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Atypical retiform hemangioendothelioma arising in a patient with Milroy disease: a case report and review of the literature.

Identifieur interne : 000761 ( Main/Exploration ); précédent : 000760; suivant : 000762

Atypical retiform hemangioendothelioma arising in a patient with Milroy disease: a case report and review of the literature.

Auteurs : Aileen Grace P. Arriola [États-Unis] ; Laura A. Taylor [États-Unis] ; Eseosa Asemota [États-Unis] ; Markus D. Boos [États-Unis] ; David E. Elder [États-Unis] ; Kristy L. Weber [États-Unis] ; Robert G. Micheletti [États-Unis] ; Paul J. Zhang [États-Unis]

Source :

RBID : pubmed:27730656

Descripteurs français

English descriptors

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.

DOI: 10.1111/cup.12844
PubMed: 27730656


Affiliations:


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Le document en format XML

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<div type="abstract" xml:lang="en">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.</div>
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