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Penis and scrotum

Other malignancies

Epithelioid sarcoma

 

Editor: Antonio Cubilla, M.D. and Alcides Chaux, M.D. (see Author/Reviewers page)

Revised: 26 May 2010, last major update May 2010

Copyright: (c) 2002-2010, PathologyOutlines.com, Inc.

 

Definition

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● High-grade soft tissue sarcoma, few cases reported in penis

● ICD-O: 8804/3

 

Terminology

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● Proximal type: rhabdoid like features, may be more aggressive (Am J Surg Pathol 1997;21:130)

● Distal type: classic features

 

Epidemiology

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● Mean age 34 years (range 23-43 years)

 

Sites

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● Predominantly shaft

 

Clinical features

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● Superficial nodule/mass in penile shaft

● May be present for several years before excision

● Less frequently has urethral stenosis, penile deviation, dysuria or erectile pain

 

Poor Prognostic factors

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● Regional lymph node involvement, vascular invasion, local recurrence, tumor size >5 cm, high mitotic rate, extensive necrosis

● Proximal type is more aggressive than classic type

 

Case reports

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● 16 year old boy (Eur Urol 2007;51:1429)

● 34 year old man with scrotal abscess (Case of the Week #69)

● 35 year old man (Pathol Int 2000;50:579)

 

Treatment

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● Total or partial penectomy

 

Gross images

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Massive destruction of body of penis

 

Micro description (Histopathology)

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Classic type

● Predominant multinodular pattern with pseudopalisading arrangement

● Nodules are composed of medium to large neoplastic cells surrounding central areas of necrosis

● Tumor cells are large and polygonal with eosinophilic cytoplasm (epithelioid appearance) mixed with plump spindle cells

● Vesicular and pleomorphic nuclei with occasionally prominent nucleoli

● Low to moderate mitotic rate

● Chronic inflammatory infiltrate along the margin of tumor nodules

 

Proximal-type variant

● Deep seated tumor with marked cellular atypia

● Some cells have rhabdoid features

● High mitotic rate

● Larger areas of necrosis

● No granuloma-type features

 

Micro images

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Characteristic nodular growth of tumor                      Various images

cells with abundant eosinophilic cytoplasm.

 

 

Site unspecified

Tightly clustered epithelioid tumor cells around the necrotic center in epithelioid sarcoma.   Remarkable epithelioid quality of tumor cells in epithelioid sarcoma. Note the diffuse tissue eosinophilia.   So-called ‘proximal variant’ of epithelioid sarcoma. Many of the tumor cells have a rhabdoid quality.   Strong keratin immunoreactivity (CAM 5.2) in epithelioid sarcoma.   

                                                                      Proximal variant    CAM 5.2

 

 

Scrotal tumor – proximal variant

         

Necrosis                  Infiltrative                Rhabdoid cells       Granuloma-like areas

 

  

EMA                           CD34

 

Positive stains

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● Keratin (CAM 5.2, AE1/AE3), EMA, vimentin

● CD99

● Often desmin, CD34, smooth muscle actin, NSE

● Occasionally HMB45 or CEA

 

Negative stains

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● CD31, S100

 

Electron microscopy images

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Electron microscopic appearance of epithelioid sarcoma. Small tonofibrils (arrow) are present in the epithelioid tumor cells. (×11,100; courtesy of Dr Robert A. Erlandson, Memorial Sloan-Kettering Cancer Center)

Small tonofibrils

 

Differential Diagnosis

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All entities below have different immunohistochemistry staining from epithelioid sarcoma

● Chronic granulomatous inflammation: no atypical epithelioid/rhabdoid cells

● Other primary penile sarcomas: no granuloma-like neoplastic nodules

● Peyronie’s disease: may be clinically similar, but no urethral obstruction, no growing or painful nodules (BJU Int 2003;91:519), fibrosis more extensive and prominent, no atypical epithelioid/rhabdoid cells (Int J Impot Res 2003;15:378)

● Sarcomatoid carcinoma: areas of squamous differentiation elsewhere, presence of PeIN in adjacent epithelia, no granuloma-like neoplastic nodules, negative for vimentin and desmin

 

Additional references

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● Stanford University

 

End of Penis and scrotum > Other malignancies > Epithelioid sarcoma

 

 

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