Endocrine Abstracts

Malignant pheocromocytoma is a rare and problematic disease because it is diagnosed only in the presence of metastases, with no definitive histological or cytological criteria of malignancy. It typically metastasizes to bone, liver, lung and lymph nodes.

A 38-year-old woman presented for hypertension (max sBP=160 mmHg), palpitation and dyspnea, with family antecedents of mammary cancer. Clinical examination was normal with the exception of palpable adenophaties at the submandibular level, bilateral laterocervical and in the left axilla, with maximum diameter of 1 cm, mobile on all plans; blood pressure equal at both arms=110/60 mmHg without orthostasis. Biological tests were normal; at hormonal profile the only modifications were metanephrine raised 5.4-fold (486.8 pg/ml) and normetanephrine raised 1.35-fold (244.6 pg/ml), the other hormonal measurements being in normal limits: TSH, iPTH, ACTH and cortisol with normal nycthemeral rhythm, prolactin, gonadotropins, estradiol, testosterone. Imaging exams revealed: ultrasonography - normal thyroid gland, laterocervical and submandibular adenophaties and a node in left breast; abdominal contrast CT: an iodophil formation of 29/26 mm, with central necrosis in the left adrenal and multiple formations in the liver space; chest radiography: pulmonary micronodes in the apical area. It was preceded unilateral adrenalectomy and the histopathological results are expected.

This is a case of young woman with catecholamine hypersecretion and a mass in the left adrenal- most probably pheocromocytoma, which associated lymphadenopathies, liver formations and pulmonary micronodules- places where pheocromocytoma usually metastasize, so most probably a malignant pheocromocytoma with the doubt of coexistence of another tumor. Although the clinical presentation and the laboratory findings were not that impressive; the malignancy in case of pheocromocytoma cannot be excluded regarding its definition, so other noninvasive and sensitive criteria of diagnosis would be necessary.