Rare primary pulmonary angiosarcoma with aberrant neuroendocrine differentiation

Angiosarcomas are aggressive, malignant endothelial cell tumors derived from the vascular or lymphatic epithelium. Angiosarcomas makes up less than 1% of all soft tissue sarcomas. Primary pulmonary angiosarcoma is a rare tumor that most commonly presents as metastatic disease and carries a grim prognosis. The disease rarity and low index of suspicion make the diagnosis problematic. In this report, we present a case of primary pulmonary angiosarcoma with aberrant neuroendocrine differentiation. The diagnostic images, pathology and management strategy are discussed in this case with overall longer survival. The unique neuroendocrine pathologic subtype may contribute to the overall longer survival, however, there are only a few limited cases published. Further recognition and investigation of primary pulmonary angiosarcoma with aberrant neuroendocrine differentiation is warranted to further characterize diagnostic markers, determine more effective treatment strategies, and establish a possible link to improved survival.