Artiles Medina Alberto, Fraile Poblador Agustín, Hevia Palacios Manuel, Carretero Barrio Irene, Reguero María Eugenia, Rodríguez-Patrón Rodríguez Rafael, Burgos Revilla Francisco Javier
Department of Urology, Hospital Universitario Ramón y Cajal, Madrid, Spain.
Instituto Ramón y Cajal de Investigación Sanitaria (IRYCIS), Madrid, University of Alcalá, Madrid, Spain.
Transl Androl Urol. 2021 Sep;10(9):3697-3703. doi: 10.21037/tau-21-277.
Epithelioid hemangioendothelioma (EHE) is a rare vascular tumour with an intermediate behaviour between benign hemangioma and malignant angiosarcoma. There is scarce data on the penile EHE's management and its natural history, as our knowledge is based on few reported cases with a short follow-up period. We present a case report and conducted a literature review, including 17 cases. The relevance of this case report derives from the need for better clinical characterization of patients with penile EHE and the importance of defining the outcomes. We report the case of a 53-year-old male with a 1-year history of sleep-related painful erections. Imaging techniques showed a well-defined hypoechoic and hypervascular solid nodule on the dorsal aspect of the penis. It was surgically removed, and the histopathological study revealed a low-risk EHE of the penis. Follow-up magnetic resonance imaging (MRI) and computed tomography did not demonstrate local recurrence nor metastases. According to the literature review, most of the patients were in their fifth and sixth decades of life at the time of diagnosis and lesions were usually located in the glans. The most common clinical presentation was as a painful mass. Follow-up period ranged from 2 months to 5 years. Three patients showed systemic metastases, two of which died due to cancer. The conclusions from the literature review are limited by the reduced number of cases and the short follow-up. This case report highlights the importance of understanding the diagnosis and treatment of this type of rare non-squamous malignant tumours of the penis. Penile EHE is a malignant vascular tumour that is very rare in this location. The best treatment is local excision, with re-excision or intraoperative margins assessment. Occasionally, systemic chemotherapy and radiation therapy can be useful. There is consensus on the importance of very strict follow-up of these patients.
上皮样血管内皮瘤（EHE）是一种罕见的血管肿瘤，其生物学行为介于良性血管瘤和恶性血管肉瘤之间。关于阴茎EHE的治疗及自然病程的数据稀缺，因为我们的认知基于少数报道病例且随访期较短。我们呈现一例病例报告并进行了文献综述，共纳入17例病例。该病例报告的意义在于需要对阴茎EHE患者进行更好的临床特征描述以及明确预后的重要性。我们报告一例53岁男性，有1年与睡眠相关的阴茎疼痛勃起病史。影像学检查显示阴茎背侧有一个边界清晰的低回声、高血供实性结节。该结节经手术切除，组织病理学研究显示为低风险阴茎EHE。随访磁共振成像（MRI）和计算机断层扫描未显示局部复发或转移。根据文献综述，大多数患者在诊断时处于第五和第六个十年，病变通常位于龟头。最常见的临床表现为疼痛性肿块。随访期从2个月到5年不等。3例患者出现全身转移，其中2例死于癌症。文献综述的结论因病例数量减少和随访期短而受限。本病例报告强调了了解此类罕见的阴茎非鳞状恶性肿瘤诊断和治疗的重要性。阴茎EHE是一种在该部位非常罕见的恶性血管肿瘤。最佳治疗方法是局部切除，并进行再次切除或术中切缘评估。偶尔，全身化疗和放射治疗可能有用。对这些患者进行非常严格的随访的重要性已达成共识。
