Angiolithic sarcoma, also known as epithelioid angiosarcoma or angioendotheliomatosis, is a rare malignant tumor that arises from the endothelial cells lining the blood vessels. It is characterized by the abnormal growth and proliferation of these cells, resulting in the development of a tumor within the blood vessels or adjacent tissues.
The term "angiolithic" refers to the tumor's tendency to invade and encase the blood vessels, a distinguishing feature of this particular subtype of angiosarcoma. This unique growth pattern often leads to the obstruction of blood flow, causing complications such as ischemia and necrosis in the affected tissues.
Angiolithic sarcoma typically presents as a deeply infiltrative mass, commonly affecting the skin, soft tissues, and less frequently, internal organs. It can manifest as a firm, purple-to-reddish nodule or plaque-like lesion, which may ulcerate and bleed. The tumor may rapidly metastasize through the bloodstream to distant sites, most commonly the lungs and liver, leading to a poor prognosis.
Histopathological examination is usually necessary to confirm the diagnosis, revealing atypical, epithelioid endothelial cells forming irregular vascular spaces with varying degrees of cellular pleomorphism. Immunohistochemistry is often performed to distinguish angiolithic sarcoma from other similar tumors.
Due to its aggressive nature and high propensity for metastasis, treatment options for angiolithic sarcoma typically involve a combination of surgical resection, radiation therapy, and chemotherapy. However, the prognosis for patients diagnosed with angiolithic sarcoma tends to be poor, primarily due to late-stage diagnosis, inherent aggressiveness of the disease, and limited treatment options.
