Perinatal and pediatric pathology

1
Perinatal and pediatric pathology
SYLLABUS RBP(Robbins Basic Pathology)
Chapter Genetic and pediatric diseases
2
Perinatal and pediatric pathology

• Retinoblastoma
• Wilms tumor
• 231 Fetal atelectasis
• 24 Hyaline membrane disease
• 76 Medulloblastoma
• 83 Craniopharyngioma

3
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
4
Retinoblastoma
5
Retinoblastoma
6
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)

7
Wilms tumor
8
Wilms tumor
9
Fetal atelectasis

• collapsed alveoli
• - stellate shape of small bronchi

10
Fetal atelectasis
11
Fetal atelectasis
12
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

13
Hyaline membrane disease
14
Hyaline membrane disease
15
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)

16
Medulloblastoma
17
Medulloblastoma
18
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

19
Craniopharyngioma
20
Craniopharyngioma