Department of Surgery, Andrology Unit, Azienda Ospedaliero-Universitaria di Bologna, Bologna, Italy Department of Urology, Azienda Ospedaliero-Universitaria di Bologna, Bologna, Italy.
J Sex Med. 2013 Nov;10(11):2871-4. doi: 10.1111/j.1743-6109.2012.02774.x. Epub 2012 May 22.
Epithelioid sarcoma of the penis is a slowly growing soft tissue neoplasm that rarely arises from the penis.
The aim of this case is to describe this rare pathology and to underline the importance of differential diagnosis with benign diseases such as Peyronie's disease.
We report the case of a 20-year-old man who referred the onset of a progressive left dorsolateral penile curvature (about 60°) started 3 years before. The patient was evaluated with two US examinations that revealed two sites of tunical thickening with normal hemodynamic evaluation. The physical examination demonstrated a dorsal fibrotic plaque of about 2.5 cm.
A juvenile form of Peyronie's Disease was diagnosed, and the patient was scheduled for surgical treatment (plaque's incision/excision and grafting). Intraoperative appearance showed that the great part of the left cavernous body was substituted by a very tough tissue which deeply involved the erectile tissue; intraoperative frozen section was suspicious for mesenchymal epithelioid neoplasm. In order to obtain definitive histological analysis and collect proper informed consent, we preferred to proceed with our original project, applying a dual graft (buccal mucosa and allograft dermal matrix) to cover the wide excised area. The final histological report confirmed the diagnosis of epithelioid sarcoma. Postoperative CT and MRI, at 3 and 6 months from surgery, were negative for metastases. The patient underwent radical intervention after 6 months.
Epithelioid sarcoma of the penis and Peyronie's Disease can present with similar clinical findings although they obviously entail a different clinical progress. Since the diagnosis of neoplasm can be established only by the pathologist on biopsy specimen, in cases of unusual clinical presentation of Peyronie's disease (especially in young men suffering from a fast-growing penile induration), an early histological assessment should be performed to avoid the possibility of misdiagnosis in case of this poor-prognosis disease and to assure the definitive diagnosis.
阴茎上皮样肉瘤是一种生长缓慢的软组织肿瘤，很少发生于阴茎。
本病例旨在描述这种罕见的病理学，并强调与良性疾病（如 Peyronie 病）进行鉴别诊断的重要性。
我们报告了一例 20 岁男性患者的病例，他在 3 年前开始出现渐进性左侧背侧阴茎弯曲（约 60°）。患者接受了两次超声检查，结果显示两个部位的白膜增厚，同时伴有正常的血流动力学评估。体格检查显示背侧有一个约 2.5 厘米的纤维斑块。
诊断为青少年型 Peyronie 病，患者接受了手术治疗（斑块切开/切除和移植物植入）。术中表现为左侧大部分海绵体被一种非常坚韧的组织所取代，该组织深部累及勃起组织；术中冷冻切片怀疑为间叶上皮样肿瘤。为了获得明确的组织学分析并获得适当的知情同意，我们选择继续进行我们的原始项目，应用双层移植物（颊黏膜和同种异体真皮基质）覆盖广泛切除的区域。最终的组织学报告证实了上皮样肉瘤的诊断。术后 3 个月和 6 个月的 CT 和 MRI 检查均未发现转移。患者在 6 个月后接受了根治性干预。
阴茎上皮样肉瘤和 Peyronie 病的临床表现相似，但显然有不同的临床进展。由于只有病理学家在活检标本上才能做出肿瘤的诊断，因此对于不典型的 Peyronie 病临床表现（尤其是在快速生长的阴茎硬结的年轻男性中），应尽早进行组织学评估，以避免误诊这种预后不良的疾病，并确保明确诊断。
