Endocrine Abstracts

Extraadrenal paraganglioma is a rare tumor. The clinical and pathological findings of patients with abdominal localization paragangliomas (perihepatic, paraaortic, interaortocaval and retroperitoneal) are described. Its characteristics and diagnostic imaging are analyzed, as well as its therapeutic management. Between January 2021 and January 2022, 3 patients with extra-adrenal paragangliomas were diagnosed, all of them women. The age of our patients was 26, 57 and 67 years. The location of these tumors was at the para-aortic, inter-aortocaval, retrocaval and perihepatic level; one of the cases also presented cervical paragangliomas, with a size of the lesions ranging from 13 to 30 mm. We reviewed the clinical data. In all cases, the paragangliomas were detected as an incidental finding in imaging tests performed for another reason, although re-examining the predominant symptoms were tinnitus, sweating, tremor and headache, only one of the cases presenting hypertensive crises. One patient presented with concomitant bilateral adrenal hyperplasia/adenomas, with incomplete dexamethasone braking, but with normal ACTH and cortisol, probable hypercortisolism (Cushing’s syndrome). Metanephrine levels in 24-hour urine and catecholamines in blood were estimated in all cases, with elevated levels in one of the cases (coinciding with the hypertensive patient), with normal levels in the rest. The lesions were initially detected by computed tomography and a study was completed with 113I-MIBG scintigraphy and DOPA PET-CT in all of them. In addition, a genetic study was requested in all patients (kif-1b, MAX, NF1, PRKAR1A, RET, SDHA, Af2,B,C,D, TME127, VHL) with negative results. All cases were evaluated by Endocrinology, with a prescription of premedication with Doxazocin prior to surgery. All but one patient underwent surgical treatment. The case associated with carotid glomus is pending evaluation by surgery for excision. The preoperative diagnosis of non-functioning abdominal paragangliomas is difficult, although they should be suspected in patients with hypertension or compatible symptoms. The 3 reported cases were found in the usual locations: paraaortic, interaortocaval, retrocaval, and perihepatic. All the patients except one had multiple tumors at the abdominal level, in addition to carotid glomus in one of the cases. Resection is the treatment of choice with prior initiation of alpha-blocker medication. These patients should follow a multidisciplinary approach.