CLINICOPATHOLOICAL SPECTRUM OF EMBRYONAL BRAIN TUMORS AND THEIR PROGNOSTIC SIGNIFICANCE – A THREE YEARS INSTITUTIONAL STUDY AT A TERTIARY CARE HOSPITAL IN SOUTH INDIA

DR.R.SHOBIJA; PROF.K.RAMA

Volume : IX, Issue : V, May - 2019

CLINICOPATHOLOICAL SPECTRUM OF EMBRYONAL BRAIN TUMORS AND THEIR PROGNOSTIC SIGNIFICANCE – A THREE YEARS INSTITUTIONAL STUDY AT A TERTIARY CARE HOSPITAL IN SOUTH INDIA

Dr. R. Shobija, Prof. K. Rama

Abstract :

BACKGROUND:Central nervous system tumors constitute second most common paediatric cancers.Emyonal tumors are neoplasms of immature cells resembling primitive neuroepithelium.All are similarly aggressive and have a tendency to disseminate throughout CNS.Therefore identification of specific subtype helps in prognosis evaluation,to avoid unnecessary treatment related neurotoxicity and further treatment implications. METHODS: This is a retrospective study conducted at The Department of neuropathology, Institute of neurosurgery MMC/RGGGH from January 2015 to December 2017.A total of 34 cases of Emyonal tumors were reviewed during this period.Among which Medulloblastoma was reclassified histopathologically based upon the World Health Organisation 2016 classification of CNS tumors.Data on clinical presentation and radiological features of all cases were collected from patients records.In all cases gross features were recorded during grossing of the resected tumors.The tissue sections were processed and stained as per standard protocols. IHC markers were done in deserving cases .Age predilection,Sex Predilection,Tumor location,Comparison with squash and Histological grade in relation to age of emyonal tumors studied. RESULTS:Out of 1422 cases evaluated in adults emyonal tumors comprised 0.21%(3 cases).Out of 150 cases evaluated in chidren aged 16 years emyonal tumors comprised 20.6%(31 cases).Sex ratio(males to females) 2:1 males outnumbering females.94.11%(32 cases) of emyonal tumors presented as posterior fossa tumors,2.94%(1 case) presented as hypothalamic SOL,2.94%(1 case) presented with multiple lesions in spine and cranium–This was a rare case of Atypical Teratoid /Rhabdoid tumor presented with drop metastasis.5.88%(2 cases) presented as recurrent tumors.82.3%(28 cases) presented histologically as classic medulloblastomas,2.94%(1 case) presented as Anaplastic/Large cell type, 2.94%(1 case) presented as Desmoplastic type,11.76%(4 cases) presented as Atypical Teratoid/Rhabdoid tumor.Most commonly affected age group is 6–10 years comprising 50%(17 cases),20.5%(7 cases) affecting 0–5 years, 20.5%(7 cases) affecting 11–16 years. CONCLUSION:Emyonal tumors are highly malignant tumors affecting children from early infancy to adolescence .Because of efforts to avoid craniospinal irradiation in an attempt to lessen treatment related neurotoxicity,diagnosis and management is very important.