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A Deep Penetrating Facial Congenital Melanocytic Tumor With Bone Involvement and Ipsilateral Eye Blindness

Bergman, Reuven MD*; Ben-Arush, Miriam W. MD†; Bar-Shalom, Rachel MD‡; Gilboa, Michael MD§; Simon, Einav PhD¶; Hershkovitz, Dov PhD, MD¶; Sabo, Edmond MD¶; Maly, Alexander MD‖; Gerami, Pedram MD**; Goldsher, Dorith MD††

American Journal of Dermatopathology: January 2015 - Volume 37 - Issue 1 - p e5–e11
doi: 10.1097/DAD.0000000000000048
Extraordinary Case Report

Abstract: Bone involvement has been described in tumors with melanocytic differentiation such as melanotic neuroectodermal tumor of infancy, and very rarely in cellular blue nevi and neurocristic cutaneous hamartoma. We present an unusual case of facial congenital melanocytic tumor that involved the underlying bones and maxillary sinus and led to unilateral blindness. A newborn with a large red bluish patch with peripheral brown and black macules overlying marked swelling on the left side of his face was presented. The tumor was shown by magnetic resonance imaging, scintigraphy, and histopathology to invade the underlying bones and maxillary sinus and to compress the left eyeball resulting in blindness. Histopathology, immunohistochemistry, morphometric computerized microscopy, molecular genetic mutation analysis, and fluorescent in situ hybridization studies were more congruent with a melanocytic nevus. An 8.5-year follow-up was uneventful, with spontaneous partial shrinkage of the tumor.

Departments of *Dermatology,

†Pediatric Oncology,

‡Nuclear Medicine,

§Ophthalmology, and

¶Pathology, Rambam Health Care Campus and The Ruth and Bruce Rappaport Faculty of Medicine, Haifa, Israel;

‖Department of Pathology, Hadassah Medical Center, Jerusalem, Israel;

**Department of Dermatology, NorthWestern University School of Medicine, Chicago, IL; and

††Department of Radiology, Rambam Health Care Campus and The Ruth and Bruce Rappaport Faculty of Medicine, Haifa, Israel.

Reprints: Reuven Bergman, MD, Department of Dermatology, Rambam Health Care Campus, P.O.B. 9602, Haifa 31096, Israel (e-mail: r_bergman@rambam.health.gov.il).

The authors declare no conflicts of interest.

© 2015 by Lippincott Williams & Wilkins.