title: Multimodality treatment approach in management of primary peripheral primitive neuroectodermal tumor of the orbit.
abstract: Primitive neuroectodermal tumor is a small round cell malignancy which rarely involves the orbit. We report a case of a two-year old male child presenting as unilateral eccentric proptosis with extraconal and intraconal mass, diagnosed as primary peripheral primitive neuroectodermal tumor (pPPNET) on histopathology and immunohistochemistry. There is no defined consensus in the management of these tumors due to its rare presentation. We describe its distinguishing features with emphasis on multimodal and aggressive treatment approach which ensures appropriate management of these cases.
authors.affiliation: Orbit, Oculoplasty and Oncology Clinic, Aravind Eye Hospital and Postgraduate Institute of Ophthalmology Madurai, Tamil Nadu, India. usha@aravind.org
authors.forename: Usha R
authors.initials: UR
authors.lastname: Kim
authors.forename: Vipul
authors.initials: V
authors.lastname: Arora
authors.forename: J
authors.initials: J
authors.lastname: Devanand
authors.forename: Hadi M
authors.initials: HM
authors.lastname: Khazei
mesh_terms: D000970:Antineoplastic Agents / Q000627:therapeutic use*; D001706:Biopsy; D002675:Child, Preschool; D005500:Follow-Up Studies; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D018241:Neuroectodermal Tumors, Primitive, Peripheral / Q000175:diagnosis / Q000188:drug therapy / Q000532:radiotherapy*; D009918:Orbital Neoplasms / Q000175:diagnosis / Q000188:drug therapy / Q000532:radiotherapy*; D018714:Radiotherapy, Adjuvant; D014057:Tomography, X-Ray Computed
chemical_list: D000970:Antineoplastic Agents
references.citation: Hum Pathol. 2001 Oct;32(10):1109-15
references.pmid: 11679946
references.citation: N Engl J Med. 1984 Aug 30;311(9):584-5
references.pmid: 6749231
references.citation: Ophthalmology. 1999 Jan;106(1):98-102
references.pmid: 9917788
references.citation: Cancer. 1980 Dec 1;46(11):2456-62
references.pmid: 7438020
references.citation: J Pediatr Ophthalmol Strabismus. 2002 Jul-Aug;39(4):242-4
references.pmid: 12148560
references.citation: Arch Ophthalmol. 1994 Feb;112(2):217-21
references.pmid: 8311776
references.citation: Can J Ophthalmol. 2007 Feb;42(1):138-40
references.pmid: 17361260
references.citation: Acta Neuropathol. 1991;82(6):502-7
references.pmid: 1723830