Combined resection and multi-agent adjuvant chemotherapy for intra-abdominal desmoplastic small round cell tumour: case report and review of the literature.
Desmoplastic small round cell tumor (DSRCT) is a rare, highly aggressive malignancy with distinctive histological and immunohistochemical features occurring in young population with male predominance. We report a case of DRSCT occurred in a 17 years old patient which presented with a large upper left quadrant abdominal mass that was treated with a very aggressive surgical approach and multi-agent chemotherapy. At a 12 months follow-up he is free of recurrence. This kind of tumour has a very poor prognosis. No standard treatment protocol has been established. Aggressive surgery combined with postoperative multi-agent adjuvant chemotherapy is justified not only to relieve symptoms but also to try to improve the outcome.
Talarico
F
F
Azienda Unitá Sanitaria Locale Bologna, Ospedale "SS Salvatore", Bologna.
Iusco
D
D
Negri
L
L
Belinelli
D
D
D000293:Adolescent; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use*; D018288:Carcinoma, Small Cell / Q000188:drug therapy / Q000601:surgery; D017024:Chemotherapy, Adjuvant; D003609:Dactinomycin / Q000008:administration & dosage; D004317:Doxorubicin / Q000008:administration & dosage; D018222:Desmoid Tumors / Q000000981:diagnostic imaging / Q000188:drug therapy* / Q000601:surgery*; D006801:Humans; D007069:Ifosfamide / Q000008:administration & dosage; D008297:Male; D012186:Retroperitoneal Neoplasms / Q000000981:diagnostic imaging / Q000188:drug therapy* / Q000601:surgery*; D014057:Tomography, X-Ray Computed; D016896:Treatment Outcome; D014750:Vincristine / Q000008:administration & dosage
D003609:Dactinomycin; D014750:Vincristine; D004317:Doxorubicin; D007069:Ifosfamide