[Desmoplastic tumors with multiple differentiation. A new entity. Six cases].
Desmoplastic tumours with divergent differentiation are principally located in the abdomen and develop locally or regionally. They occur in adolescents or young adults and are characterized at histology by a proliferation of undifferentiated small cells surrounded by a dense stroma. Only immuno-histo-chemistry provides the diagnosis. Since their chemosensitivity is rare and often partial the outcome is usually lethal. Six new cases of this recently described entity are presented here. The authors are in favour of a multidisciplinary and aggressive management, combining intensive polychemotherapy, extensive surgical exerisis and total abdominal radiotherapy.
Bouffet
E
E
Département de Pédiatrie, Centre Léon Bérard, Lyon.
Plantaz
D
D
Frappaz
D
D
Bouvier
R
R
Pasquier
D
D
Bailly
C
C
Schaerer
R
R
Louis
D
D
Chappuis
J P
JP
Philip
T
T
D000008:Abdominal Neoplasms / Q000175:diagnosis* / Q000473:pathology / Q000628:therapy; D000293:Adolescent; D000328:Adult; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D016026:Bone Marrow Transplantation; D002648:Child; D003131:Combined Modality Therapy; D006801:Humans; D007150:Immunohistochemistry; D008113:Liver Neoplasms / Q000556:secondary; D008297:Male; D008479:Mediastinal Neoplasms / Q000556:secondary; D010534:Peritoneal Neoplasms / Q000556:secondary
Tumeurs desmoplastiques à différenciation multiple. Une nouvelle entité. Six observations.