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C. Westerfeld, C. Allen, M. Mihm, P. Rubin; Non-Involuting Congenital Hemangioma Presenting as an Orbital Mass in a Newborn. Invest. Ophthalmol. Vis. Sci. 2007;48(13):3582.
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To present a rare and only recently described vascular tumor not previously reported to occur in the orbit. To review the utility of the GLUT-1 immunohistochemical stain in the differentiation of vascular tumors of infancy.
Observational case study and literature review.
A one-week old infant was evaluated for an area of bluish discoloration and swelling below the medial canthal region of her right eye present since birth. A presumptive diagnosis of dacryocystocele was made; however, nasolacrimal duct probing failed to relieve the mass. Computed tomography revealed an enhancing lesion, most suggestive of a hemangioma. Given the location and concern for amblyopia, early management with excision was performed. Histopathology showed extensive fibrosis and extramedullary hematopoesis. Notably, the tumor was found to be negative for the GLUT-1 immunohistochemical stain. Based on these findings, the patient was diagnosed with a non-involuting congenital hemangioma of the orbit.
Congenital hemangiomas were first described by Boon, et al. (1996). They are a rare entity accounting for less than 3% of hemangiomas present in the neonatal period. They present at birth fully grown which separates them from the more common infantile hemangiomas which appear weeks after birth and undergo a period of growth before stabilizing and involuting. Congenital hemangiomas can be further distinguished from infantile hemangiomas by negative staining for GLUT-1. GLUT-1 is a glucose transporter protein identified by North, et al. (2000). It is present in the endothelia of vascular channels of infantile hemangiomas, allowing them to be distinguished from other vascular tumors of infancy. The majority of congenital hemangiomas involute rapidly after birth. However, Enjolras, et al. (2001) identified a less common subset of congenital hemangiomas that fail to involute. Thus, congenital hemangiomas are now further classified as rapidly involuting congenital hemangiomas (RICH) or non-involuting congenital hemangiomas (NICH). In our patient’s case, negative GLUT-1 staining combined with the characteristic histopathology enabled us to categorize the tumor as a NICH. In summary, this case of a NICH demonstrates an uncommon vascular tumor of infancy not previously reported in the orbit. We hope to promote a better understanding of the defining features of congenital hemangiomas, in particular, the recently described histopathology and immunohistochemistry, which have allowed further elucidation of this rare entity.
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