Primary Cutaneous Epithelioid Angiosarcoma: A Clinicopathologic Study of 13 Cases
R Suchak, K Thway, B Zelger, C Fisher, E Calonje. St John's Institute of Dermatology, London, United Kingdom; Royal Marsden Hospital, London, United Kingdom; Innsbruck Medical University, Innsbruck, Austria
Background: Epithelioid angiosarcomas (EAS) are rare aggressive neoplasms, most frequent in deep soft tissues. Visceral / cutaneous lesions are reported. Primary cutaneous lesions are rare, with discrepancy in the literature regarding their behaviour. Some authors suggest they may be less aggressive than their soft tissue counterparts. We report a series of 13 cases of cutaneous EAS, and analyse their clinicopathologic features.
Design: 19 cases of EAS were retrieved from our referral archives. Lesions on the head and neck of elderly patients, post-radiotherapy, in association with lymphoedema, or deemed to be metastatic / extension from soft tissues were excluded. Thirteen cases of primary cutaneous EAS were examined for histopathological and immunohistochemical features. Clinical details, treatment and follow-up were requested from referring pathologists in all cases.
Results: Primary cutaneous epithelioid angiosarcoma occurred in adults (mean age 66 years) with an equal sex distribution, and presented as solitary (n=11) or multiple (n=2) nodules ranging in size from 8 – 80 mm, with predilection for the limbs (n=10), Histopathologically the lesions comprised infiltrative sheets of atypical epithelioid cells within the dermis +/- subcutis. 5 lesions were at least focally polypoid. Epidermal hyperplasia was noted in 5 cases, and ulceration in 4. Vascular channel formation and intracytoplasmic lumina were seen at least focally in 11 and 10 cases respectively. Mitoses were readily identified and necrosis was seen in 5 cases. The neoplasm was immunoreactive for the vascular markers CD31 and FLI-1 in all cases, and CD34 in half of the cases tested. Pancytokeratin (MNF116) was positive in two-thirds of cases, and EMA in a quarter of cases. There was rare focal expression of SMA. S100 protein, HMB45, desmin, CD30 and HHV8 were negative, whilst INI1 was positive in all cases tested. Follow-up information was available for 11 patients. 7 patients had staging CT scans, 5 showing evidence of dissemination, with regional lymph node involvement (n=1), liver (n=1), lung (n=1), bones (n=1) and regional lymph node + liver (n=1). Treatment was surgical +/- adjuvant radiotherapy in all cases. 6 patients died from metastatic disease after a median follow-up of 12 months (3-36 months), and 1 from unrelated causes.
Conclusions: Primary cutaneous epithelioid angiosarcoma is a highly aggressive malignant tumour with mortality rates in excess of 55% after 3 years.
Category: Bone & Soft Tissue
Monday, March 22, 2010 1:00 PM
Poster Session II # 19, Monday Afternoon