Introduction
An angiosarcoma (AS) is an aggressive, rare malignant endothelial cell tumor of lymphatic or vascular origin that can arise in the liver, breast, spleen, bone, or heart, but frequently they are multicentric 1,2. Metastasis occurs in more than half of all patients, most involving the lung that usually presents with multiple pulmonary nodules and diffuse alveolar hemorrhage3–5.
Patients with primary disease or metastasis to the lung are often misdiagnosed due to common clinical manifestation and little clinical suspicion index of the illness, which often leads to late diagnosis and a poorer prognosis 5.
Angiosarcomas have insidious growth, and they may be asymptomatic until the disease is well advanced 6. Clinical features of angiosarcomas depend on sites and organs involved. The patient may present with disseminated intravascular coagulation, bleeding, anemia, thrombocytopenia, pathologic fractures, compression of adjacent neurovascular structures, or hepatic dysfunction 7. Patients with soft tissue angiosarcomas usually present with a moderately paced growing mass in the extremities8.
In the low-grade form, angiosarcoma may resemble a haemangioma. In contrast, the aggressive form may have overlapping features9. The tumor can present at any age; recent cases have been reported in a 5-year-old child and an elderly of 97 years10.
Since 1879, when Langhans and colleagues reported the first angiosarcoma in the spleen 11, the exact etiology of these tumors has never been established; however, several risk factors have been implicated and include exposure to environmental chemical toxins and foreign bodies, radiation therapy, chronic lymphedema, Human Immunodeficiency Virus infection and Acquired Immune Deficiency Syndrome (HIV/AIDS), and chronic use of calcium channel blockers1,5,6,12–14. Long-term use of anabolic steroids has also been linked with the development of angiosarcomas14,15.
The differential diagnosis of angiosarcoma includes melanoma, pyogenic granuloma, fibrosarcoma, liposarcoma, Kaposi Sarcoma, and metastatic cancer with unknown primary site.
The site of disease involved and staging guide the choice of imaging modalities like Magnetic Resonance Imaging (MRI), Computed Tomography (CT), and Positron Emitted Tomography (PET). The treatment options are minimal, especially with the aggressive nature of the disease. To date, there is no single or combined definitive therapy which confers long term favorable outcome with metastatic disease 16.