Penis & scrotum
Mesenchymal tumors
Epithelioid sarcoma

Topic Completed: 1 May 2010

Minor changes: 13 December 2019

Copyright: 2002-2019,, Inc.

PubMed Search: Epithelioid sarcoma[TIAB] penis

Alcides Chaux, M.D.
Antonio L. Cubilla, M.D.
Page views in 2019: 482
Page views in 2020 to date: 368
Cite this page: Chaux A, Cubilla AL. Epithelioid sarcoma. website. Accessed August 12th, 2020.
Definition / general
  • Mean age 34 years (range 23 - 43 years)
  • Predominantly shaft
Clinical features
  • 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
Prognostic factors
Poor prognostic factors:
  • Regional lymph node involvement, vascular invasion, local recurrence, tumor size > 5 cm, high mitotic rate and extensive necrosis
  • Proximal type is more aggressive than classic type
Case reports
  • Total or partial penectomy
Gross images


Massive destruction of body of penis

Microscopic (histologic) description
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
Microscopic (histologic) images


Characteristic nodular growth

Scrotal tumor - proximal variant:



Rhabdoid cells

Granuloma-like areas




Positive stains
Negative stains
Differential diagnosis
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 disease: may be clinically similar but no urethral obstruction, no growing or painful nodules (BJU Int 2003;91:519), fibrosis more extensive and prominent and 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
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