Primary adrenocortical sarcomatoid carcinoma: case report and review of literature. - Inserm - Institut national de la santé et de la recherche médicale Access content directly
Journal Articles Archiv für pathologische Anatomie und Physiologie und für klinische Medicin Year : 2008

Primary adrenocortical sarcomatoid carcinoma: case report and review of literature.

Abstract

Adrenocortical carcinoma (AC) mixed with a sarcoma or sarcoma-like component is exceptional, and only six cases have been detailed in the literature, three including osteo-, chondro-, or rhabdomyosarcoma components, and three others only showing a malignant spindle cell component. These histological subtypes, respectively called adrenal carcinosarcomas and sarcomatoid AC, represent poorly differentiated and extremely aggressive forms of carcinoma, with locoregional recurrence and metastases rapidly arising from the sarcomatous or sarcomatoid component, and death occurring in a few months. We report a case of AC in a 31-year-old man presenting as a nonfunctional tumor, with a histological biphasic pattern combining few areas of differentiated AC and extensive areas of sarcomatoid spindle cell proliferation. The patient died 3 months of locoregional and distant recurrences after surgery despite apparently total tumor resection and VP16-cisplatinum chemotherapy. This case underlines the necessity to identify and isolate these carcinoma's subtypes with worse prognosis and the difficulties to distinguish them from metastatic carcinomas and retroperitoneal sarcomas, in relation to the particular adrenal cortex immunoprofile. According to the World Health Organization principles of terminology, we suggest these tumors be collectively classified as "adrenal sarcomatoid carcinomas," a designation that tends to unify all carcinomas with "pleomorphic, sarcomatoid, or sarcomatous elements."

Domains

Cancer

Dates and versions

inserm-00360949 , version 1 (12-02-2009)

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Nathalie Sturm, Nabila Moulai, Marie-Hélène Laverrière, Olivier Chabre, Jean-Luc Descotes, et al.. Primary adrenocortical sarcomatoid carcinoma: case report and review of literature.. Archiv für pathologische Anatomie und Physiologie und für klinische Medicin, 2008, 452 (2), pp.215-9. ⟨10.1007/s00428-007-0536-y⟩. ⟨inserm-00360949⟩

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