8806/3 Desmoplastic small round cell tumor


Definitions

Retroperitoneum and peritoneum
ICD-O-3 topography code: C48
ICD10: C48
           

An aggressive malignant neoplasm of uncertain lineage. It is characterized by the presence of small round cells in a desmoplastic stroma. It usually affects children and young adults. The most common site of involvement is the abdomen. Patients usually present with abdominal distention, pain, ascites, and a palpable abdominal mass. The prognosis is usually poor.



Connective, subcutaneous and other soft tissues
ICD-O-3 topography code: C49
ICD10: C49
           

An aggressive malignant neoplasm of uncertain lineage. It is characterized by the presence of small round cells in a desmoplastic stroma. It usually affects children and young adults. The most common site of involvement is the abdomen. Patients usually present with abdominal distention, pain, ascites, and a palpable abdominal mass. The prognosis is usually poor.



Testis/Male genital organs
ICD-O-3 topography code: C62-C63
ICD10: C62-C63
           

An aggressive malignant neoplasm of uncertain lineage. It is characterized by the presence of small round cells in a desmoplastic stroma. It usually affects children and young adults. The most common site of involvement is the abdomen. Patients usually present with abdominal distention, pain, ascites, and a palpable abdominal mass. The prognosis is usually poor.



Pancreas
ICD-O-3 topography code: C25
ICD10: C25
           

An aggressive malignant neoplasm of uncertain lineage. It is characterized by the presence of small round cells in a desmoplastic stroma. It usually affects children and young adults. The most common site of involvement is the abdomen. Patients usually present with abdominal distention, pain, ascites, and a palpable abdominal mass. The prognosis is usually poor.

These tumours can occur adjacent to the pancreas. They are small round blue cell tumours characterized by a distinctive desmoplastic stroma 1
 
Hruban RH, Pitman MB, and Klimstra DS
Tumors of the Pancreas. Armed Forces Institute of Pathology
Washington, DC 2007



2
Click to access Pubmed
Ryan A, Razak A, Graham J, Benson A, Rowe D, Haugk B, Verrill M (2007)
Desmoplastic small round-cell tumor of the pancreas.
J Clin Oncol 25: 1440-2



. They have divergent differentiation, and express keratins, desmin, and neuron-specific enolase (NSE) 3
 
Hruban RH, Pitman MB, and Klimstra DS
Tumors of the Pancreas. Armed Forces Institute of Pathology
Washington, DC 2007



. Unlike PNETs, desmoplastic small round cell tumours do not strongly express CD99. These tumours typically harbour the EWSR1-WT1 gene fusion4
 
Bosman FT, Carneiro F, Hruban RH, Theise ND (Eds.)
WHO Classification of Tumours of the Digestive System.
4th Edition
International Agency for Research on Cancer: Lyon 2010



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