Virchows Archiv

, Volume 452, Issue 2, pp 215–219

Primary adrenocortical sarcomatoid carcinoma: case report and review of literature

  • Nathalie Sturm
  • Nabila Moulai
  • Marie-Hélène Laverrière
  • Olivier Chabre
  • Jean-Luc Descotes
  • Elisabeth Brambilla
Case Report

DOI: 10.1007/s00428-007-0536-y

Cite this article as:
Sturm, N., Moulai, N., Laverrière, MH. et al. Virchows Arch (2008) 452: 215. doi:10.1007/s00428-007-0536-y

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.”

Keywords

Adrenal glandCarcinosarcomaSarcomatoid and sarcomatous componentsAdrenocortical carcinoma

Copyright information

© Springer-Verlag 2007

Authors and Affiliations

  • Nathalie Sturm
    • 1
    • 4
  • Nabila Moulai
    • 1
  • Marie-Hélène Laverrière
    • 1
  • Olivier Chabre
    • 3
  • Jean-Luc Descotes
    • 2
  • Elisabeth Brambilla
    • 1
  1. 1.Department of PathologyCHU Albert MichallonGrenobleFrance
  2. 2.Department of SurgeryCHU Albert MichallonGrenobleFrance
  3. 3.Department of EndocrinologyCHU Albert MichallonGrenobleFrance
  4. 4.Département d’Anatomie et de Cytologie PathologiquesCHU Albert MichallonGrenoble Cedex 9France