Peritoneal carcinomatosis from ovarian paraganglioma: Report of a rare case and systematic review of the literature

Abstract

Paraganglioma is one of the rarest neoplasms involving the ovary, with only 10 previous reports. We present a case of peritoneal carcinomatosis from primary ovarian paraganglioma and a systematic review of the literature. Clinical information was retrieved from medical records, and a systematic review of the literature was performed according to meta-analysis of observational studies in epidemiology guidelines. A 33-year-old woman presented with a 12-month 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-analogue. 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.

Paraganglioma of the Fallopian Tube Presenting as Isolated Fallopian Tube Torsion

“…The dispersed neuroendocrine system throughout the body may give rise to neoplasms with neuronal and neuroendocrine features, such as paraganglioma [ 1 , 2 ]. Half of paragangliomas arise from the organs of Zuckerkandl [ 2 ], which comprise a small mass of chromaffin cells derived from the neural crest along the aorta, while the histogenesis of a rare type of paragangliomas in the female genital organs remains controversial [ 3 , 4 , 5 , 6 , 7 , 8 , 9 ].…”

“…In paragangliomas of the ovary [ 4 , 5 , 6 , 7 ], which are most frequent in the female genital tract, potential developmental mechanisms include involvement of the ovary through the spread of paraganglioma from nearby extra-adrenal paraganglia or preexisting paraganglia within the ovarian parenchyma [ 5 ], although these hypothetical models have not yet been clarified. Alternatively, unidirectional differentiation to paraganglioma occurring within a tumor could be a rational explanation when paraganglioma of the ovary is associated with mature cystic teratoma [ 6 ].…”

“…Clinical manifestation of paragangliomas is variable because they can be either functional or nonfunctional [ 1 , 2 , 3 , 4 , 5 , 6 , 7 , 8 , 9 , 10 ]. When paragangliomas occur from extra-adrenal chromaffin cells in parasympathetic paraganglia, they are usually nonfunctional; however, paragangliomas arising from sympathetic paraganglia could possess the ability to secrete catecholamine, which produces symptoms due to catecholamine excesses, such as the classic triad of headaches, palpitations, and profuse sweating [ 1 ].…”

“…Although widely recognized [ 5 ], the malignant potential of paraganglioma is a significant concern from oncological aspects. Paraganglioma is a slowly growing tumor with a tendency to show benign disease course or low latent malignancy [ 8 ].…”

“…Paraganglioma rarely occurs in the female genital organs [ 2 ] with reported sporadic cases in the uterus [ 3 ], ovaries [ 4 , 5 , 6 , 7 ], vagina [ 8 ], and vulva [ 9 ]; however, its occurrence in the fallopian tube has never been described. Isolated fallopian tube torsion (IFTT) without the involvement of the ipsilateral ovary is a relatively rare form of adnexal torsion [ 10 ].…”