ECE2019 Poster Presentations Adrenal and Neuroendocrine Tumours 3 (70 abstracts)
Association of neurofibromatosis type 1 and neuroendocrine tumor
Faten Hadj Kacem , Asma Zargni , Fatma Mnif , Fatma Rekik , Mouna Elleuch , Dhouha Ben Salah , Nedia Charfi , Mouna Mnif , Nabila Rekik & Mohamed Abid
Department of Endocrinology and Diabetology, Hedi Chaker Hospital, Sfax, Tunisia.
Introduction: Pheochromocytoma occurs in 0.1–5.7% of patients with Neurofibromatosis type 1(NF1). We report two cases of adrenal pheochromocytoma in patients with NF1.
Observation: A 46 year-old male was admitted to our department for further examinations of an adrenal mass. The CT-scan showed two well-defined right adrenal masses measuring each 128×87×86 mm and 60×52×37 mm with central necrosis and calcifications in the biggest one. It suppresses the right kidney and fills in the vertebral foramens D12-L1 and L1-L2 without osteolysis. The magnetic resonance imaging (MRI) showed the same well-defined heterogeneous masses with intense enhancement without spread to adjacent organs. The second patient is a 27 year-old female, her CT-scan showed a left adrenal heterogeneous mass measuring 80×80×74 mm with central necrosis as well. In their past medical history, both patients had palpitation, sweating and headache and hypertension in one case. They had multiple neurofibromas over the entire skin, café-au-lait spots on the trunk and extremities and skinfold freckling. A left-eye-redness was noticed in one patient and the presence of Lisch nodule in his left eye was confirmed after ophthalmological examination. Biochemical tests were initiated and showed in both cases increases in norephrine and epinephrine. Aldosterone, renin and Aldosteron-renin ratio were suggestive of a secondary hyperaldosteronsim. A 131Iodine-metaiodobenzylguanidine (131I-MIBG) scan showed an intense uptake super-imposed on the adrenal gland in the CT. Adrenalectomy was indicated but the male patient refused the surgery. As for the female patient, she had left adrenalectomy with a regular follow-up.
Conclusion: Because of the increased prevalence of pheochromocytoma in patients with NF1, and the potential associated adverse effects, we emphasize the importance of periodic clinical evaluation with biochemical testing and imaging studies.
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Endocrine Abstracts
ISSN 1470-3947 (print) | ISSN 1479-6848 (online)
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