Abstract
Angiosarcoma is an aggressive rare tumour developing from endothelial cells that lines blood vessels (Weiss and Goldblum in Enzinger and Weiss’s soft tissue tumors, Mosby, St. Louis, 2001). They constitutes around 1–2% of soft tissue sarcomas (Mark et al. in Cancer Interdiscip Int J Am Cancer Soc 77(11):2400–2406, 1996; Cafiero et al. in Cancer 77:2496–2502, 1996; Aust et al. in Ann Otol Rhinol Laryngol 106(11):943–951, 1997; Fury et al. in Cancer J 11(3):241–247, 2005). Angiosarcomas usually occur in adult and elderly patients. Reported cases of angiosarcomas in children are of in the head and neck region and mediastinum (Ayadi and Khabir in Arch Pathol Lab Med 134(3):481–485, 2010). Female genital tract angiosarcomas are rarely reported. Here we are reporting a rare case of angiosarcoma ovary in a 12-year-old girl associated with Kasabach–Merritt syndrome. Kasabach–Merritt syndrome describes a consumptive coagulopathy seen in association with vascular tumours. Platelets get trapped in these highly vascular tumours leading to increased consumption of clotting factors and subsequent bleeding episodes (Ohayi et al. in Niger J Med J Natl Assoc Resid Dr Niger 22(4):362–364, 2013).
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Nath, A.G., Rema, P., Suchetha, S. et al. Angiosarcoma Ovary and Kasabach–Merritt Syndrome in a Pre-pubertal Girl: An Exceedingly Rare Case Report. Indian J Gynecol Oncolog 18, 74 (2020). https://doi.org/10.1007/s40944-020-00427-2
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DOI: https://doi.org/10.1007/s40944-020-00427-2