Cutaneous Angiosarcoma - A Clinicopathological Study of 16 Cases over a Period of Nine Years

Abstract

Reema Miria Abraham1 , Shiny Padinjarayil Manakkad2 , Sathi Puthen Parampath3 , Bindu Valiaveetil4

BACKGROUND Cutaneous angiosarcoma is an aggressive malignant mesenchymal vasoformative neoplasm accounting for 1 % of all soft tissue sarcomas. It carries a poor prognosis with high local recurrence rate. Cutaneous angiosarcoma occurs in 3 clinical settings namely idiopathic angiosarcoma of head and neck, angiosarcoma associated with chronic lymphedema and post irradiation angiosarcoma. Idiopathic angiosarcoma of head and neck of elderly are distinct from other types of angiosarcoma. Clinicopathological correlation of cutaneous angiosarcomas diagnosed during a period of nine years from January 2010 to July 2019 was done. METHODS We retrospectively studied and analysed specific data such as age, sex, site, size, clinical setting as well as six histopathological parameters and follow up. RESULTS There were 30 patients diagnosed as angiosarcoma. 16 of these cases were cutaneous. Their mean age was 60 years (44 – 80 years). Mean size of cutaneous tumours was 4 cms (3 – 10 cm). 15 were primary; one had history of preceding lymphedema. Most common location was scalp (14) followed by leg (2). Predominant pattern was vasoformative (75 %) which included spindle (62.5 %) and epithelioid (37.5 %) cell types. Four (25 %) showed extensive necrosis; ten (66.6 %) had high mitotic index and 12 (75 %) showed tumour infiltrating lymphocytes. All cases (100 %) were positive for CD31 & CD34. All underwent wide local excision. Adjuvant chemotherapy was given in two cases and adjuvant radiotherapy in three cases. Of the seven cases with follow up, five cases died of their disease and two survived. CONCLUSIONS Larger tumour size & older age group were associated with worse outcome. All our 14 cases very well fit with the angiosarcoma of scalp & face in elderly persons as described in the literature. This disease has unique characteristics compared to angiosarcoma in other locations.

image