title: Orbital medulloepithelioma in an adult patient: Radiation-induced second neoplasia?
abstract: Second cancers in survivors of hereditary retinoblastoma occur much more commonly than in the general population. This can be attributed both to the germline mutation of the RB gene and chemoradiation used for treatment of this paediatric cancer. Medulloepithelioma is an uncommon tumor of neuroectodermal origin, seen largely in the paediatric population and rarely reported in adults. Though the incidence of second malignancies is common in retinoblastoma, medulloepithelioma as a second malignancy in retinoblastoma survivors is rare, with only one case reported so far. Herein, we present a case of a 29-year-old patient presenting with medulloepithelioma of the right orbit, arising in the radiation field of previously treated retinoblastoma. This case was also peculiar in that though the origin of tumor was in the eyeball it had a very aggressive clinical course.
authors.affiliation: a Department of Radiation Oncology , All India Institute of Medical Sciences , New Delhi , India.
authors.forename: Antony George Francis
authors.initials: AG
authors.lastname: Thottian
authors.affiliation: a Department of Radiation Oncology , All India Institute of Medical Sciences , New Delhi , India.
authors.forename: Rony
authors.initials: R
authors.lastname: Benson
authors.affiliation: b Department of Ocular Pathology , All India Institute of Medical Sciences , New Delhi , India.
authors.forename: Seema
authors.initials: S
authors.lastname: Kashyap
authors.affiliation: a Department of Radiation Oncology , All India Institute of Medical Sciences , New Delhi , India.
authors.forename: K P
authors.initials: KP
authors.lastname: Haresh
authors.affiliation: a Department of Radiation Oncology , All India Institute of Medical Sciences , New Delhi , India.
authors.forename: Subhash
authors.initials: S
authors.lastname: Gupta
authors.affiliation: a Department of Radiation Oncology , All India Institute of Medical Sciences , New Delhi , India.
authors.forename: Dayanand
authors.initials: D
authors.lastname: Sharma
authors.affiliation: a Department of Radiation Oncology , All India Institute of Medical Sciences , New Delhi , India.
authors.forename: Goura Kishor
authors.initials: GK
authors.lastname: Rath
mesh_terms: D000328:Adult; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D009381:Neoplasms, Radiation-Induced; D018242:Neuroectodermal Tumors, Primitive / Q000000981:diagnostic imaging / Q000209:etiology*; D009918:Orbital Neoplasms / Q000000981:diagnostic imaging / Q000209:etiology*; D061766:Proton Therapy / Q000009:adverse effects*; D011880:Radiotherapy Planning, Computer-Assisted; D019572:Retinal Neoplasms / Q000532:radiotherapy*; D012175:Retinoblastoma / Q000532:radiotherapy*; D014057:Tomography, X-Ray Computed
keywords: Medulloepithelioma; retinoblastoma; second malignancy