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Perinatal and pediatric pathology. SYLLABUS: RBP( Robbins Basic Pathology ) Chapter : Genetic and pediatric diseases. Perinatal and pediatric pathology. Retinoblastoma Wilms tumor 231 Fetal atelectasis 24 Hyaline membrane disease 76 Medulloblastoma 83 Craniopharyngioma.
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Perinatal and pediatric pathology SYLLABUS: RBP(Robbins Basic Pathology) Chapter: Genetic and pediatricdiseases
Perinatal and pediatric pathology • Retinoblastoma • Wilms tumor 231Fetal atelectasis 24Hyaline membrane disease 76Medulloblastoma 83Craniopharyngioma
Retinoblastoma - sheets of neoplastic small round cells with hyperchromatic nuclei, scant cytoplasm and many mitoses - extensive areas of ischemic necrosis and calcification with nuclear debris, sparing perivascular regions - rosettes helpful to strengthen diagnosis but not always seen: - Flexner-Winterstein with small lumen - Homer-Wright (neuroblastomatous) with central neuropil
Wilms tumor • usually three cellular components: blastema, mesenchymal, epithelial (proportions may vary) • blastema- very cellular with primitive round to oval cells with little cytoplasm • mesenchymal cells usually myxoid and spindled but may form smooth or skeletal muscle • epithelial cells form primitive tubules and glomeruli (can have papillary or fibroadenomatous architecture or be small and round)
Fetal atelectasis • collapsed alveoli • - stellate shape of small bronchi
Hyaline membrane disease • alveoli poorly developed, and those present collapsed • eosinophilic hyaline membranes lining the respiratory bronchioles, alveolar ducts, and random alveoli • the membranes are largely made up of fibrinogen and fibrin admixed with cell debris derived chiefly from necrotic type II pneumocytes • paucity of neutrophilic inflammatory reaction • congestion
Medulloblastoma • Small round blue cell tumour with substantial nuclear atypia • densely packed cells, may resemble normal lymphocytes or small cell carcinoma • scant eosinophilic fibrillar background • variable mitotic rate • frequent apoptosis, less commonly geographic necrosis. • may have Homer-Wright rosettes (cleared area of neuropil with no lumen)
Craniopharyngioma • palisading lines of cuboidal to columnar cells separated by stellate cells in a myxoid strom • architecture variable: sheets, whorls, trabeculae, cloverleafs, cysts • many histiocytes, calcification, nodules of eosinophilic ghost cells or „wet keratin”