title: Extraocular retinoblastoma in Indian children: clinical, imaging and histopathological features.
abstract: OBJECTIVE
  TO STUDY EYES WITH EXTRAOCULAR DISSEMINATION (EORB), WITH THE FOLLOWING AIMS: first to establish the mean lag period and to understand various reasons for delayed presentation, second to study their imaging profiles and third to analyze histopathological features of eyes enucleated after neoadjuvant chemotherapy.
  
  
  METHODS
  Prospective study of clinical and imaging features of EORBs (stage III and IV International Retinoblastoma Staging System) presenting to a tertiary eye care centre. Histopathological features of eyes enucleated after receiving neoadjuvant chemotherapy were analyzed. A pictorial illustration of the varied imaging profile of EORB was also presented.
  
  
  RESULTS
  Over a period of one year, 97 eyes were diagnosed with retinoblastoma; 32 children (36 eyes) (37.1%) had EORB. Mean age 3.6±1.9 years, 71.9% males, 71.9% unilateral, 3.1% with positive family history and 40.6% with metastasis. On imaging, there was extrascleral involvement in 22.2%, involvement of orbital part of optic nerve in 33.3%, involvement of central nervous system in 27.8% and orbital wall involvement in 2.9% eyes. On histopathological analysis of eyes enucleated after neoadjuvant chemotherapy, 25.0% had no residual viable tumour tissue and rest all tumours were poorly differentiated.
  
  
  CONCLUSIONS
  There are very few human malignancies where definitive treatment is started without any confirmed histopathological diagnosis and imaging plays an important role in diagnosis and appropriate staging of the disease. Chemotherapy has a variable effect on EORB, 75.0% of eyes with EORB had residual viable tumour tissue when enucleated after receiving neoadjuvant chemotherapy.
authors.affiliation: Oculoplasty and Pediatric Ophthalmology Services, Dr. Rajendra Prasad Centre for Ophthalmic Sciences, All India Institute of Medical Sciences, New Delhi, India.
authors.forename: Sumita
authors.initials: S
authors.lastname: Sethi
authors.forename: Neelam
authors.initials: N
authors.lastname: Pushker
authors.forename: Seema
authors.initials: S
authors.lastname: Kashyap
authors.forename: Sanjay
authors.initials: S
authors.lastname: Sharma
authors.forename: Mridula
authors.initials: M
authors.lastname: Mehta
authors.forename: Sameer
authors.initials: S
authors.lastname: Bakhshi
authors.forename: Saurbhi
authors.initials: S
authors.lastname: Khurana
authors.forename: Supriyo
authors.initials: S
authors.lastname: Ghose
keywords: extraocular retinoblastoma; histopathological features; imaging