Introduction: Although Pheochromocytoma (PHEO) is usually a solid tumour, rarely it can present as a cystic lesion. Very few cases of MEN 2A with contemporaneous cystic pheochromocytoma and medullary carcinoma of the thyroid (MTC) are reported in the literature. Here, we present a case of young women with incidentally detected cystic pheochromocytoma with synchronous medullary carcinoma of the thyroid gland and discuss its management.
Case report: 28 years old female referred to our centre with incidentally detected left adrenal mass. Clinical history was insignificant. On examination pulse and blood pressure were normal. Neck examination showed multinodular thyroid swelling with cervical lymphadenopathy. FNAC of thyroid nodule revealed MTC. CECT was showed a cystic lesion measuring 8.7x 9.4x 9.6 cm with irregular asymmetric wall thickening with contrast enhancement. On evaluation, 24-hours urinary normetanephrine and metanephrine were significantly elevated (4355.90 µg and 4012.42 µg, respectively). The rest of the functional workup was normal. The patient underwent Left open adrenalectomy followed by total thyroidectomy with central neck lymph node dissection one month later. 24-hour urinary metanephrines at 3 months follow up were normal.
Conclusion: Although Cystic adrenal masses are often asymptomatic, they can be functional.
A young patient with pheochromocytoma should be evaluated for possible MTC.
Genetic and phenotypic correlation is important in management decisions.
Preoperative optimization and intensive intraoperative monitoring by experienced anaesthetists are vital for successful surgical outcomes.
In MEN 2 Syndrome Pheochromocytoma is often multifocal, bilateral and recurrent. Hence, long term follow up is important
Key words: Cystic Pheochromocytoma, MEN 2A, Hereditary pheochromocytoma, medullary carcinoma of thyroid
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