Perinatal and pediatric pathology

SYLLABUS:

RBP(Robbins Basic Pathology) Chapter:Genetic and pediatric diseases

Perinatal and pediatric pathology

222 Retinoblastoma

265 Wilms tumor

231 Fetal atelectasis

24 Hyaline membrane disease

76 Medulloblastoma

83 Craniopharyngioma

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

Retinoblastoma

Retinoblastoma

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)

Wilms tumor

Wilms tumor

Fetal atelectasis

- collapsed alveoli

- stellate shape of small bronchi

Fetal atelectasis

Fetal atelectasis

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

Hyaline membrane disease

Hyaline membrane disease

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)

Medulloblastoma

Medulloblastoma

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”

Craniopharyngioma

Craniopharyngioma