復發性尿道腺癌的大範圍根除性手術及皮瓣重建 – 病例報告

蘇楷森¹、林子平^1,2、黃志賢^1,2 

¹臺北榮總泌尿部；²國立陽明交通大學書田泌尿科學研究中心

Extensive Radical Surgery and Flap Reconstruction for

Recurrent Urethra Adenocarcinoma: A Case Report

Kai-Sen Su¹, Tzu-Ping Lin ^1,2, William J. Huang^1,2

¹ Department of Urology, Taipei Veterans General Hospital;

² Department of Urology, School of Medicine and Shu-Tien Urological Science Research Center, National Yang Ming Chiao Tung University

Introduction:

Urethra adenocarcinoma is a rare neoplasm that accounts for less than 1% of all genital tract malignancies in female patients. The disease is associated with poor prognosis and aggressive features, tend to metastatic spread both locally and distantly. Female urethral adenocarcinoma often requires extensive surgical debulking and warrant multimodal therapies. We hereby present a case of urethra adenocarcinoma, who underwent anterior pelvic exenteration surgery with ileal conduit as primary surgery, and modified radical vulvectomy + partial vaginectomy + pubic bone resection + double barrel ileostomy + end colostomy + myocutaneous rotation flap for local recurrence.

Case report:

This is a 68-year-old female with a history of duplex uterine status post (s/p) hysterectomy + bilateral salpingo-oophorectomy at the age of 42-year-old. She presented with dysuria with urinary retention was noted in early 2020. Suprapubic cystostomy was done at ER of other hospital. She then went to ShuTien Urology Clinic for help, where cystourethroscopy was done and revealed neogrowth over the bladder neck and proximal urethra, with nearly complete obstruction. Cystoscopic biopsy yielded primary adenocarcinoma of the urethra. She was then referred to our hospital for further management.

Initial staging workup revealed locally advanced urethral cancer with puborectalis muscle involvement, cT4N0M0. Under the impression of locally advanced urethra adenocarcinoma, anterior pelvic exenteration was done on 2020/11, which included radical cystectomy, urethrectomy, ileal conduit reconstruction, and total vaginectomy by gynecologist. Pathology report yield urethra adenocarcinoma with vaginal wall invasion, pT4N2, with negative resection margin. The patient resumed normal activity after surgery. Adjuvant radiotherapy toward surgical bed and pelvis was done with total of 12000 Grays. Adjuvant chemotherapy of cisplatin stopped prematurely due to intolerance of the patient.

However, local recurrence occurred in 2021/9 as yellowish discharge from vaginal stump . Whole body simultaneous positron emission tomography and magnetic resonance imaging (PET/MRI) yield hypermetabolism at the exenteration surgical bed No abnormal uptake was noted elsewhere in the whole-body scan. Since radiation and systemic therapy is not suggested at multi-discipline team, salvage surgery is planned with involvement of multi-surgery discipline including urology, gynecology, colorectal surgery, plastic surgery and orthopedic surgeon.

Radical vulvectomy + partial pubic bone excision + proctosigmoidectomy + ileostomy + myocutaneous flap reconstruction by Gracilis muscle was conducted on 2021/11/12.  Pathology report yield moderately differentiated adenocarcinoma involving the soft tissue beneath clitoris mucosa and urethra, and also the soft tissue adjacent to bone, with the cut margins free of tumor. Patient recovered from surgery fairly.

Conclusions:

Urethra adenocarcinoma is a rare malignancy with aggressive disease characteristics. This patient presented as a medical emergency necessitated suprapubic cystostomy, which is detrimental to her disease control. Despite the invasive nature of the disease and the complexity of the anatomical structure that was involved by the tumor, radical surgery can be achieved by thorough planning and close cross-specialties communication and collaboration.