Carcinosarcoma

Adrenal gland and paraganglia
Adrenocortical carcinoma
Carcinosarcoma

Author: Pallav Gupta, M.D. (see Authors page)

Revised: 18 January 2016, last major update September 2013

Copyright: (c) 2002-2016, PathologyOutlines.com, Inc.

PubMed Search: carcinosarcoma adrenal

Table of Contents

Cite this page: Carcinosarcoma. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/adrenocorticalcarcinomacarcinosarcoma.html. Accessed July 27th, 2017.

Definition / general

Defined as malignant neoplasms showing both epithelial and mesenchymal differentiation with heterologous features including rhabdomyoblastic, chondroid, or osteogenic differentiation
Within the broad category of sarcomatoid carcinoma, tumors with histological areas of both carcinoma and sarcoma containing differentiated sarcomatous elements, such as malignant cartilage, bone or skeletal muscle are subclassified as carcinosarcoma

Epidemiology

Ages 29 to 79 with a mean age of 53, which appears similar to conventional adrenocortical carcinoma (mean age 40-50 years old)
No gender preference (conventional adrenocortical carcinoma has female predominance) (Diagn Pathol 2010;5:51)

Clinical features

Initial clinical presentation is usually flank / abdominal pain or discomfort (Diagn Pathol 2010;5:51)
Two of 9 (22%) tumors were functional with production of dehydroepiandrosterone sulfate or aldosterone
Mean postoperative survival 5 months

Radiology description

Radiographically and even grossly, often difficult to confirm adrenal origin due to the advanced presentation

Case reports

29 year old woman with virilization and rhabdomyosarcomatous elements (Am J Surg Pathol 1992;16:626)
31 year old man with primary adrenocortical sarcomatoid carcinoma (Virchows Arch 2008;452:215)
45 year old man with sarcoma arising in oncocytic adrenal cortical carcinoma (Am J Surg Pathol 2012;36:470)
61 year old Korean man with flank pain (J Korean Med Sci 1997;12:374)
75 year old woman with abdominal pain (Pathol Res Pract 2010;206:59)
79 year old man with osteoid differentiation and hyperaldosteronism (Am J Surg Pathol 1993;17:941)

Treatment

Surgical resection, often combined with systemic adjuvant chemotherapy

Gross description

Tumors tend to be very large at presentation (mean size 13.0 cm, weight 1113 grams)
Cut surface: heterogenous gray to pink-yellow friable

Gross images

Image hosted on other servers:

A. Tumor adherent to pancreas
B. Tumor compressing kidney

Microscopic (histologic) description

By definition, has an epithelioid component and a pleomorphic/spindled component
Epithelioid component: sheet and nests of loosely cohesive polygonal cells with clear and eosinophilic cytoplasm resembling adrenocortical cells
Pleomorphic/spindled component: predominantly spindle-shaped cells arranged in fascicular pattern with highly pleomorphic nuclei with dense irregularly clumped chromatin and prominent dense eosinophilic nucleoli

Microscopic (histologic) images

Images hosted on other servers:

Various images

Positive stains

Carcinomatous component: inhibin, MelanA, synaptophysin, calretinin
Sarcomatous component: vimentin; variable myogenin, desmin, myoglobin, caldesmon, SMA

Negative stains

Pancytokeratin, EMA, Hepar1, chromogranin

Molecular / cytogenetics description

Negative by FISH or PCR for CDK4 and MDM2 (seen in liposarcoma)

Differential diagnosis

Retroperitoneal malignancies including other carcinomas, particularly renal cell carcinoma, true sarcoma, large cell lymphoma, and metastases