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Primary retroperitoneal mucinous cystadenocarcinomas (PRMCs) are extremely rare tumors with limited understanding of their pathogenesis and biological behavior. We describe a case of a 50-year-old female patient who underwent surgical treatment. The patient had a history of previous surgeries for mesenteric mucinous cystadenoma, without evidence of recurrence.

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Background/aims: The aim of this study was to perform a systematic review on primary retroperitoneal cystoadenocarcinoma (PRC), which is an extremely rare disease.

Methods: According to PRISMA guidelines, all the literature about PRC from 1977 to 2015 was reviewed. Thirty articles were selected; characteristics of the patients were collected and described; time to recurrence and overall survival (OS) were investigated when available.

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Primary retroperitoneal mucinous cystoadenocarcinoma (PRMC) is an extremely rare clinical entity with about 50 cases described by the literature. Given the rarity of this pathology, the sharing of accurate available informations is  important to  improve its knowledge. We reported a case of a woman diagnosed with PRMC who received different lines of chemotherapy and radiotherapy and we also performed a review of the literature on the issue.

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Appendiceal mucocele is a term used to describe the dilatation of the vermiform appendix produced by an intraluminal accumulation of mucus. Four pathological processes have been described that may lead to an appendiceal mucocele: obturation of cecoappendiceal communication, mucosal hyperplasia, mucinous cystadenoma and mucinous cystoadenocarcinoma. The most frequent is mucinous cystadenoma, seen in 50% of appendiceal mucoceles.

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Two cases of primary retroperitoneal mucinous cystoadenocarcinoma of the ovarian type are reported. Both tumors occurred in females with bilateral normal ovaries and contained benign, borderline, and malignant mucinous epithelium. Full-thickness infiltration of the cyst wall was not found.

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