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Int J Clin Exp Pathol 2012;5(3):247-253

Case Report
HIF-1α, VEGF and WT-1 are protagonists in bilateral primary angiosarcoma of breast: a
case report and review of literature

Arshad Rahmani, Mohammad Alzohairy, Habeeb Khadri, Ashish K Mandal, Moshahid A Rizvi

1Pathology department, Faculty of medicine and health Sciences, United Arab Emirates University, Al Ain, UAE; 2Department of Surgery,
Tawam Hospital in Affliation with John Hopkins International Medicine, Al Ain, UAE; 3Department of Surgery, Tawam Hospital in Affliation with
John Hopkins International Medicine, Al Ain, UAE; 4Department of Laboratory Medicine, Tawam Hospital, ALAIN, UAE; 5Department of
Laboratory Medicine, Tawam Hospital, ALAIN, UAE

Received January 28, 2012; accepted February 11, 2012; Epub March 25, 2012; Published March 30, 2012

Abstract: Bilateral primary angiosarcoma of breast is an extremely rare disease. Only 4 cases had been described in the literature. Hypoxia
inducible factor- 1 α (HIF-1α) is a transcription factor that binds to hypoxia response elements in the promoters of target genes. Vascular
endothelial growth factor (VEGF) is an important signaling protein involved in angiogenesis. Wilms tumor -1 protein (WT-1) is a transcription
factor that plays an important role in angiogenesis. We present a 29-year old female with bilateral primary angiosarcoma of breast. Five-µm
sections were stained with CD31, FLI-1, HIF-1α, WT-1, VEGF, VEGF-R, D2-40, estrogen receptor, and progesterone receptor. The neoplastic
cells show diffuse immunoreactivity to CD31, FLI-1, HIF- 1α, VEGF, VEGFR, and WT-1 protein. The neoplastic cells show no immunoreactivity to
estrogen receptor, progesterone receptor and D2-40. In conclusion, HIF- 1α, WT-1 and VEGF are possible protagonists in the development of
bilateral primary angiosarcoma of breast. The neoplastic process involves endothelial cell of blood vessels lineage rather than lymphatic
lineage. Painless breast tumors in young women that are highly vascular at the time of biopsy should be considered as malignant until proven
otherwise. Tissue biopsy is the gold standard in the diagnosis of primary angiosarcoma of breast. (IJCEP1201010
).

Keywords: Breast, angiosarcoma, HIF-1α, VEGF, WT-1


Address all correspondence to:
Dr. Suhail Al-Salam,
Pathology Department, Faculty of Medicine and Health Sciences
United Arab Emirates University, Al Ain, UAE.
Tel: 0097137672000 Ext-464; Fax: 0097137671966
E-mail: suhaila@uaeu.ac.ae