Primary Retroperitoneal Cystoadenocarcinoma: A Systematic Review

Abstract

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. Results: Thirty seven patients were included of whom 33 were females; the median age at presentation was 43. PRC was more common in postmenopausal women. Surgery was the standard therapy; the role of chemotherapy and/or radiotherapy was uncertain. Thirty percent of the patients relapsed after 58 months from the surgery; the rupture of the cyst occurred in 13% of the cases and it was associated with poor prognosis as well as premenopausal status. At 125 months from the diagnosis, 72% of the patients were alive and the median OS was not reached. Conclusions: The present systematic review about PRC is the first performed until the date of drafting this paper. We described some clinical features of PRC and their possible prognostic value. No conclusive data can be presented due to the small population analyzed and to publication bias.

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

“…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. 2 , 3 , 21 In previous studies, serum tumour markers such as CEA, CA 125, and CA 199 were reportedly elevated in a small portion of patients, but it has been demonstrated that they have neither diagnostic nor prognostic significance, as is the case with pre-operative biopsy. 2 , 3 In our series, not surprisingly, these aforementioned diagnostic tools exhibited poor diagnostic efficacies.…”

Management of Primary Retroperitoneal Mucinous Tumors: A Retrospective Meta-Analysis

“…20 Our finding, however, is also consistent with the results from 2 recent metaanalyses on PRMT-Cs. 3,21 Because symptoms are mostly nonspecific they do not contribute to a correct diagnosis. The presence of symptoms did not differentiate benign and malignant disease.…”

Primary retroperitoneal mucinous tumors

“…Conform unei revizuiri sistemice a literaturii de către B.Pellegrino și coaut. [38] a constatat că 46% din observațiile CACMR provin din țările asiatice.…”

“…Principiul de bază al tratamentului chirurgical este îndepărtarea completă a tumorii fără a-i încălca integritatea, în principal prin abord laparotomic [37,38,39,40,41,42,43,44,49]. Cu toate acestea, rupturi tumorale intraoperatorii au fost observate în 13% din cazuri [38].…”

“…Principiul de bază al tratamentului chirurgical este îndepărtarea completă a tumorii fără a-i încălca integritatea, în principal prin abord laparotomic [37,38,39,40,41,42,43,44,49]. Cu toate acestea, rupturi tumorale intraoperatorii au fost observate în 13% din cazuri [38]. Rolul disecției ganglionilor limfatici în tratamentul chirurgical al CACMR nu a fost stabilit și se realizează după principiul case-by-case [50].…”