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Clinical information was retrieved from medical records, and a systematic review of the literature was performed according to meta\u2010analysis of observational studies in epidemiology guidelines. A 33\u2010year\u2010old woman presented with a 12\u2010month history of hypertension and weight loss. She was diagnosed with ovarian paraganglioma and was treated with extensive debulking surgery to no residual disease after three cycles of neoadjuvant chemotherapy. She recurred after 6 months and was started on somatostatin\u2010analogue. Following further disease progression with bone metastasis (treated with palliative radiotherapy), a trial with Sunitinib was started. The patient died 30 months after initial diagnosis. Of the cases reported to date, only one had peritoneal metastasis at presentation but none of them had such an ominous prognosis. Ovarian paraganglioma is an extremely rare condition. We report the first case of primary malignant ovarian paraganglioma with an exceptionally aggressive behavior. Clinicopathological correlation with immunohistochemistry is essential to avoid misdiagnosis. A standard treatment is not recommended yet but cytoreductive surgery seems to be a favorable approach to prolong survival.<\/jats:p>","DOI":"10.1111\/jog.13713","type":"journal-article","created":{"date-parts":[[2018,7,6]],"date-time":"2018-07-06T08:35:22Z","timestamp":1530866122000},"page":"1682-1692","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":3,"title":["Peritoneal carcinomatosis from ovarian paraganglioma: Report of a rare case and systematic review of the literature"],"prefix":"10.1111","volume":"44","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-1727-904X","authenticated-orcid":false,"given":"Nicol\u00f2","family":"Bizzarri","sequence":"first","affiliation":[{"name":"Northern Gynaecological Oncology Centre Queen Elizabeth Hospital  Gateshead UK"},{"name":"Academic Unit of Obstetrics and Gynecology Policlinico San Martino  Genoa 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