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Phakomatous Choristoma in a 10‐Week‐Old Boy: A Case Report and Review of the Literature
Author(s) -
Romano Ryan C.,
McDonough Patrick,
Salomao Diva R.,
Fritchie Karen J.
Publication year - 2014
Publication title -
pediatric dermatology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.542
H-Index - 73
eISSN - 1525-1470
pISSN - 0736-8046
DOI - 10.1111/pde.12493
Subject(s) - choristoma , histopathology , medicine , rhabdomyosarcoma , orbit (dynamics) , embryonal rhabdomyosarcoma , pathology , stroma , eyelid , lesion , cuboidal cell , eosinophilic , subcutaneous fat , anatomy , immunohistochemistry , sarcoma , radiology , adipose tissue , engineering , aerospace engineering
Phakomatous choristoma ( PC ) is a rare benign congenital lesion of lenticular anlage. It presents in young patients as a firm subcutaneous mass in the medial eyelid or orbit and may raise clinical concern for neoplasms such as rhabdomyosarcoma, but its histopathology is distinct, consisting of dense collagenous stroma and eosinophilic cuboidal epithelial cells forming nests, tubules, cords, or pseudoglands. We present a case of PC in a 10‐week‐old boy to illustrate the unique clinical, histopathologic, and immunophenotypic features of this condition and to reaffirm that familiarity with this rare entity aids accurate diagnosis.