尿道黑色素瘤病例分享及最新治療回顧

楊佩珊 張慧朗 陳建綸 侯鎮邦 林友翔

林口長庚醫院 外科部 泌尿科

Urethral Melanoma Case Presentation and Review on Latest Guidelines

Pei Shan Yang, Phei Lang Chang, Chien-Lun Chen, Chen-Pang Hou, Yu-Hsiang Lin

Divisions of Urology, Department of Surgery, Chang Gung Memorial Hospital, Linkou, Taiwan

Purpose:

Melanoma can occur at any organ of the body, pathology can be categorized into cutaneous and mucosal origin.  Urethra melanoma is categorized as mucosal melanoma, which comprises of four domains, including the upper digestive tract, conjunctiva, ano-rectal and uro-genital tracts.  Ano-uro-genital (AUG) mucosal melanoma is a rare entity with grim prognosis and limited scientific evidence. We presented a case of urethral melanoma and a comparison review on the anorecatal, vulvovaginal and penile melanoma with the latest guidelines.

Materials and Methods:

 Our patient was a 72-year-old female presented with 2-3cm reddish purple urethral mass, denied of other irritative symptoms. She underwent a surgical site-specific resection and a second resection to ensure negative margins. She then later chose undergoing immunotherapy and has been following up closely for 6 months without recurrence.

Results

Bleeding, pigmentation and irritative symptoms depending on the tumor location are the most common presentations in mucosal melanomas. MRI of pelvis and brain are usually required as imaging studies, proctoscopy shall be done in ano-rectal origin and cystoscopy is carried out in vaginal and urethral origin. Molecular testing of BRAF, c-kit, RAS family, NF2 and rarely in GNAQ and GNA11 are suggested. Currently there is no evidence of overall survival benefits of radical surgeries, hence other options such as radiotherapy, systemic therapy and close follow up shall be offered as alternatives.

Conclusions:

Melanoma is a rare malignancy with grave prognosis. We shared a case of urethral melanoma with local excision and follow up.  A comparison review of the latest guideline was presented including presentation, investigation, surgery, adjuvant therapy, immunotherapy, metastatic disease and follow up.