An 18-year-old Afghan man with a history of cystolithotomy seven years ago in Kabul, Afghanistan, was admitted to Shohada-e-Tajrish Hospital with the complaint of 1-year intermittent total gross painless hematuria with amorphous clots. There was no conclusive history from the patient, and no medical records could be obtained documenting such evidence. The medical and familial history were unremarkable.
Ultrasound evaluations revealed nonuniform decrease in both kidney parenchyma and bilateral hydronephrosis which was dominant in the left side (Grade III) and partial defect on the left bladder wall. Computed tomography (CT) imaging with and without intravenous contrast administration demonstrated bilateral hydronephrosis which was dominant in the left side; nonuniform decrease in the cortex of both kidneys and multiple filling defects were present in the bladder lumen without distant metastasis (Figure 1).
After clinical examination, the patient underwent a transurethral resection of the bladder tumor (TURT) that revealed a white, calcified sessile tumor extending from the left bladder wall to the dome and obstructing the left ureteral orifice. Tissues were taken by bipolar loop resection and left nephrostomy was inserted. Specimen was sent for the polymerase chain reaction to evaluate tuberculosis, because of race or ethnicity (Afghanistan) where TB is widespread, diffuse uretral stricture, nonpapillary mass, and diffuse erythema; and the results were negative.
Histological findings were consistent with poorly differentiated mixed mucinous and signet-ring cell adenocarcinoma. Immunohistochemical (IHC) studies demonstrated strong positivity for CK7, CK20, and CDX-2 and negativity for PSA, CK34betaE12, and Vimentin. Primary urinary bladder adenocarcinoma versus metastatic adenocarcinoma from gastrointestinal tract origin was suggested.
After this pathology report, hematology and radiotherapy consults suggest gastrointestinal tract workup. Esophagogastroduodenal endoscopy, rectosigmoidoscopy, and analysis of tumor markers CA 19-9 and CA 125 were performed to exclude other possible extravesical primary lesions. Transrectal needle biopsy of prostate was done and all examinations showed normal results.
After this diagnosis, the patient underwent radical cystectomy with prostate and seminal vesicle sparing technique and orthotopic diversion with "W" ileum pouch made for him, pelvic lymphadenectomy to the bifurcation of the aorta was performed, and bilateral JJ catheters were embedded in both ureters. We chose this technique because the patient was single and emphasized his future potency.
Macroscopic examination of the cystectomy specimen showed an ill-defined ulcerative and necrotic tumor mass with soft and gelatinous consistency filling the whole bladder cavity measuring 2.5 cm in maximal thickness. Microscopically, the bladder wall was infiltrated into deep muscularis propria (outer half) by a necrotic neoplasm composed of lakes of mucins containing floating nests or cords of epithelioid cells and many signet-ring cells (Figures 2(a) and 2(b)). No urachal remnant was identified. The diagnosis of signet-ring variant of mucinous adenocarcinoma of the urinary bladder was made. No metastatic lymph node was identified (T2N0M0).
Six months postoperatively, clinical examinations, chest X-rays, CT scans, and cystoscopy showed normal results without distal spread or any recurrence.