Objective: Pheochromocytoma is a rare disease. The systemic lupus erythematosus (SLE) could be a manifestation of paraneoplastic syndrome of pheochromocytoma. we report a MEN 2A case with right adrenal pheochromocytoma and medullary thyroid cancer associated with a newly diagnosed SLE.
Methods: The clinical manifestation, lab results and imaging were described and follow-up was carried out to the changes of autoantibody after surgery.
Results: A 52-year-old female was admitted to department of endocrinology in PUMCH with paroxysmal headache, palpitation for 11 years in 2016. She didn’t monitor blood pressure. The CT scan showed a 4.6×4.7cm left adrenal mass in the local hospital. Then she was sent to operation without further examination and medical preparation in local hospital. The blood pressure raised to the 240/180mmHg when the surgeon detected the tumor. The operation was discontinued and the patient was transferred to PUMC Hospital. 24h urine catecholamine revealed normal (NE 21.35ug, E 31.06ug↑, DA 133.94ug). The MIBG and 99mTc-octreotide scanning showed high uptake in the left adrenal region. The thyroid ultrasonography revealed 3.0×1.8×1.8cm hypoechoic solid nodule in the left thyroid. The nodule had irregular shape, unclear boundary and multiple microcalcifications. The several enlarged cervical lymphnodes were detected with the disappearance of normal medulla structure. The ANA 1:640, decreased C3. C4 and several positive autoantibodies were found due to low count of white blood cell. The SLE was diagnosed by the rheumatologist. After the management of phenoxybenzamine, the operation of pheochromocytoma and thyroid were carried out successively. The pathology revealed the MTC of the thyroid. MEN2A could be diagnosed.
Conclusions: SLE accompanying with the pheochromocytoma was reported very rarely. After the removal of pheochromocytoma, the improvement of SLE could be attained in some cases. The SLE was considered it could be one of the rare paraneoplastic syndrome of the pheochromocytoma.