Primary Epithelioid Pulmonary Angiosarcoma with WAIHA and Thrombocytopenia: A Rare Association

Abstract

Abstract Introduction/Objective Angiosarcomas accounts for approximately 1%-2% of sarcomas, particularly involving the deep soft tissue. As a subtype, primary epithelioid angiosarcoma is an extremely rare neoplasm. Herein, we present an unusual primary epithelioid angiosarcoma of the right upper lung lobe with multiple nodal and bony metastases and associated severe form of autoimmune hemolytic anemia (WAIHA) and thrombocytopenia. Methods/Case Report Our case is a 79-year-old female with a recent onset of warm type autoimmune hemolytic anemia with positive coombs test and thrombocytopenia. The patient serum was also positive for EBV (IgG and IgM). On chest CT scan, a right upper lobe, metabolically active lung mass with mediastinal lymphadenopathy was found. Metabolically active bilateral osseous lesions identified within thoracic spine, ribs, bilateral humeri and femurs. A transbronchial core biopsy of the lung mass revealed poorly differentiated epithelioid hemorrhagic neoplasm with extensive necrosis and vascular background. The tumor cells are positive for AE1/AE3, CK7, FLI-1, BRG1 (SMARCA4, retained), and vimentin; and faint weak focal membranous staining for CD31, Factor VIII, and ERG. They are negative for CK20, TTF-1, Napsin-A, p63, p40, SMA, Melan-A, HMB45, GATA3, PAX-8, CDX-2, HepPar, synaptophysin, calretinin, CAMTA1, EBER (EBV) and S100 (Figure-1-A-D, H&E, 10X, CK AE2/AE3, FLI-1, CD31). This immunoprofile and histologic features favor epithelioid angiosarcoma. Results (if a Case Study enter NA) NA. Conclusion Comprehensive morphological and immunohistochemical findings supported the diagnosis of epithelioid angiosarcoma. The importance of reporting this case was the unusual pulmonary primary presentation, associated nodal and osseous secondary involvement, together with the unique systemic presentation with warm autoimmune hemolytic anemia and thrombocytopenia in a patient with active EBV infection.