Pheochromocytoma with paraneoplastic phenomena manifested as myelodysplastic syndrome

Abstract

We present a clinical case of 22yo patient with diagnosis of pheochoromocytoma complicated with myelodysplastic syndrome as manifestation of paraneoplastic phenomenon. The onset of the disease displays typical clinical picture of pheochromacytoma. After medical examination and survey it was discovered a tumor in left adrenal gland and elevation of metanephrines and normetanephrines. In addition, patient has severe anemia and thrombocytopenia. Sternal punction with morphological examination and immunophenotyping were performed. Patient had consulted by hematologist. After all additional analysis cause of anemia and thrombocytopenia remained unknown and related to presence of tumor.Patient was performed a long-time and vast pre-operative preparation with administration of doxazosin and transfusions of blood and platelet concentrate. In continuation, mass in left adrenal gland was excised with spleen by thoracophrenolaparotomy. Morphology confirmed pheochromacytoma, which has typical histological structure and circulatory disorders. Spleen has no specifical features except of focuses of extramedullar hematopoiesis. Patient has short period of hormone therapy to avoid adrenal crisis on post-operative stage. Also massive transfutions of blood and platelet concentrate was performed due to persisting anemia and thrombocytopenia. There were no manifestations of hemorrhage syndrome after the surgery. Myelodysplastic syndrome was detected 3 month later and manifested itself in form of refractory anemia and severe thrombocytopenia, which persisted for long period and required corrections with therapy and transfusions. Only through 5-year observation after adrenalectomy patient has positive dynamic and leveling of laboratory tests without symptoms of myelodysplastic syndrome.