Oliva E, Quinn T R, Amin M B, Eble J N, Epstein J I, Srigley J R, Young R H
Department of Pathology, Harvard Medical School, Massachusetts General Hospital, Boston 02114, USA.
Am J Surg Pathol. 2000 Jun;24(6):785-96. doi: 10.1097/00000478-200006000-00003.
The clinical and pathologic features of 15 primary urethral melanomas occurring in patients (nine women and six men) age 44 to 96 years (mean age, 73 yrs) are described. In the men the tumor involved the distal urethra. In eight women it involved the distal urethra, usually the meatus; both the distal and proximal urethra were involved in one woman. The tumors were typically polypoid and ranged from 0.8 to 6 cm (mean, 2.6 cm) in maximum dimension. A vertical growth phase was present in all tumors, with a prominent nodular component in seven of them. A radial growth phase was seen in nine tumors. The depth of invasion ranged from 2 to 17 mm. The tumors had diffuse, nested, storiform, or mixed growth patterns. The neoplastic cells typically had abundant eosinophilic cytoplasm, large nuclei with prominent nucleoli, and brisk mitotic activity. Melanin pigment was seen in 12 tumors but was conspicuous in only six. At the time of diagnosis, 13 tumors were confined to the urethra and two patients had lymph node metastasis. Nine patients died of disease 13 to 56 months after initial diagnosis and treatment, and one patient had a local recurrence at 4 years and subsequently died of sepsis 1 year later. Three patients were alive and well at 11 months, 23 months, and 7 years. One patient died at the time of the initial operation, and one died of a ruptured aortic aneurysm at 3 years without evidence of melanoma at autopsy. Primary malignant melanomas of the urethra, one fifth of which are amelanotic, must be included in the differential diagnosis of a number of primary neoplasms that involve the urethra, including transitional cell carcinoma, sarcomatoid carcinoma, and sarcomas. Conventional prognostic factors, such as depth of invasion or tumor stage, do not seem to play as important a role in predicting survival as the mucosal location and the nodular growth present frequently in these tumors.
本文描述了15例原发性尿道黑色素瘤患者（9名女性和6名男性）的临床及病理特征，患者年龄在44至96岁之间（平均年龄73岁）。男性患者的肿瘤累及尿道远端。8名女性患者的肿瘤累及尿道远端，通常为尿道口；1名女性患者的肿瘤累及尿道远端和近端。肿瘤通常呈息肉状，最大直径为0.8至6厘米（平均2.6厘米）。所有肿瘤均有垂直生长期，其中7例有明显的结节成分。9例肿瘤可见放射状生长期。浸润深度为2至17毫米。肿瘤具有弥漫性、巢状、束状或混合性生长模式。肿瘤细胞通常具有丰富的嗜酸性细胞质、大核及明显的核仁，有活跃的有丝分裂活性。12例肿瘤可见黑色素沉着，但仅6例明显。诊断时，13例肿瘤局限于尿道，2例患者有淋巴结转移。9例患者在初次诊断和治疗后13至56个月死于疾病，1例患者在4年后局部复发，随后1年死于败血症。3例患者分别在11个月、23个月和7年后存活且情况良好。1例患者在初次手术时死亡，1例患者在3年后死于主动脉瘤破裂，尸检未发现黑色素瘤证据。尿道原发性恶性黑色素瘤（其中五分之一为无黑色素性）必须纳入多种累及尿道的原发性肿瘤的鉴别诊断，包括移行细胞癌、肉瘤样癌和肉瘤。传统的预后因素，如浸润深度或肿瘤分期，在预测生存方面似乎不如这些肿瘤常见的黏膜位置和结节性生长起的作用重要。
