Szabó Balázs, Szűcs Miklós, Horváth András, Székely Eszter, Pánczél Gitta, Liszkay Gabriella, Holló Péter, Wikonkál Norbert, Nyirády Péter
Urológiai Klinika, Semmelweis Egyetem, Általános Orvostudományi Kar Budapest, Üllői út 78/B, 1082.
II. Patológiai Intézet, Semmelweis Egyetem, Általános Orvostudományi Kar Budapest.
Orv Hetil. 2019 Mar;160(10):378-385. doi: 10.1556/650.2019.31303.
Both primary and metastatic cases of mucosal melanoma in urogenital localization are rare tumors. Only 4-5% of all primary melanomas do not arise from the skin. Extracutaneous melanomas have a complex clinical presentation, but these aggressive tumors have a poor prognosis.
In our department, we found 7 patients with malignant melanoma of the genitourinary tract in the past few years. The 7 cases were: primary amelanotic melanoma of the female urethra, a primary melanoma of the bladder, two primary melanomas of the penis, a metastatic melanoma of the urethra and another to the testis and a metastatic melanoma of the bladder with melanuria. We retrospectively analyzed the available data to describe the presentation, management, and clinical outcome of the patients.
In the three inoperative cases, palliative, urologic surgical procedures and systemic antitumor therapy were performed. Two of the four primary urogenital tumors were localized to the penis. In one case, local recurrence developed after surgical treatment, but with a radical, repeated surgery, the patient has been asymptomatic for a year and a half. In the other, neglected case, the penis melanoma spread through the urethra and the inguinal lymph nodes two years after radical surgery and inguinal block dissection. In the female primary urethral melanoma case, the first histological study reported a primary mesenchymal tumor, and the recurrent tumor that occurred one and a half years later showed melanoma diagnosis. Radical surgery performed because of urethral involvement resulted in a 5-year asymptomatic state, followed by local recurrence and distant metastasis. In the fourth case of a primary bladder melanoma, the rapid progression of the disease and the BRAF positivity of the tumor suggested that not the firstly diagnosed bladder melanoma was the primary tumor.
The occurrence of urinary tract melanoma is very rare and its discovery happens often in a disseminated state, so the expected prognosis of the cases is also poor. The most important factors for increasing therapeutic efficacy are early diagnosis and radical surgical intervention. Tumors appearing in different localizations require different urological surgical approaches. The literature recommendations for treatment are not uniform. Their prognosis is worse compared to the cutaneous melanoma, which may be due to clinical and pathological diagnostic difficulties. The latest targeted and immunotherapeutic agents can significantly improve the survival of metastatic patients. Orv Hetil. 2019; 160(10): 378-385.
泌尿生殖系统原发性和转移性黏膜黑色素瘤均为罕见肿瘤。所有原发性黑色素瘤中仅有4 - 5%并非起源于皮肤。皮肤外黑色素瘤临床表现复杂，但这些侵袭性肿瘤预后较差。
在我们科室，过去几年共发现7例泌尿生殖道恶性黑色素瘤患者。这7例病例分别为：女性尿道原发性无色素性黑色素瘤、膀胱原发性黑色素瘤、阴茎原发性黑色素瘤2例、尿道转移性黑色素瘤1例、睾丸转移性黑色素瘤1例以及伴有黑尿的膀胱转移性黑色素瘤。我们回顾性分析了现有数据，以描述患者的临床表现、治疗及临床结局。
在3例无法手术的病例中，实施了姑息性泌尿外科手术及全身抗肿瘤治疗。4例原发性泌尿生殖系统肿瘤中有2例位于阴茎。其中1例在手术治疗后出现局部复发，但经再次根治性手术，患者已无症状生存一年半。另一例被忽视的病例中，阴茎黑色素瘤在根治性手术及腹股沟淋巴结清扫术后两年，经尿道及腹股沟淋巴结扩散。在女性原发性尿道黑色素瘤病例中，首次组织学检查报告为原发性间叶组织肿瘤，一年半后复发肿瘤确诊为黑色素瘤。因尿道受累而进行的根治性手术使患者处于5年无症状状态，随后出现局部复发和远处转移。在原发性膀胱黑色素瘤的第4例病例中，疾病进展迅速且肿瘤BRAF呈阳性，提示最初诊断的膀胱黑色素瘤并非原发性肿瘤。
尿路黑色素瘤的发生极为罕见，且往往在疾病播散状态下被发现，因此这些病例的预期预后也较差。提高治疗效果的最重要因素是早期诊断和根治性手术干预。不同部位出现的肿瘤需要不同的泌尿外科手术方法。文献中关于治疗的建议并不统一。与皮肤黑色素瘤相比，其预后更差，这可能是由于临床和病理诊断困难所致。最新的靶向治疗和免疫治疗药物可显著提高转移性患者的生存率。《匈牙利医学周报》2019年；160(10): 378 - 385。
