Postpheochromocytoma Paraneoplastic Syndrome

Abstract

A clinical case of the disease in a 22-year-old patient with pheochromocytoma of the left adrenal gland, complicated by the development of paraneoplastic syndrome in the form of myelodysplastic syndrome, is presented. The onset of the disease was accompanied by a classic clinical picture characteristic of pheochromocytoma. Multislice computed tomography of the abdominal cavity revealed a large tumor of the left adrenal gland. Laboratory examination confirmed a high concentration of urinary catecholamines, as well as severe anemia and thrombocytopenia. The patient was consulted by a hematologist, a sternal puncture with immunophenotyping was performed, and secondary hypoplastic syndrome of unspecified etiology was diagnosed. After long-term preoperative drug preparation with α-blockers, correction of anemia and thrombocytopenia, surgery was performed - thoracofrenolaparotomy, left-side adrenalectomy, splenectomy. Histological conclusion after surgery: mature pheochromocytoma with circulatory disorder; morphological changes in the spleen characteristic of anemia. In the early postoperative period, the patient underwent replacement therapy for adrenal insufficiency, plasma hemotransfusion and infusion of platelet concentrate to correct anemia and thrombocytopenia. In the late postoperative period, anemia and thrombocytopenia persisted without manifestations of hemorrhagic syndrome. Myelodysplastic syndrome, such as refractory anemia and severe thrombocytopenia, persisted for a long period of time and required periodic correction. Only 5 years after surgical removal of pheochromocytoma, the patient showed a tendency towards normalization of blood counts and leveling of laboratory signs of myelodysplastic syndrome.