title: Unusual primary ocular neoplasm in a child: leiomyosarcoma of the ciliary body.
abstract: Primary uveal-tract neoplasms are extremely rare in childhood; the most common lesions found are melanocytic. We report here the case of a 7-year-old girl who underwent enucleation of the right eye with clinical suspicion of choroid melanoma as a result of a ciliary body mass that extended to the posterior chamber. Histologically, the neoplasm featured spindle cell morphology, atypia, and mitoses. The tumor expressed smooth muscle alpha actin, pan-actin HHF-35, and desmin, whereas immunohistochemistry for melanocytic markers, such as S-100, Melan-A, and HMB-45, was negative. Based on these features, the diagnosis of leiomyosarcoma of the ciliary body was firmly established. Although several leiomyomas have been reported in the literature, there are only 2 previously reported cases of primary leiomyosarcoma of the uveal tract. Immunohistochemical expression of muscle proteins allowed distinction from the most common melanocytic tumors arising in this location.
authors.affiliation: Department of Anatomic Pathology, Hospital do Câncer AC Camargo-São Paulo, Brazil. mmcajaiba@yahoo.com
authors.forename: Mariana M
authors.initials: MM
authors.lastname: Cajaiba
authors.forename: Martha M
authors.initials: MM
authors.lastname: Chojniak
authors.forename: Isabela W
authors.initials: IW
authors.lastname: Cunha
mesh_terms: D014408:Biomarkers, Tumor / Q000032:analysis; D049109:Cell Proliferation; D002648:Child; D002829:Choroid / Q000473:pathology; D002924:Ciliary Body / Q000737:chemistry / Q000473:pathology* / Q000601:surgery; D003937:Diagnosis, Differential; D015353:Eye Enucleation; D005260:Female; D006801:Humans; D007150:Immunohistochemistry; D007890:Leiomyosarcoma / Q000737:chemistry / Q000473:pathology* / Q000601:surgery; D008545:Melanoma / Q000175:diagnosis; D014604:Uveal Neoplasms / Q000737:chemistry / Q000473:pathology* / Q000601:surgery
chemical_list: D014408:Biomarkers, Tumor