Endocrine Abstracts

Introduction: Pheochromocytomas and paragangliomas (PPGLs) are chromaffin cell tumors that arise from neuroectodermal cells. These tumors are characterized by a very heterogeneous natural history and an unpredictable ability to metastasize. The rate of metastatic disease ranges from lessthan 1% to 79%, depending on tumor site and size, age at diagnosis and genotype.30% are considered to be hereditary. Primary hyperparathyroidism can be associated with PPLGs. We report a case of a metastatic giant pheochromocytoma measuring 15 cm associated to an asymptomatic primary hyperparathyroidism

Case report: 64-year-old female, with no significant past medical, family and psychosocial history, admitted for management of a right adrenal lesion measuring 153×100 mm, revealed by deep asthenia, anorexia, weight loss and recurrent abdominal pain. Clinical examination reveals an anorexic patient without clinical signs of mineral, androgen or catecholamine tumoral hypersecretion. Screening test for hypertension revealed low blood pressure with a mean of 90/56 mmHg. Biochemical tests revealed a high level of catecholamines (Noradrenaline). Laboratory investigations on three occasions showed a high level on calcium adjusted for albumin (2.67–2.88 mmol/l), parathyroid hormone (102-198 pg/ml) and low phosphor (0.6 mmol/l), which confirm the diagnosis of primary hyperparathyroidism. Neck ultrasound and 99mTc-MIBI scintigraphy revealed a superior right parathyroid adenoma. Morphological exploration revealed a large right adrenal mass measuring 153×100 mm with a suspicious appearance, with liver and lung metastases. MIBG -131 scintigraphy confirms the neuroectodermic tumor of adrenal with pulmonary and hepatic localizations. The patient underwent surgery. The anatomopathological and immunohistochemical study shows an aggressive pheochromocytoma with PASS score 10, expressing synaptophysin and chromogranin A with positive PS100. Unfortunately, she died following surgery.

Conclusion: Metastatic pheochromocytoma and primary hyperparathyroidism are two endocrine entities which can occur in an isolated form or as a part of a syndromic association. The prognosis of metastatic PPGL is heterogeneous, depending on local invasion, associated with a high mortality rate and several complications. Treatments includes medications helping control blood pressure and heart rate, but surgical removal of the tumor is the main curable treatment.