A Rare Abdomino-Pelvic Tumor: Paraganglioma

Abstract

RESUMOOs paragangliomas são tumores raros com uma incidência descrita de 2-8 por milhão. São tumores de células cromafins do sistema neuroendócrino que provêm dos gânglios simpáticos ou parassimpáticos. Apresentamos um caso de uma jovem de 32 anos, nulípara referenciada à nossa Unidade de Infertilidade. No decurso da investigação, na ecografia abdomino-pélvica identificámos a presença de uma massa que se estendia da pélvis ao abdómen, desde ovário direito até junto dos vasos renais, cujo ponto de partida era extra-ovárico (foram identificados os dois ovários com características morfológicas normais). A ressonância magnética revelou uma massa anexial multiquística e muito vascularizada junto aos vasos ilíacos, tendo colocado em hipótese origem ovárica. Desta forma, procedemos a uma laparotomia exploradora e encontrámos uma massa encapsulada, muito vascularizada que se estendia desde o bordo inferior da artéria renal direita até ao ligamento largo direito. A massa foi totalmente removida e a histologia foi consistente com um Paraganglioma. Palavras-chave: Neoplasias Abdominais; Neoplasias Pélvicas; Paraganglioma. ABSTRACTParagangliomas are rare tumors, with a reported incidence of 2-8 per million. They are chromaffin cell tumors that develop from the neural crest cells and may be divided in tumors derived from the parasympathetic or sympathetic ganglia. We report a case a of a 32-year-old nulliparous woman, referred to our Infertility Clinic. Abdomino-pelvic ultrasound identified a large abdominopelvic tumor, without ovarian origin (both ovaries were identified and had normal morphology). Magnetic Resonance Imaging suggested a right adnexal multicystic, vascularized mass close to iliac vessels and questioning an ovarian origin. At exploratory laparotomy, a 10 cm encapsulated and vascularized mass was found beginning just below right renal artery and extending to the level of the broad ligament. This mass was totally excised and histopathology was consistent with Paraganglioma.

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

“…Of the remaining 445 articles, 46 were excluded as in non‐English language, 128 were excluded as laboratory research/animal model, 190 were excluded as clinical studies but not involving paraganglioma/pheochromocytoma and 66 were excluded as they were including paraganglioma/pheochromocytoma not involving ovary or peritoneum. Six articles described cases of paraganglioma initially misdiagnosed as of primary ovarian origin but then found to be extra‐ovarian primary after surgical treatment . One case has been excluded as describing a case of peritoneal carcinomatosis from extra‐adrenal paraganglioma in a male patient .…”

An unusual presentation of Mesenteric Paraganglioma

“…Only 5-10% of chromaffin cell Tumors are extra-adrenal [1]. Thus, paragangliomas are rare Tumors, with an annual incidence of around 2-8 per million [4].…”

Asymptomatic Presacral Paraganglioma: Management of an Unpredictable Intraoperative Finding

“…2 Paragangliomas occur most commonly in the head and neck region, but can be found anywhere along the sympathetic chain. 3 Symptoms of catecholamine secretions such as hypertension, hyperhidrosis, and hyperglycemia can be present. 4 While only 15% of the patients remain asymptomatic, patients are typically diagnosed during imaging conducted for other medical purposes.…”

“…4 While only 15% of the patients remain asymptomatic, patients are typically diagnosed during imaging conducted for other medical purposes. 3 Presacral paragangliomas are rarer still and pose a diagnostic and therapeutic challenge due to their obscure anatomical location and the difficulty of performing an R0 resection. 5 We present a rare case report of an asymptomatic presacral mass causing an unpredictable acute hypertensive crisis intraoperatively, which is unmanageable with medical treatment.…”