The long-term survival in primary retroperitoneal mucinous cystadenocarcinoma: a case report

Abstract

BackgroundPrimary retroperitoneal mucinous cystadenocarcinoma (PRMC) is extremely rare, and its biological behavior, pathogenesis, optimum treatments, and prognosis remain to be elucidated. We herein report a case of PRMC with an 80-month follow-up.Case presentationA 29-year-old woman was diagnosed with unknown retroperitoneal tumor with benign right ovarian cyst and uterine fibroids, and she underwent laparotomy. The tumor was completely resected with a subsequent histopathological diagnosis of primary retroperitoneal mucinous cystadenocarcinoma (PRMC). Eighty months after surgery, she remains recurrence-free.ConclusionPRMC is an extremely rare tumor. Only around 60 cases have so far been published in the literature. The preoperative diagnosis of PRMC is difficult, and a definitive diagnosis can usually only be made based on the findings of histopathological examinations after surgery. Presently, only radical resection is useful for both diagnostic and therapeutic purposes. The optimal long-term management after surgery is still not well established. Further studies on PRMC are therefore needed to elucidate the etiology and establish effective treatments.

<p>Laparoscopic Resection and Pre-Operative Imaging of Primary Retroperitoneal Mucinous Neoplasms: A Retrospective Case Series</p>

“…There are only 153 published cases to date, most of which are single case reports of varied quality or small case series of no more than 3 patients. 2 , 17 – 20 The estimated prevalence of PRMN among retroperitoneal neoplasms resected in our institution is 1.96%. One systematic review and one meta-analysis of PRMCa, and one meta-analysis of PRMN have presented general information on epidemiology and clinical features; the results of these prior studies are consistent with our study.…”

First Report of Retroperitoneal Mucinous Cystadenoma in a Patient with Hirsutism

“…2,9,10,24,27,31,34,46,47 It is important to note that in a comprehensive review of the English literature, there are 21 cases of borderline malignant mucinous cystadenomas and 80 cases of malignant mucinous cystadenocarcinoma. 9,[53][54][55] The presence of these other categories of cystic lesions supports the need for surgical resection to definitively diagnose and reduce the potential for future cancer development in these lesions.…”

Primary retroperitoneal mucinos cystadenoma: A case report

“…Ove invaginirane epitelne ćelije kolona formiraju ciste koje mogu da podsećaju na epitelno tkivo jajnika i prolaze kroz proces Milerove diferencijacije. Na kraju epitelno tkivo ovih cista podleže metaplaziji i razvija spektar histoloških ćelija u različitim fazama (5). Najčešće se otkriva slučajno, rutinskim pregledom ili samopalpacijom.…”

“…Pacijenti dugo nemaju simptome. Oni se uglavnom ispoljavaju kada PrMC dovoljno naraste da vrši pritisak na susedne organe ili je palpabilna na trbušnom zidu (5,15). u nekim slučajevima PrMC može da ošteti creva te se prezentuje kliničkom slikom akutnog abdomena (15).…”

“…Životno doba pacijenata sa PrMC je u rasponu od 18 do 86 godina, prosečno 44,7 godina. srednja veličina tumora iznosi 13,6 cm (3-26 cm) (5). za sada ne postoje jasni kriterijumi za postavljanje dijagnoze PrMC.…”