SkIndia Quiz 22: Scalp Tumor

A 72-year-old Caucasian male presented with a four month history of an asymptomatic, violaceous, ulcerated tumor over the scalp of 3 cm diameter surrounded by multifocal violaceous nodules [Figure 1]. He reported local trauma 1 month prior to appearance of the lesion. There was no lymphadenopathy or hepatosplenomegaly. Histopathologic examination showed a vascular tumoral lesion occupying the entire dermis, with a jagged irregular branching angiomatous pattern with vascular clefts between the collagen fibers filled with erythrocytes and lymphocytes around vessels [Figure 2a]. The endothelial cells were polygonal with hyperchromatic nuclei and rare atypical mitoses. The immunostaining was positive for CD31, CD34 and FVIII-related antigen [Figure 2b].Figure 1: Violaceous ulcerated tumor on the scalp surrounded by multifocal violaceous nodulesFigure 2: (a) Histopathologic examination showed a vascular tumoral lesion on the dermis, with irregular vascular clefts between the collagen fibers filled with erythrocytes and lymphocytes (H and E ×100), (b) Immunostaining positive for FVIII-related antigenWHAT IS THE DIAGNOSIS? ANSWER Diagnosis - Idiopathic cutaneous angiosarcoma of the scalp. Cutaneous angiosarcoma is a rare, malignant tumor of vascular endothelial cells.[12] It usually affects the face and scalp region, most often in elderly patients.[12] Men are more frequently affected than women in a ratio of 3:1.[2] The clinical presentation of angiosarcoma is quite variable.[2] It has three clinical settings: Angiosarcoma in the elderly, post.-radiation, or associated with chronic lymphedema.[34] Angiosarcoma accounts for approximately 10% of soft.-tissue sarcomas of the head and neck.[25] It is histopathologically characterized by irregular vascular channels dissecting between collagen bundles lined by hyperchromatic atypical endothelium and chronic inflammation.[3] Vascular and lymphatic immunohistochemical markers are essential to establish the diagnosis… - CD31, CD34, factor.-VIII and podoplanin are positive.[356] One of the main differential diagnoses is Kaposi sarcoma, in which spindled cells are CD34 and variably CD31. positive, but anti-human herpes-virus-8 antibody is always positive.[3] Angiosarcoma treatment includes surgery, wide.-field post.-operative radiotherapy and/or palliative chemotherapy.[5] The local-regional recurrence is high and the prognosis very poor. (5-year survival rate of approximately 10%).[2] This tumor can metastasize by lymphatic or hematogenous routes to the lung, lymph nodes and bone.[25] The most important determinant factors of outcome are tumor diameter, depth of invasion, margin status, recurrence and metastasis.[57]