Sporadic Cutaneous Angiosarcomas Generally Lack HIF1α; a Histologic and Immunohistochemical Study of 45 Cases
MM Desouki, M Abedalthagafi, EJ Rushing, J Marwaha, A Auerbach, JC Fanburg-Smith. MUSC, Charleston, SC; Georgetown University Medical Center, DC; AFIP, DC; AIPL, MD
Background: Cutaneous angiosarcoma (AS) is a rare malignant neoplasm of dermis composed of infiltrating endothelial cells. Hypoxia-inducible factor-1 (HIF1) is a transcription factor that mediates cellular homeostatic responses to hypoxia. We studied the utility of HIF-1-alpha as a marker or explanatory factor in cutaneous AS.
Design: Forty five cases of cutaneous AS were retrieved from the Soft Tissue and Dermatopathology Registries of the Armed Forces Institute of Pathology (AFIP). Cases were re-reviewed for inclusion based on patient folder, slides, and obtained IHC. IHC for HIF-1 alpha was performed on 18 cases with available material.
Results: Seventeen and 83% of cases were females and males, respectively. The mean age at presentation is 67 years (range, 27-88). Tumors presented most commonly in the scalp followed by lower leg, face, arm and thigh. Associated BCC was noted in one case. No history of other primary, lymphedema, radiation, or Thorotrast-induced AS present. The tumors ranged in size from 0.4 -9.5 (mean of 2.4 cm). Histologically, most tumors were vasoformative, with either solid architecture (n=35) or papillary endothelial hyperplasia like foci (n=7). All cases demonstrated infiltrative growth pattern, cytologic atypia and mitotic activity. Surface ulceration was present in 44% and solar elastosis in the majority of cases. Epithelioid morphology was present in 29% (n=13), and mild to moderate lymphocytic inflammatory response in 62% (n=28) cases. CD31 highlighted malignant endothelial cells in all cases and SMA was generally absent. HIF-1alpha was focally positive in the cytoplasm of 3/18 (17%) cases. Follow-up data was available on 4 cases: 2 died of disease within 4 years and 2 had recurrence within 2 years.
Conclusions: Cutaneous AS is largely found on the scalp of older patients. Requirement for diagnosis includes extravascular proliferation of atypical endothelial cells with mitotic activity in vasoformative, solid or papillary patterns. Absence of SMA, indicating lack of pericytes around vessels, indicates extravascular extension of tumor outside normal vessels. Cutaneous AS generally lacks HIF-1alpha. Accordingly, the hypoxic response pathway cannot be documented as a common mechanism of angiogenesis in this entity.
Monday, March 22, 2010 9:30 AM
Poster Session I Stowell-Orbison/Surgical Pathology/Autopsy Awards Poster Session # 61, Monday Morning