, you can search the ClinicalTrials.gov database at https://clinicaltrials.gov/search?cond=desmoplastic%20small%20round%20cell%20tumor.
Actionable guidance for navigating care for desmoplastic small round cell tumor
To navigate your care for desmoplastic small round cell tumor, consider seeking a specialist in sarcomas or a medical oncologist with experience in treating rare tumors. Additionally, inquire about participation in clinical trials, as these may offer access to cutting-edge treatments. Resources such as the National Organization for Rare Disorders (NORD) at https://rarediseases.org can provide valuable information and support. While no patient organizations are currently identified for DSRCT, connecting with broader cancer support groups may also be beneficial.
Consider asking your healthcare providers these condition-specific questions
Helpful links for rare disease information and support
The documentation on desmoplastic small round cell tumor is limited due to its extreme rarity, affecting fewer than 1 in 1,000,000 individuals. This rarity restricts the scope of systematic clinical studies and comprehensive data collection. As a result, the understanding of its genetic basis and clinical management is still evolving, leaving many questions unanswered for patients and families.
The clinical features of desmoplastic small round cell tumor include lymphadenopathy (80-99%), abdominal pain (80-99%), and abdominal distention (80-99%). Other symptoms may include mediastinal lymphadenopathy (30-79%), weight loss (30-79%), nausea and vomiting (30-79%), hepatomegaly (30-79%), and ileus (30-79%). These symptoms reflect the aggressive nature of the tumor and its tendency to spread within the abdominal cavity.
To navigate your care for desmoplastic small round cell tumor, consider seeking a specialist in sarcomas or a medical oncologist with experience in treating rare tumors. Additionally, inquire about participation in clinical trials, as these may offer access to cutting-edge treatments. Resources such as the National Organization for Rare Disorders (NORD) at https://rarediseases.org can provide valuable information and support. While no patient organizations are currently identified for DSRCT, connecting with broader cancer support groups may also be beneficial.
Currently, there are 19 active clinical trials investigating various treatment approaches for desmoplastic small round cell tumor. These trials may explore novel therapies and combinations that could improve outcomes for patients. For more information on ongoing studies, you can search the ClinicalTrials.gov database at https://clinicaltrials.gov/search?cond=desmoplastic%20small%20round%20cell%20tumor.
Actionable guidance for navigating care for desmoplastic small round cell tumor
To navigate your care for desmoplastic small round cell tumor, consider seeking a specialist in sarcomas or a medical oncologist with experience in treating rare tumors. Additionally, inquire about participation in clinical trials, as these may offer access to cutting-edge treatments. Resources such as the National Organization for Rare Disorders (NORD) at https://rarediseases.org can provide valuable information and support. While no patient organizations are currently identified for DSRCT, connecting with broader cancer support groups may also be beneficial.
Consider asking your healthcare providers these condition-specific questions
Helpful links for rare disease information and support
Clinical profile data for this condition is not yet available. Phenotype information may still be loading below.
Research studies investigating treatments and therapies for this condition.
Active Trials
Total Trials
Data from ClinicalTrials.gov Jan 31, 2026
Consider asking your healthcare providers these condition-specific questions
European rare disease database
Genetic and Rare Diseases Info Center
AI-Generated Content: This summary was generated using AI. Content has been fact-checked. Always consult with qualified healthcare providers for medical guidance.
Kisho delivers this disease record via API, including phenotypes (HPO), genes, orphan drug designations, screening status, and PAG mapping, with version history and governance.