Malignant pheochromocytoma with late distant metastases and secondary hypercalcaemia

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Introduction: Pheochromocytoma is a rare neuroendocrine tumour of the adrenal medulla. Malignant variants occur with a very low frequency and can be diagnosed only after the spread of distant metastasis.

Aim(s): To report a case of malignant pheochromocytoma with distant metastases and secondary hypercalcaemia that occur more than a decade after the resection of the primary lesion.

Materials and methods: A 54-year-old female patient underwent an extirpation of immunohistochemically confirmed pheochromocytoma measuring 12/10 cm. Postoperatively, full regression of symptoms and normal urine metanephrines were present. Two years later, the patient was lost to follow-up. Her clinical course was uneventful up to the 13th postoperative year, when she presented with left-sided abdominal pain, weight loss, recurrence of the paroxysmal hypertension and diabetes mellitus. Imaging diagnostic revealed multiple intra-abdominal masses (sigmoid colon, splenic hilus, omentum, parietal peritoneum). Histological analysis after radical resection of the lesions confirmed metastatic pheochromocytoma. After a disease-free interval of 4 years, deterioration of the diabetes control was noted. The latest CT detected liver, peritoneal and retroperitoneal masses. Laboratory assessments showed elevated urine metanephrines and newly-onset mild to moderate hypercalcaemia.

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Authors: Kirova I, Vandeva S, Markova M, Elenkova A, Robeva R,

Keywords: neuroendocrine, malignant pheochromocytoma, metastasis, hypercalcaemia,