title: Isolated Extranodal Natural Killer T-Cell Lymphoma of the Orbit in a Young Patient: Complete Regression with the SMILE Regimen.
abstract: OBJECTIVE
  Natural killer T-cell lymphoma (NKTL) is a highly malignant tumor that typically arises in the nose and/or paranasal sinuses. Isolated orbital involvement by NKTL is extremely rare, and only few case reports exist in the literature. Herein, we report a case of primary orbital NKTL in a young patient who showed very good response to chemotherapy with the SMILE regimen.
  
  
  METHODS
  A 28-year-old healthy female presented to us with epiphora and fullness of the lower eyelid. She had previously been diagnosed as orbital pseudotumor and was prescribed oral steroids. However, there was no response to the treatment. She also had a history of recurrent sinusitis. An incision biopsy revealed NKTL. A systemic workup did not reveal any other site of involvement. The patient received 6 cycles of chemotherapy (SMILE) and external beam radiotherapy.
  
  
  RESULTS
  The tumor showed complete regression after 2 cycles of chemotherapy. The patient is disease free at 24 months of follow-up.
  
  
  CONCLUSIONS
  Reported median survival for primary orbital NKTL is extremely poor. This is perhaps the first report of a primary orbital NKTL demonstrating a remarkable treatment response to a newer chemotherapy regimen (SMILE) in combination with radiotherapy.
authors.affiliation: Oculoplasty and Ocular Oncology Services, Dr. R.P. Centre for Ophthalmic Sciences, All India Institute of Medical Sciences, New Delhi, India.
authors.forename: Rachna
authors.initials: R
authors.lastname: Meel
authors.affiliation: Oculoplasty and Ocular Oncology Services, Dr. R.P. Centre for Ophthalmic Sciences, All India Institute of Medical Sciences, New Delhi, India.
authors.forename: Rebika
authors.initials: R
authors.lastname: Dhiman
authors.affiliation: Oculoplasty and Ocular Oncology Services, Dr. R.P. Centre for Ophthalmic Sciences, All India Institute of Medical Sciences, New Delhi, India.
authors.forename: Meenakshi
authors.initials: M
authors.lastname: Wadhwani
authors.affiliation: Ocular Pathology Services, Dr. R.P. Centre for Ophthalmic Sciences, All India Institute of Medical Sciences, New Delhi, India.
authors.forename: Seema
authors.initials: S
authors.lastname: Kashyap
authors.affiliation: Ocular Radiology Services, Dr. R.P. Centre for Ophthalmic Sciences, All India Institute of Medical Sciences, New Delhi, India.
authors.forename: Sanjay
authors.initials: S
authors.lastname: Sharma
authors.affiliation: Oculoplasty and Ocular Oncology Services, Dr. R.P. Centre for Ophthalmic Sciences, All India Institute of Medical Sciences, New Delhi, India; Dr. B.R. Ambedkar Institute Rotary Cancer Hospital, New Delhi, India.
authors.forename: Ajay
authors.initials: A
authors.lastname: Gogia
keywords: Lymphomas; Natural killer T-cell lymphoma; Non-B-cell lymphomas; SMILE regimen