We report a rare case of a solitary primary paraganglioma arising in the mesentery, found in a 72-year-old woman who presented with abdominal pain and a palpable abdominal mass. This extra-abdominal paraganglioma developed from paraganglionic cells that travelled by vertebral migration from the root of the superior mesenteric artery. Extra-adrenal paraganglia extend anywhere from the neck down to the base of the pelvis. Ultrasonography, computed tomography (CT), and angiography showed a solid and cystic heterogeneously enhanced mass, which was fed by the superior mesenteric artery, without distant metastasis. Exploratory laparotomy revealed a large, dark, brownish-red mass in the mesentery of the ileum, which was distinct from the ileum. The mass consisted of peripherally solid areas with central hemorrhage and cystic degeneration. It was diagnosed as a paraganglioma histologically. The patient is free from recurrence of paraganglioma after 1 year of follow up. To our knowledge, this represents only the seventh case of a paraganglioma arising in the mesentery.
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http://dx.doi.org/10.1007/s00595-004-2966-3 | DOI Listing |
Cureus
December 2024
Pediatric Radiology, Detroit Medical Center, Wayne State University, Detroit, USA.
Pheochromocytoma is a catecholamine-secreting tumor that arises from the medullary chromaffin cells but can rarely be extra-adrenal in origin. We present a case of a 16-year-old female patient with uncontrolled hypertension, despite being on lisinopril and metoprolol, and associated left-sided chest pain, recurrent headaches, and an unintentional weight loss of 10 pounds in one month. Laboratory work-up showed a markedly elevated plasma metanephrine level of 4463.
View Article and Find Full Text PDFInt J Surg Case Rep
January 2025
Neurosurgery Section, Department of Surgery, The Aga Khan Hospital, P. O Box 2289, Dar Es Salaam, Tanzania.
Introduction And Importance: Paragangliomas are rare neuroendocrine tumors, typically arising from extra-adrenal chromaffin cells. Primary intra-spinal paragangliomas are uncommon, and metastatic spinal paragangliomas without paraneoplastic symptoms are even rarer. This case highlights the diagnostic challenges posed by such rare tumors.
View Article and Find Full Text PDFInt J Surg Case Rep
January 2025
Department of Thoracic surgery, National University Hospital, Damascus University, Damascus, Syria.
Introduction: Mediastinal paragangliomas are rare neoplasms arising from extra-adrenal neural crest cells, presenting as either functional or nonfunctional tumors. Clinical manifestations range from catecholamine-related symptoms to physical compression effects. Accurate recognition of these tumors is crucial for diagnosis and management due to their rarity and association with vital mediastinal structures.
View Article and Find Full Text PDFCurr Oncol Rep
January 2025
Neuroendocrine Tumour Unit, ENETS Centre of Excellence, 1st Department of Propaedeutic and Internal Medicine, Laiko Hospital, National and Kapodistrian University of Athens, Agiou Thoma 17, Athens, 11527, Greece.
Purpose Of Review: The purpose of this review is to outline the current knowledge on epidemiology, diagnosis and management of neuroendocrine neoplasms (NENs) that develop in the context of Von Hippel-Lindau (VHL) syndrome.
Recent Findings: Pancreatic NENs develop in 8-17% of VHL patients (vPNENs) and are mostly multi-focal, cystic and non-functioning. Surgical resection is recommended for vPNENS > 3 cm that exhibit higher metastatic potential or in tumors with short doubling time while in the 20% of cases with metastatic disease the HIF-2 A inhibitor belzutifan is considered a promising option.
J Cardiothorac Surg
January 2025
Department of Thoracic Surgery, The Second Xiangya Hospital of Central South University, Changsha, Hunan, P. R. China.
Background: Paragangliomas are rare neoplasms arising from extra-adrenal chromaffin cells, with mediastinal paragangliomas representing an exceptionally rare subset. This report details the surgical management of a complex mediastinal paraganglioma case, presenting with refractory hypertension and invasion of critical surrounding structures. A comprehensive review of the current literature is included to underscore existing cases, enhance clinical awareness, and share our insights and experience in the diagnosis and treatment of this challenging condition.
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