Dept. of Radiation Oncology, Guwahati State Cancer Institute, Assam , India.
Dept. of Radiation Oncology, Kidwai Memorial Institute of Oncology, Bangalore, India.
Gulf J Oncolog. 2021 Jan;1(35):92-95.
Malignant mucosal melanoma (MM) is a rare and aggressive neoplasm, which behaves differently from its cutaneous counterpart. MM of head and neck account for 55.4%, genital tracts - 18%, ano-rectal - 23.8% and urinary tract - 2.8%. Less than 200 cases of urethral melanomas have been reported in literature, out of which MM of female urethra represent only 0.2% of primary melanomas. Owing to the delayed presentation, early onset of metastasis and aggressive tumor biology, prognosis has remained largely poor despite adequate local control in MM. Surgery is the mainstay of management, with adjuvant radiation contributing to local control but not overall survival benefit. Chemotherapy and immunotherapy options are being explored in both adjuvant and palliative setting. We hereby, report the clinical presentation, management, and course of a rare case of urethral melanoma in a young female patient. After ruling out metastatic disease at presentation, local control was achieved by wide local excision with distal urethrectomy and modified Singapore flap reconstruction along with bilateral modified superficial inguinal block dissection; followed by adjuvant radiotherapy in view of close margins. However, she developed distal recurrence three months post adjuvant therapy, thus reflecting the aggressive biology of this class of rare tumor.
恶性黏膜黑色素瘤(MM)是一种罕见且侵袭性的肿瘤,其表现与皮肤型黑色素瘤不同。头颈部 MM 占 55.4%,生殖道占 18%,肛门直肠占 23.8%,泌尿系统占 2.8%。文献中报道的尿道黑色素瘤病例不到 200 例,其中女性尿道 MM 仅占原发性黑色素瘤的 0.2%。由于出现症状较晚、早期转移和侵袭性肿瘤生物学特性,尽管 MM 局部控制良好,但预后仍很差。手术是治疗的主要方法,辅助放疗有助于局部控制,但对总体生存获益没有影响。化疗和免疫治疗方案正在辅助和姑息治疗中进行探索。我们在此报告一例年轻女性罕见尿道黑色素瘤的临床表现、治疗和病程。在排除了初诊时的转移疾病后,通过广泛局部切除联合远端尿道切除术和改良新加坡皮瓣重建术,以及双侧改良浅层腹股沟淋巴结清扫术实现了局部控制;然后鉴于边缘接近,进行了辅助放疗。然而,她在辅助治疗后三个月出现远端复发,这反映了这种罕见肿瘤的侵袭性生物学特性。