Epibulbar complex and osseous choristoma: Clinicopathological study with interesting associations

Abstract

Materials and methodsA retrospective cohort study of cases with tissue diagnosis of epibulbar choristoma in 2 centers presenting during the period: 2000–2016 with focus on cases of complex and osseous choristoma. Demographic and clinical data were collected by the resident from medical records. The histopathological slides were reviewed by 2 pathologists and cases of complex and osseous choristoma were analyzed by biostatical staff.Results120 epibulbar choristomas were identified. 13/120 patients (10.8%) with complex choristoma and 2/120 patients (1.7%) with osseous choristoma. 15 cases were further analyzed: 7 were males and 8 were females. Mean age at presentation was 7.6 year. Commonest location was temporal in 66.6%. The presence of smooth muscle component was associated with a larger size choristoma (p = 0.042). 73.3% had other ophthalmic manifestations (mostly eyelid anomalies) while 9 systemic associations (mostly Goldenhar's syndrome) were found in 8/13 cases of complex choristoma.ConclusionEpibulbar complex and osseous choristomas are rare. Histopathologically, the presence of smooth muscle significantly correlates with the lesion size. Complex choristoma is more likely to be associated with systemic manifestations. Further genetic studies for this condition are recommended.

Epibulbar osseous choristoma: Two case reports

“…The prevalence of osseous choristoma in our hospital was 0.676%. Similar results were observed in a study conducted by Aldossary MM et al [ 2 ], in which among the 120 patients with myoblastoma of the ophthalmic surface, two had osteogenic myoblastoma, with a prevalence of 1.7%. Among the osseous choristoma cases in this study, one case was observed in a young woman, and it presented as a hard mass on the upper left temporal quadrant, which was in accordance with the previous reviews.…”

“…At present, there is no unified conclusion on the relationship between the occurrence and development of osseous choristoma and sex. Although it has been reported that young women tend to have a high incidence[ 4 - 7 ], the association with sex was not significant due to the small number of cases[ 2 ]. Additionally, osseous choristoma is observed more frequently in the right eye than in the left eye, and its sites are mainly distributed in the conjunctiva, sclera, and ophthalmic muscle, with most of them located in the fascia of the superior temporal quadrant[ 8 - 10 ], However, osseous choristoma occurring in the rectus muscle or eyelid is rare[ 6 ], and the frequency of these cases has not yet been statistically analyzed[ 1 ].…”

“…Choristoma is a rare, benign, congenital proliferative tumor[ 1 ], which is defined as normal tissue that stops migrating during embryonic development and is located in an abnormal position. Epibulbar choristoma normally occurs sporadically and develops alone, or it may be associated with a variety of syndromes[ 2 ], such as Goldenhar syndrome, epidermal nevus syndrome, and encephalo-cranio cutaneous lipomatosis. Ocular choristomas can be classified as dermoid, dermolipoma, complex choristoma (choristomas with more than one tissue type), and single-tissue choristoma, among which osseous and complex choristomas are the rarest.…”

“…Ocular choristomas can be classified as dermoid, dermolipoma, complex choristoma (choristomas with more than one tissue type), and single-tissue choristoma, among which osseous and complex choristomas are the rarest. The prevalence of epibulbar choristoma ranges from 1/10000 to 3/10000[ 2 ] and can occur at multiple sites, predominantly in the cornea, rectus muscle, and conjunctiva. In this study, we reviewed myoblastoma cases treated at our hospital since 2010 and provided reports and detailed preoperative, intraoperative, and postoperative lesion images, including gross and pathological images, of two patients with osseous choristoma.…”

Ophthalmic Manifestation and Pathological Features in a Cohort of Patients With Linear Nevus Sebaceous Syndrome and Encephalocraniocutaneous Lipomatosis

“…Complex choristomas contain cells from two or more different tissue types, such as bone, cartilage, nerve, muscle, sebaceous secretions, or lacrimal tissues. Complex choristomas can be sporadic cases or can be associated with Goldenhar syndrome and LNSS (25)(26)(27)(28). For patient 24, the first biopsy of the nasal part showed a dermolipoma, and the result of the second biopsy of the temporal part disclosed a complex choristoma with immature hair follicle tissue, sebaceous gland, and hyperplasia of adipose tissue.…”