[Desmoplastic small round-cell tumor: two cases of diffuse abdominopelvic infiltration]

Gastroenterol Clin Biol. 2008 Mar;32(3):278-81. doi: 10.1016/j.gcb.2008.01.023.
[Article in French]

Abstract

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.

Publication types

  • Case Reports
  • English Abstract

MeSH terms

  • Abdominal Neoplasms / pathology*
  • Abdominal Neoplasms / therapy
  • Adult
  • Chemotherapy, Adjuvant
  • Fatal Outcome
  • Fibromatosis, Aggressive / pathology*
  • Fibromatosis, Aggressive / therapy
  • Humans
  • Lymphatic Metastasis
  • Male
  • Neoplasm Invasiveness
  • Pelvic Neoplasms / pathology*
  • Pelvic Neoplasms / therapy