案例報告:原發性膀胱黏液腺癌
林巧文1 張延驊12 黃志賢12
1臺北榮民總醫院 泌尿部 2國立陽明交通大學醫學院泌尿學科、書田泌尿科學研究中心
Primary Mucinous Adenocarcinoma of Urinary Bladder:
A Case Report and Literature Review
Chyau-Wen Lin1, Yen-Hwa Chang123, William J. Huang123
1 Department of Urology, Taipei Veterans General Hospital;
2 Department of Urology, College of Medicine and Shu-Tien Urological Research Center,
National Yang Ming Chiao Tung University
Introduction:
Primary mucinous is a cell type of bladder cancer, with aggressive clinical behavior and poor response to traditional chemotherapy and radiotherapy.(1) Surgery is the mainstay of treatment that provides the only curative option. Herein, we report a case of primary mucinous adenocarcinoma of bladder, with similar immunohistochemistry to colon carcinoma, who underwent radical cystoprostatectomy and subsequent adjuvant therapy with FOLFOX-4 regimen.
Case Presentation:
A 42-year-old Asian male without any previous medical condition was presented with incidental finding of elevated CEA (38.8 ng/ml) and bilateral obstructive nephropathy. Abdominal CT revealed bilateral hydroureteronephrosis with obstruction level at ureterovesical junction. Infiltrative tumor in prostate, seminal vesicle, bladder neck and posterior bladder wall was also noted. Bilateral PCND for urine diversion was done and TURBT was performed. Diffuse polypoidal, hypervascular broad-based tumor was noted extending from prostate to trigone of bladder, obliterating bilateral ureteral orifices. The pathology report from both bladder and prostate revealed mucinous adenocarcinoma with abundant extracellular mucin production. Tumor cells are immunoreactive for CDX2 and CK20, while negative for GATA3 and CK7. These features are compatible with primary bladder adenocarcinoma while metastasis from colorectal origin cannot be ruled out. Low GI endoscopy was negative.
Under the impression of primary mucinous adenocarcinoma of bladder, radical cystoprostatectomy and extended PLND with ileal conduit was performed. The patient had an uneventful recovery and was discharged with normal renal function and CEA decreased to 10.1 ng/ml.
The final pathology shows pT4 N1 (1/36 +) mucinous adenocarcinoma of bladder with abdundant extracellular mucin production that directly invade perivesical soft tissue, periprostatic soft tissue, prostate and bilateral seminal vesicles. IHC stain results remains the same as the previous TURBt specimen. All the surgical margins, including bilateral ureter, urethral and seminal vesicle are free of tumor. Only one of the right obturator lymph nodes shows metastatic tumor cells.
The patient was transferred to medical oncologist for adjuvant FOLFOX-4 regimen chemotherapy as recommended by the multidisciplinary (MDP) tumor bords discussion.
Discussion:
The majority of primary mucinous adenocarcinoma of bladder originates from urachus(2)The histological feature of this malignancy was extracellular mucin mixed with collections of tumor cells, which was also observed in our case. Hematuria and lower urinary symptoms were often the initial presentations of the disease.(3) It was often associated with schistosomiasis and congenital bladder exstrophy. Patients were often diagnosed in advanced stage with metastasis. FOLFOX-4 (fluorouracil, leucovorin, oxaliplatin) regimen had been reported to have a complete durable response.(4, 5)
Conclusion:
We presented a rare case of locally advanced primary bladder mucinous adenocarcinoma who underwent curative intent surgery and received subsequent treatment for his node positive disease. A complete history taking, gastrointestinal tract survey and a comprehensive immunohistochemical stains might provide insight to this relative difficult diagnosis.