Metastatic malignant paraganglioma

Abstract

Introduction: Paragangliomas and pheochromocytomas are rare neuroendocrine tumors, indistinguishable at the cellular level, from chromaffin cells derived from the neural crest. The term paraganglioma refers to extra-adrenal tumors and pheochromocytoma to intra-adrenal tumors. Parasympathetic paragangliomas are located along the glossopharyngeal and vagal nerves of the neck and at the basis of the skull and generally do not secrete catecholamines. Sympathetic tumors, on the other hand, are located in the sympathetic paravertebral ganglia of the chest, abdomen and pelvis, and produce excess catecholamines, which induce a clinical history of severe hypertension, headache and tachycardia. Most sympathetic paragangliomas are familial, associated with mutations in the SDHD and SDHB genes, which the last one have a higher risk of malignancy, being necessary to search for metastatic lesions as part of the preoperative evaluation. Malignant paragangliomas are a rarer form of presentation and patients should be followed up on an outpatient basis for a long term due to the risk of metastases, which usually affect bones, lungs and liver. Objective: To report a case of a patient diagnosed with malignant paraganglioma who presented metastases 4 years after the diagnosis and surgical removal of his tumors. Case report: VM, male, a 59-year-old man comes to the health unit with a previous diagnosis of hypertension, using beta-blocker, angiotensine receptor antagonist and diuretic, complaining of difficulty in control disease and recurrent headache. Dyslipidemic, using simvastatin, social drinker, denies smoking and other pathologies. Tests were requested to investigate secondary causes, including abdominal ultrasound showing mass in retroperitoneum. Imaging investigation proceeded with abdominal computed tomography (CT) indicating a heterogeneous mass of regular contours measuring 10.9 x 9.9 x 8.3 cm with areas of cystic degeneration and contrast enhancement, in the topography of the head/uncinate process of the pancreas exerting slight compression on the inferior vena cava. The anatomopathological study after attempting surgical resection showed paraganglioma. He started alpha and beta-blockers due to a hypertensive peak in the first approach undergoing a new surgery after 21 days for complete resection of the tumor and histopathology that found malignant pheochromocytoma. He followed an outpatient follow-up showing improvement of symptoms. After 4 years without signs of recurrence, he had a CT scan of the liver showing at least 6 nodular images with characteristics of secondary involvement. He returned to oncology to investigate the new injuries and is awaiting liver biopsy and/or metastasectomy. Keywords: Malignant paraganglioma; Neuroendocrine tumors; Paraganglioma.