[Desmoplastic small round-cell tumor: two cases of diffuse abdominopelvic infiltration].
Desmoplastic small round-cell tumors are a rare malignant tumor that affects male children and young adults. It frequently presents as a large abdominal mass with widespread peritoneal involvement at diagnosis. We report two cases of desmoplastic small round-cell tumors, with diffuse infiltration in the abdomen and pelvis in two adult caucasian males. The first case-report is a middle-aged man and the second a young adult man with early recurrence and diffuse metastatic lymph nodes. Both tumors have a distinct morphology, a polyphenotypic differentiation and a t(11;22) (p13;q12) translocation. The prognosis remains poor and leads to death in most cases, despite surgical resection, radiotherapy and high-dose chemotherapy.
Coulibalya
B
B
Service d'Anatomie Pathologique et de Neuropathologie, CHU La Timone, 264 Rue Saint-Pierre, 13385 Marseille Cedex 05, France. bema_coulibaly@yahoo.fr
Liprandia
A
A
Le Hémon
A
A
Fernandez
C
C
Hardwigsen
J
J
Berthet
B
B
Bouvier
C
C
D000008:Abdominal Neoplasms / Q000473:pathology* / Q000628:therapy; D000328:Adult; D017024:Chemotherapy, Adjuvant; D017809:Fatal Outcome; D018222:Desmoid Tumors / Q000473:pathology* / Q000628:therapy; D006801:Humans; D008207:Lymphatic Metastasis; D008297:Male; D009361:Neoplasm Invasiveness; D010386:Pelvic Neoplasms / Q000473:pathology* / Q000628:therapy