Primary retroperitoneal mucinous cystadenocarcinoma: a rare surgico-pathological entity

Ovarian cancer (OC) is the seventh most common cancer for females in the world. Epithelial OC is the most predominant pathologic subtype (85%-90%), with five major histotypes- serous, mucinous, endometrioid, brenner and clear cell tumour. Each have three pathological subtypes ie benign, borderline and malignant. Mucinous cystadenomas and carcinomas of the ovary are well-established and common tumors. Primary retroperitoneal mucinous cystadenomas and carcinomas are being very rare and histopathogenesis of which is still uncertain. Most pathologists suggest their origin through mucinous metaplasia in a pre-existing mesothelium-lined cyst. An accurate preoperative diagnosis of these tumors is challenging due to lack of established effective diagnostic measures. Hereby presenting a 50-year-old woman, who visited to the hospital with abdominal distension and discomfort since two months. Sonography and computed tomography scans were performed and showed large predominantly cystic lesion arising from right adnexa. Patient underwent exploratory laparotomy for removal of the tumor. Histology and immunohistochemistry revealed primary retroperitoneal mucinous cystadenocarcinoma.

Primary Retroperitoneal Mucinous Cystadenocarcioma Involving the Splenic Hilum

Primary retroperitoneal mucinous cystadenocarcinoma is a rare tumor, and it is similar to its ovarian counterpart, but it is without any evidence of ovarian, pancreatic or any other extra-retroperitoneal origin. The histogenesis of this neoplasm remains uncertain. Mucinous or colemic metaplasia of the retroperitoneal mesothelium has recently been proposed as its origin. A 39-year-old woman was diagnosed with a 13-cm cystic lesion in the left retroperitoneum; this was mucinous cystadenocarcinoma and no primary tumor was identified. We report here on a primary retroperitoneal mucinous cystadenocarcinoma that involved the splenic hilum, and we include a review of literature.

A case of primary retroperioneal mucinous cystadenocarcinoma / 대한산부인과학회잡지

Primary retroperitoneal mucinous cystadenocarinoma is extremely rare with only five case reported in the literature.Primary mucinous adenocarcinoma is apparent that a primary retroperitoneal process in development results in tissue susceptible to malignants degeneration. It is impossible to clarify the precise defect in embryologic growth that allows these tumors to develop. The theory of coelomic metaplasia has gained increasing support in recent years for origin of the epithelial neoplasms of the ovary, and an invagination of the same coelomic or pertioneal epithelial layer with concurrent or subsqeunt metaplasia account for retroperitoneal mucinous neoplasmsThe preoperative course of these cysts appears indolent, yet the eoveall prognosis is poor. The role of chemotherapy in the treatment of these tumors remains undrfined. colse follow-up and aggressive management based on histologic appearance are indicated to improve the outcome of the patients with retroperitoneal mucinous cystadenocarinoam.Recently, We repoorted one case of primary retroperitoneal nucinous cytadeno -carcioma. The patient was a 32 year-old female complaing a palpable mass and discomfort in the left flank. Laparotomy revealed a huge retroperitoneal mass in the presence of bilateral normal ovaries. Histologically, it was a mucinous cystic neoplasm with flankly malignat area. No ovarian issue was found din the mass.