Zinner syndrome合併儲精囊腺癌:案例報告與文獻分析

莊明勲、戴大堯

成功大學醫學院附設醫院 泌尿部

Zinner syndrome complicated with primary adenocarcinoma of seminal vesicle: A case report and literature review

Ming-Syun Chuang、Ta-Yao Tai

Department of Urology, National Cheng Kung University Hospital, College of Medicine, National Cheng Kung University, Tainan, Taiwan

Background: Zinner syndrome (ZS) was first described by Zinner in 1914, and it refer to the triad of mesonephric duct abnormalities, including unilateral renal agenesis, ipsilateral seminal vesicle cyst, and ejaculatory duct obstruction. ZS is a rare congenital anomaly and the symptoms were varied and nonspecific. We presented the case of ZS complicated with a malignant neoplasm of seminal vesicles.

Case presentation: A 21-year-old man with history of G6PD deficiency, presented with gross hematuria for one month. Proteinuria and microscopic hematuria have also been noticed on urinalysis for one year. No lower urinary tract symptoms nor ejaculatory discomforts were complained. He was referred to our hospital with the impression of urinary bladder tumors on the transabdominal bladder ultrasound. Abdominal computed tomography revealed absent right kidney and an enhanced cystic tumor in the pelvic cavity, suspected right seminal vesicle tumor. Cystoscopy showed a protruding mass over right trigone, with hematuria. For the malignant concern, he received robotic-assisted laparoscopic vesiculectomy and bilateral pelvic lymph nodes dissection. The tumor was capsuled with papillary and polypoid tumor on the inner lining. Microscopically, the muscle layer of the tumor is continuous to the muscle layer of seminal vesicle. Additionally, the immunohistochemistry (IHC) of the tumor was positive for cytokeratin 7 (CK7) and negative for prostate-specific antigen (PSA), Wilms' tumor 1 (WT-1), thyroid transcription factor 1 (TTF-1), calretinin, and thyroglobulin. The pathology report demonstrated primary seminal vesicle adenocarcinoma (PSVA) with papillary pattern. No lympho-vascular nor perineural invasion was identified.

Discussion: PSVA is a rare disease and it can occur at any age (range 19–90 years). The diagnosis is also difficult according to nonspecific clinical symptoms and signs. The IHC of pathology plays an important role in establishing the diagnosis. Guo et al concluded that various combination treatment of radical surgery, radiation therapy, chemotherapy and androgen deprivation therapy may benefit in the case series of PSVA. Nevertheless, the prognosis is still poor mainly due to delayed diagnosis; the mean survival is two years after the diagnosis. In our case, the treatment was challenging when concerning of fertility and erectile function. For this reason, precise vesiculectomy could be the most feasible treatment option.

Furthermore, Zinner syndrome associated with adenocarcinoma of seminal vesicles was first described in 1992, and only five cases were reported from 1999 to 2020. However, relatively high incidence of malignancy was reported in ZS. Further investigation is necessary to identify how ZS may be a stimulating factor for malignancy