A rare case of multiple paragangliomas in the head and neck, retroperitoneum and duodenum: A case report and review of the literature

A rare case of multiple paragangliomas in the head and neck, retroperitoneum and duodenum: A case report and review of the literature

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Pheochromocytomas and paragangliomas (PGLs) are rare non-epithelial neuroendocrine neoplasms of the adrenal medulla and extra-adrenal paraganglia respectively.Duodenal PGL is quite rare and there are only two previous reports.Herein, we report a case of multiple catecholamines (CAs)-producing PGLs in the middle ear, retroperitoneum, and duodenum, and review the literature of duodenal PGLs.A 40-year-old man complained right-ear hearing loss, and an intracranial tumor was suspected.Magnetic resonance imaging of the head revealed a 3-cm mass at the right transvenous foramen, which was surgically resected following preoperative embolization.

The pathological diagnosis was a sympathetic PGL of the right middle ear.Six years later, family history of PGL with germline mutation of succinate dehydrogenase g5210t-p90 complex iron sulfur subunit B, SDHB: c.268C>T (p.Arg90Ter) was clarified.The patient had elevated levels of plasma and urine CAs again.

Abdominal computed tomography scanning revealed two retroperitoneal tumors measuring 30-mm at the anterior left renal vein and 13-mm at near the ligament of Treitz.The larger tumor was laparoscopically resected, but the smaller tumor was not identified by laparoscopy.After the operation, the patient remained hypertensive, and additional imaging tests suggested a tumor localized in the duodenum.The surgically resected tumor was confirmed to be a duodenal PGL.After that, the patient remained hypertension free, and urinary levels of noradrenaline and normetanephrine decreased to normal values.

No recurrence or metastasis has been found at 1 year after the second operation.CAs secretion from PGLs in unexpected location, like the duodenum of our patient, may be overlooked and leads read more to a hypertensive crisis.In such cases, comprehensive evaluation including genetic testing, fluorodeoxyglucose-positron emission tomography scanning, and measurement of CAs will be useful for detecting PGLs.Most previous reports on duodenal PGL were gangliocytic PGL which has been renamed composite gangliocytoma/neuroma and neuroendocrine tumor, and defined the different tumor from duodenal PGL.We reviewed and discussed duodenal PGLs in addition to multiple PGLs associated with SDHB mutation.