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Erythrocytosis As A Paraneoplastic Syndrome in A Case of Angiosarcoma

Author(s): Caroline Hana, George Hanna, Atif Hussein

Angiosarcoma is a rare form of sarcoma that is rarely associated with paraneoplastic syndromes. Its association with increased Erythropoietin (EPO) secretion has only been reported in one case. We report the case of a 73-yearold female patient presenting with a mass on the parotid gland, which was identified to be an angiosarcoma, demonstrated through biopsy and Positron Emission Tomography (PET) scan. The laboratory studies demonstrated elevated hemoglobin and hematocrit levels, with a markedly increased serum erythropoietin level. The workup for primary polycythemia was negative. The patient was treated with immediate phlebotomy on 3 occasions and then started paclitaxel, initially alone and then in combination with external beam radiation therapy for the angiosarcoma. After 8 weeks of the initiation of chemotherapy alone, the patient's hemoglobin and erythropoietin levels normalized. The repeat PET scan after the third cycle of paclitaxel showed a mixed response with improved size of the mass but increased intensity probably a reflection of the inflammatory response from chemotherapy. These findings suggest that the patient's polycythemia was secondary to increased erythropoietin secretion from her angiosarcoma.

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