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Title: Atypical retiform hemangioendothelioma arising in a patient with Milroy disease: a case report and review of the literature. Author: Arriola AG, Taylor LA, Asemota E, Boos MD, Elder DE, Weber KL, Micheletti RG, Zhang PJ. Journal: J Cutan Pathol; 2017 Jan; 44(1):98-103. PubMed ID: 27730656. Abstract: Retiform hemangioendothelioma (RH) is a rare vascular neoplasm with a high rate of local recurrence and low metastatic potential. We describe an unusual case of RH in a 45-year-old patient with Milroy disease, with a prominent solid component diffusely involving a chronic lymphedematous leg. This case is consistent with the postulated relationship between lymphedema and vascular neoplasms developing as a result of local immune dysfunction, and highlights the need to closely monitor patients with Milroy disease for pathologic changes. Our case highlights a unique example of RH with atypical features. There are several noteworthy unusual clinical and histologic findings including diffuse involvement of an entire limb, solid component with cytologic atypia, D2-40 expression, and first-time-reported association with Milroy disease. Given the atypical histologic presentation of cytologic atypia, solid areas and atypical immunohistochemical profile with D2-40 positivity, this case could cause diagnostic difficulty, especially in the setting of such a broad clinical differential.[Abstract] [Full Text] [Related] [New Search]