Medicine an Surgery notes

Apr 25th, 2015
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RETINOBLASTOMADefinition:Malignant tumor of the embryonic neural retina.Common congenital tumour arising from theneurosensory retina in one or both eyesAffects children under five yrs.EpidemiologyMost common primary malignant intraocular tumourof childhoodOccur 1 in 20,000 live births. [1 in 14000 - 1 in20000] Age- Though congenital, it is not recognised atbirth, and is usually seen btwn 1 & 2 yrs {ave ageof Dx 15 months for B/L, 25 Months for U/L}Sex- There is no sex predisposition.Race- No racial predilectionClassification:Three overlapping methods for classifyingretinoblastoma:Laterality: Tumors may be unilateral or bilateral Focality: Tumors may be unifocal or multifocal Genetics: Tumors may be hereditary ornonhereditaryThe disease may be familial or sporadic.ContBilateral -25%Hereditary,~40%multifocalUnilateral- 15%RetinoblastomaUnilateral- 85%Non-hereditary~60%UnifocalBilateral- 15%Trilateral Retinoblastoma Trilateral retinoblastoma is a well-recognizedsyndrome that occurs in children under the age of 5years. It consists usually of bilateral hereditaryretinoblastoma associated with an intracranialneuroblastic tumor of the pineal gland Occurs in approximately 515% of children withfamilial, multifocal, or bilateral retinoblastoma. With the onset of systemic neoadjuvantchemotherapy, the incidence of this syndromeappears to be decreasing.PathogenesisMali

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