A rare case of urachal carcinoma in a young man
- Young Kwon Hong, Haeyoun Kang1, Moon Hyung Kang, Dong Soo Park
Urachal carcinoma accounts for less than 1% of bladder cancers and approximately 40% of vesical adenocarcinomas. Historically, patients tend to have a poor prognosis, with 5-year survival rates of 6.5–61.0%. Therefore it is important to detect the disease as early as possible. Most urachal cancers have been reported at the middle or old age, and it is hard to think that urachal remnant has malignant change in a young man at the age of twenties. Here we introduce a very rare urachal cancer case in a young man and we would like to remind that urachal remnants have potential to change from benign to malignant as earlier than expected.
A 26 year old man visited our clinic with gross hematuria starting a few months ago. Urinalysis showed many RBC, and CT scan showed a 4.0 x 6.8 cm sized lobulated cystic mass over the bladder dome which was located midline to the right side. The mass has calcified thick wall with suspicious contrast enhancement (Fig 1). Cystoscopy showed a ball-shape extrinsic mass from the bladder dome with intact bladder mucosa (Fig 2). With an impression of urachal cancer laparoscopic surgery was performed. Camera port was placed 5cm above the umbilicus, and two instrument ports were placed 7cm lateral and 2cm caudal to the camera port. Anterior peritoneum was dissected deep and widely from the level of umbilicus down to the UB including median umbilical ligament and part of both medial umbilical ligaments. Cystoscope was introduced from the urethra into the bladder to illuminate the margin of the mass and to facilitate keeping enough distance from the mass during laparoscopic partial cystectomy. Bladder was repaired continuously with 4-0 vicryl suture. After placing a closed suction drain the mass was wrapped into an Endo CatchTM bag and squeezed out through the extended umbilicus opening. A Foley catheter was inserted into the bladder and the port sites were closed. Cross section of the surgical specimen showed a unilocular cyst without definite solid part, filled with gray tan to brownish thick mucus material. It also showed whitish to yellowish thick wall with multifocal calcifications (Fig 3). Immunohistochemical staining was strong positive on CEA, positive on CD15 (LeuMI), and weak positive on CK7 and CK20. Final diagnosis was well differentiated mucinous urachal adenocarcinoma invading bladder muscle, pT3a based on Sheldon’s staging system, pT2b based on Mayo system, and pT2 based on Ontario system. Surgical margin was negative.
Malignant involvement of the urachus is rarely reported, one in 5 million people, accounting for 0.35% to 0.7% of all bladder cancers . It has been reported mostly in adults over 30 years old. To our best knowledge, only one adult case of urachal carcinoma diagnosed before the age of 26 has been reported in the English written literature, although two cases have been reported in 15 year-old girls [2-7]. The youngest adult case in a 22 year-old patient is found on MD Anderson cancer center experience written by Siefker-Radtke et al . According to the MSKCC study of 24 cases, two young men who was 26, 29 years old each found to have urachal cancer . The 26 year-old man received extended partial cystectomy, umbilectomy and pelvic LN dissection, and survived at only 0.9 month of follow up without long-term survival data. The 29-year-old man received extended partial cystectomy and umbilectomy, and survived at 17 months of follow up without local recurrence or metastasis. Even in a large series study, Mayo clinic study of 49 cases for 53 years, the youngest was 43 years old . Our case is an urachal adenocarcinoma found in a 26 year-old man, the second youngest adult case ever reported. Age at the diagnosis of urachal carcinoma is important to understand pathogenetic transitional mechanism from benign to malignancy. Also clinicians should be aware that urachal carcinoma may occur early in the life time at the age of twenties.
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