title: Therapy and outcome of orbital primitive neuroectodermal tumor.
abstract: Primary orbital primitive neuroectodermal tumor (PNET) is rare with no reported series. We report six cases of orbital PNET treated at a tertiary care oncology center in northern India from 2003 to 2008. None of them had distant metastases. All were treated with neoadjuvant chemotherapy followed by exenteration in two, radiotherapy and adjuvant chemotherapy in five cases. Three out of six achieved complete remission at end of therapy with globe salvage in three and vision in two cases. Chemoradiotherapy may help us to avoid mutilating surgery in large or locally advanced tumors, allowing preservation of vision or the globe.
authors.affiliation: Department of Medical Oncology, Dr B.R.A. Institute Rotary Cancer Hospital, All India Institute of Medical Sciences, New Delhi, India. sambakh@hotmail.com
authors.forename: Sameer
authors.initials: S
authors.lastname: Bakhshi
authors.forename: Rachna
authors.initials: R
authors.lastname: Meel
authors.forename: Syed Ghaffar Hasan
authors.initials: SG
authors.lastname: Naqvi
authors.forename: B K
authors.initials: BK
authors.lastname: Mohanti
authors.forename: Seema
authors.initials: S
authors.lastname: Kashyap
authors.forename: Neelam
authors.initials: N
authors.lastname: Pushker
authors.forename: Seema
authors.initials: S
authors.lastname: Sen
mesh_terms: D000293:Adolescent; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D017024:Chemotherapy, Adjuvant; D002648:Child; D002675:Child, Preschool; D003131:Combined Modality Therapy; D005260:Female; D006801:Humans; D007223:Infant; D008297:Male; D020360:Neoadjuvant Therapy; D018242:Neuroectodermal Tumors, Primitive / Q000401:mortality / Q000473:pathology / Q000628:therapy*; D013508:Ophthalmologic Surgical Procedures; D009918:Orbital Neoplasms / Q000401:mortality / Q000473:pathology / Q000628:therapy*; D011878:Radiotherapy; D016896:Treatment Outcome; D055815:Young Adult