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

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 gland Carcinosarcoma Sarcomatoid and sarcomatous components Adrenocortical carcinoma 

References

  1. 1.
    Barksdale SK, Marincola FM, Jaffe G (1993) Carcinosarcoma of the adrenal cortex presenting with mineralocorticoid excess. Am J Surg Pathol 17:941–945PubMedGoogle Scholar
  2. 2.
    Collina G, Maldarizzi F, Betts CM, Eusebi V (1989) Primary sarcomatoid carcinoma of the adrenal gland. First case report. Virchows Arch A Pathol Anat Histopathol 415:161–167PubMedCrossRefGoogle Scholar
  3. 3.
    Decorato JW, Gruber H, Petti M, Levowitz BS (1990) Adrenal carcinosarcoma. J Surg Oncol 45:134–136PubMedCrossRefGoogle Scholar
  4. 4.
    Fischler DF, Nunez C, Levin HS, McMahon JT, Sheeler LR, Adelstein DJ (1992) Adrenal carcinosarcoma presenting in a woman with clinical signs of virilization. A case report with immunohistochemical and ultrastructural findings. Am J Surg Pathol 16:626–631PubMedGoogle Scholar
  5. 5.
    Guarino M, Tricomi P, Giordano F, Cristofori E (1996) Sarcomatoid carcinomas: pathological and histopathogenic considerations. Pathology 28:298–305PubMedCrossRefGoogle Scholar
  6. 6.
    Lee MS, Park IA, Chi JG, Ham EK, Lee KC, Lee CW (1997) Adrenal carcinosarcoma. A case report. J Korean Med Sci 12:374–377PubMedGoogle Scholar
  7. 7.
    Okazumi S, Asano T, Ryu M, Nagashima T, Odaka M, Isono K, Nishizowa T (1987) Surgical resection of adrenal carcinoma extending into the vena cava, right atrium and ventricle: case report and review of the literature. Nippon Geka Gakkai Zasshi 88:231–238PubMedGoogle Scholar
  8. 8.
    Tauchmanova L, Colao A, Marzano L, Sparano L, Camera L, Rossi A, Palmieri G, Marzano E, Salvatore M, Pettinato G, Lombardi G, Rossi R (2004) Adrenocortical carcinomas: twelve-year prospective experience. World J Surg 28:896–903PubMedCrossRefGoogle Scholar
  9. 9.
    Travis WD, Brambilla E, Muller-Hermelink HK, Harris CC (2004) World Health Organization classification of tumours. pathology and genetics of tumours of the lung, pleura, thymus and heart. IARC Press, LyonGoogle Scholar
  10. 10.
    Van’t Sant HP, Bouvy ND, Kazemier G, Bonjer HJ, Hop WCJ, Feelders RA, de Herder WW, de Krijger RR (2007) The prognostic value of two different histopathological scoring systems for adrenocortical carcinomas. Histopathology 51:239–245PubMedCrossRefGoogle Scholar
  11. 11.
    Wajchenberg BL, Albergaria Pereira MA, Medonca BB, Latronico AC, Carneiro PC, Ferreira-Alves VA, Zerbini MC, Liberman B, Gomes GC, Kirschner MA (2000) Adrenocortical carcinoma. Clinical and laboratory observations. Cancer 88:711–736PubMedCrossRefGoogle Scholar
  12. 12.
    Weiss LM (1984) Comparative histologic study of 43 metastasizing and non-metastasizing adrenocortical tumors. Am J Surg Pathol 8:163–169PubMedGoogle Scholar
  13. 13.
    Weiss LM, Medeiros LJ, Vickety AL (1989) Pathologic features of prognostic significance in adrenocortical carcinoma. Am J Surg Pathol 13:202–206PubMedCrossRefGoogle Scholar

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

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