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.
Original language | English (US) |
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Pages (from-to) | 98-103 |
Number of pages | 6 |
Journal | Journal of cutaneous pathology |
Volume | 44 |
Issue number | 1 |
DOIs | |
State | Published - Jan 1 2017 |
Keywords
- Milroy disease
- chronic lymphedema
- retiform hemangioendothelioma
- vascular neoplasm
ASJC Scopus subject areas
- Pathology and Forensic Medicine
- Histology
- Dermatology