Epithelioid sarcoma masquerading as Peyronie's disease

Abstract

A 23-year-old man presented witha firm plague-like penile mass with pain and deviationon erection, suggesting Peyronie's disease. Biopsy was erroneously interpreted asconformatory. Irradiation was the one mode of therapy which gave temporary subjective relief. Extension of the mass and focal gangrene led to rebiopsy 6 years later. Epithelioid sarcoma was present microscopically, and was also found on re-examination of theoriginal biopsy. Partial penectomy was performed, but pulmonary metastasis had alreadyoccurred. He was treated with chemotherapy and radiotherapy and is now alive with disease 2 years later. The diagnosis of possible penile sarcoma, although rare, must be considered in patients with symptons suggesting Peyronie's disease, especially if the lesion continues to enlarge.

Epithelioid sarcoma of the penis—a rare differential diagnosis of Peyronie's disease

“…21 The young age is a very important aspect as epithelioid sarcoma occurred mostly in men under 40 years. [1][2][3][5][6][7][8][9][10][11][12][13][14][15][16][17][18] However, also the pathological differential diagnosis is also difficult and includes especially the extrarenal malignant rhabdoid tumor. 22,23 The principal microscopic characteristics of epithelioid sarcoma are the distinctive nodular arrangement of the tumor cells, their tendency to undergo central degeneration and necrosis, and their epithelioid appearance and eosinophilia.…”

“…[1][2][3][4][5][6][7][8][9][10][11][12][13][14][15][16][17][18] It mainly affects younger men under 40 years. [1][2][3][5][6][7][8][9][10][11][12][13][14][15][16][17][18] It is a malignant soft tissue tumor of unclear cell type and origin. Morphologically, epithelioid sarcoma is characterized by a mixture of epithelioid and spindle cells, that are often arranged in nodular aggregates around a central necrosis.…”

“…20 Since its first description, epithelioid sarcoma has been found to occur in the extremities, the trunk, the scalp, the vulva, and the penis, respectively. [1][2][3][4][5][6][7][8][9][10][11][12][13][14][15][16][17][18][19][20] The recommended treatment is a wide resection with adequate margins, lymph node dissection, and optional adjuvant radiotherapy. 20 The best therapeutic results (Table 1) have been achieved after total or subtotal penectomy, with or without adjuvant radiation therapy.…”

“…20 The best therapeutic results (Table 1) have been achieved after total or subtotal penectomy, with or without adjuvant radiation therapy. 4,7,9,10,12,13,17,18 Prognostic data are unprecise concerning the very short follow-ups of up to 24 months, [1][2][3][4][5][6][7]9,10,[12][13][14][15][16][17][18] there have been only two observations up to 8 and 10 years, respectively. 8,11 There is a tendency for a better prognosis after excision, but since the follow-up periods in all cases are too short with regard to the formation of metastases, 8 a real prognostic statement appears to be impossible at present.…”

“…2,9,10,12,[16][17][18] Other misdiagnoses were urethral stricture, 4 infection of the glans penis. 6 In two other cases, penile epithelioid sarcoma was primarily misdiagnosed histologically as granulomatous inflammation or and fibroxanthoma.…”