title: Phakomatous choristoma in a 10-week-old boy: a case report and review of the literature.
abstract: 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.
authors.affiliation: Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, Minnesota.
authors.forename: Ryan C
authors.initials: RC
authors.lastname: Romano
authors.affiliation: Department of Pathology, Prevea Clinic, Green Bay, Wisconsin.
authors.forename: Patrick
authors.initials: P
authors.lastname: McDonough
authors.affiliation: Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, Minnesota.
authors.forename: Diva R
authors.initials: DR
authors.lastname: Salomao
authors.affiliation: Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, Minnesota.
authors.forename: Karen J
authors.initials: KJ
authors.lastname: Fritchie
mesh_terms: D002828:Choristoma / Q000151:congenital*; D005141:Eyelid Diseases / Q000151:congenital*; D006801:Humans; D007223:Infant; D007908:Lens, Crystalline; D008279:Magnetic Resonance Imaging; D008297:Male