Peritoneal mucinous cystadenocarcinoma of probable urachal origin: a challenging diagnosis

Abstract

This report describes the case of a mucinous cystadenocarcinoma of probable urachal origin that presented with mass effect, precipitating deep venous thrombosis and pulmonary embolism. The patient presented with acute symptoms of leg swelling, pain and dyspnoea, and a vague awareness of lower abdominal distension. Computer tomography showed a cystic mass closely related to the anterior abdominal wall and the superior aspect of the bladder. A 1500 cm3cyst adherent to the dome of the urinary bladder was resected on laparotomy. Partial cystectomy was not carried out in the belief that the cyst represented a benign lesion. Subsequent imaging has shown cystic changes in the anterior bladder wall, and the patient has been referred for partial cystectomy.

Primary mucinous cystadenocarcinoma of the pelvic retroperitoneum in a male

“…Reported primary sources for this malignancy include ovary, bowel and pancreas, [1][2][3] but may also include bladder and urachal remnants. 4 The retroperitoneum is an extremely rare location for this cancer to develop, with Thamboo and colleagues 5 and Green and colleagues 6 having reported the only other 2 cases of retroperitoneal mucinous cystadenocarcinoma in a male.…”

Adenocarcinoma of urachal cyst associated with pseudomyxoma peritonei masquerading as abdominal tuberculosis: A case report and review of literature

“…[1–3] Gore and associates described the clinico-pathological criteria to label urachal adenocarcinoma and distinguish it from bladder or metastatic process: (1) sharp demarcation between tumor and an intact, normal surface urothelium, (2) the absence of cystitis cystica or glandularis, and (3) tumor growth in the bladder with extension in the bladder dome or anterior wall. [2] Immunohistochemically, cytokeratin 20 is expressed in more than 80% of urachal adenocarcinoma and tumor cells may express CEA and CA 19-9 with raised serum levels as tumor markers. [12] In 1901, Frankel reported a case in which PMP was found associated with a ruptured mucocele of the appendix.…”

“…[2] Immunohistochemically, cytokeratin 20 is expressed in more than 80% of urachal adenocarcinoma and tumor cells may express CEA and CA 19-9 with raised serum levels as tumor markers. [12] In 1901, Frankel reported a case in which PMP was found associated with a ruptured mucocele of the appendix. [3] The PMP is a rare and indolent disease that preferentially affects women between 50 and 70 years and appendix is now established as the primary source of most PMPs, which may then spread to other sites like ovaries.…”

“…The urachal remnants are usually lined by transitional type epithelium, but focal mucinous glandular metaplasia is often seen and may provide morphological basis for the development of intestinal-type mucinous neoplasms. [12] Pseudomyxoma peritonei (PMP) associated with mucinous cysts of the ovary was first reported by Wells. [3] The PMP arising from colon, pancreas, endometrium, breast, common bile duct and urachus carcinoma have been reported in the literature.…”

“…[3] The PMP arising from colon, pancreas, endometrium, breast, common bile duct and urachus carcinoma have been reported in the literature. [2] We report a case of mucinous cystadenocarcinoma arising in a urachal cyst associated with PMP masquerading as abdominal tuberculosis.…”