[Intra-abdominal desmoplastic small round cell tumor in childhood: case report and review of the literature].
Desmoplastic small round cell tumor (DSRCT) correspond to a recent clinicopathological entity, individualized in 1989 into the group of tumors with small round cells. This pathology puts ethiopathogenic, diagnostic, therapeutic and prognosis problems. Indeed, the ethiopathogenic is still unknown, diagnosis is asserted only by immuno-histochimic and cytogenetic study because of the big number of differential diagnoses and the anatomopathologic polymorphism. Its treatment is not well codified and its outcome remains dark in spite of therapeutic progress. The objective of this work is to report a personal observation of a DSRCT and to proceed to a review of the literature to clarify the epidemiological, clinical, paraclinical and therapeutic aspects of this rare tumor.
Jellouli
Mohamed
M
Service de chirurgie pédiatrique, centre hospitalier universitaire Fattouma-Bourguiba, 5000 Monastir, Tunisie.
Mekki
Mongi
M
Krichene
Imed
I
Zakhama
Abdelfattah
A
Belghith
Mohsen
M
Jouini
Riadh
R
Sahnoun
Lassaad
L
Rammeh
Soumaya
S
Harbi
Abdelaziz
A
Nouri
Abdellatif
A
D000008:Abdominal Neoplasms / Q000188:drug therapy / Q000473:pathology*; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D002648:Child; D006801:Humans; D008297:Male; D018228:Sarcoma, Small Cell / Q000188:drug therapy / Q000473:pathology*