Abstract
Background: Desmoplastic small round cell tumour (DSRCT) is a rare, highly aggressive malignancy typically presenting as an abdomen mass. It usually occurs in young population with male predominance.
Aim: To report a rare case of DSRCT arising from the pleura in a 15-year old female
Methods: We present a clinical case of a young patient who presented with dyspnoea on exertion thoracic back pain and significant weight loss. Physical examination revealed decreased breath sounds of the right hemithorax. A computed tomography scan suggested extensive pleural effusion combined with a large pleural mass of the right side extending in the abdomen and paraortic lymphadenopathy. A biopsy of the mass revealed histological and immunohistochemical characteristics consistent with desmoplastic small round cell tumor. The patient received 7 courses of chemotherapy according to the P6 protocol which involves cyclophosphamide, doxorubicin and vincristine for courses 1-3 and 6, ifosfamide and etoposide for courses 4, 5 and 7.
Results: The patient obtained a partial response after completion of chemotherapy and, subsequently, she received local radiotherapy. Eight months after the initial diagnosis the patient remains in a clinically stable condition.
Conclusion: Desmoplastic small round cell tumor is an uncommon aggressive malignancy. It appears to be chemosensitive but response to treatment is only short-lasting. Current literature suggests a multidisciplinary approach including chemotherapy, radiation and surgery. However, prognosis remains poor.
- © 2011 ERS