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Table of Contents
Definition / General | Epidemiology | Clinical Features | Radiology Description | Case Reports | Treatment | Gross Description | Gross Images | Micro Description | Micro Images | Positive Stains | Negative Stains | Molecular / Cytogenetics Description | Differential DiagnosisDefinition / 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
Micro 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
Micro 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
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