Trilateral Retinoblastoma: A rare case report
International Conference on Pathology and Surgical Pathology
September 06-07, 2018 | Edinburgh, Scotland
Sai Kumar Maley, Rahul Dev Singh, Uma Nagendra Vishnu, Chanakya Charan Tirumala, Siddartha Reddy Musali and Sandeep Lakkarasu
Osmania Medical College, India
Posters & Accepted Abstracts : J Pathol Dis Biol
Abstract:
Trilateral retinoblastoma syndrome (TRB) is a rare condition, it is characterized by intra cranial neoplasm in the pineal region or Sella/suprasellar region along with a unilateral or a bilateral retinoblastoma. The intra-cranial neoplasm can occur after many years in post treatment phase of successfully treated ocular retinoblastoma. Here we present a case of a 9months old baby girl brought by her mother with complaints of vomiting, loss of eye contact and one episode of seizure. On thorough evaluation it was found that both the eyes were showing leukocoria and fundoscopic examination revealed tumour in both the eyes. Right fundus showed exudative retinal detachment and left fundus was not visualized. Subsequent MRI of brain revealed showed moderately enhancing T1 hypo & T2 isointense lesions in the posterior aspects of both the globes; 1.7x0.6cm on right side and 2x1.6cm on the left side with extension up to optic nerve heads bilaterally and up to lens on left side noted. Large intensely enhancing suprasellar and sellar mass measuring 5.4x4.5x4.8cm with focal cystic changes and calcifications seen exerting mass effect on 3rd ventricle causing obstructive hydrocephalus. Nodular Dural metastasis along brainstem cisterns, cerebellar occipital surface and the spinal canal in the dorso-lumbar region noted. Intra-cranial mass was partially removed, and the histopathology showed Homer- Wright rosettes and Flexner Winter Steiner rosettes and immunohistochemistry w a positive suggesting feature of retinoblastoma. A possibility of direct intracranial extension was considered as both the optic nerve heads were involved. Such kind of cases require a genetic screening, as most of them are hereditary and harbour a worse prognosis over unilateral ocular retinoblastoma.
Biography:
Sai Kumar Maley has completed his residency training in pathology and senior residency from Osmania Medical College. He is interested in research on pulmonary malignancies and his post-doctoral dissertation work was centered on the immunocytochemical diagnosis of pulmonary malignancies in low resource setup’s and his work highlighted an effective and economical usage of immunohistochemical markers and worked on the preanalytical variables influence on the outcomes.
E-mail: sai.maley@gmail.com
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