ABSTRACT
CK19 ●● Prognosis depends on tumor size, cellular-
ity, and cytologic atypia
●● When more than 10% of islets are over 200 mm in diameter (infants under 2 months)
●● Islet cell hyperplasia/hypertrophy seen in infants of diabetic mothers
INFANTS OF DIABETIC MOTHER
●● Maternal hyperglycemia and anti-insulin antibodies
●● Maternal insulin and glucagon do not normally cross placenta
●● Glucose and antibodies to insulin normally cross placenta
●● Other associated anomalies: Cardiac, lumbosacral agenesis, macrosomia
phism of β-cell nuclei ●● Eosinophilic insulitis and peri-insular
fibrosis ●● Obese infants, neonatal hypoglycemia,
macrosomia and tendency to develop diabetes mellitus in life
DIABETES MELLITUS (DM)
Type I (insulin-dependent DM, juvenile-onset)
Type II (adult type)
Neonatal DM
mia within first 3 months of life ●● Pancreatic hypoplasia/aplasia ●● Wolcott-Rallison syndrome
Maturity onset diabetes of the young (MODY)
●● Genetic defects in pancreatic β-cell transcription factors
HYPERINSULINISM
Congenital hyperinsulinemia (CHI) with B-cell ATP-sensitive potassium channel abnormalities
●● Recessive inactivating mutations of ABCC8 and KCNJ11 (most frequent CHI)
●● Nesidioblastosis: Histologic finding in hyperinsulinemia (obsolete terminology)
●● Nesidioblastosis (direct transformation of ductal epithelial cells into islet tissue), includes hypertrophic islets with atypical β-cells, ductoinsular complexes
Diffuse hyperinsulinemia
mutations located on chromosome 11p ●● Hypoglycemia at birth ●● Intraoperative frozen section biopsies (from
head, body, and tail); islet cell nucleomegaly/bizarre nuclei with pseudoinclusions
Focal hyperinsulinemia
●● Paternally inherited mutations of genes ABCC8 and KCNJ11
●● Loss of maternal alleles of chromosome 11p15, and tumor suppressor genes
●● Intraoperative frozen section usually shows normal islet cell nuclei
●● Islet cell adenomatous/adenoma lesions; localized increase in islet tissue with nucleomegaly and nuclear pleomorphism
adenomatous lesion ●● Resect the focal lesion only; no need for
total pancreatectomy ●● Better prognosis than diffuse
PANCREATIC ISLETS IN SHOCK
tory genes clustered at 11p15 ●● Macrosomia, macroglossia, omphalocele,
visceromegaly, hemihyperplasia, tumors ●● Hyperinsulinemia-associated hypoglycemia ●● Pancreas contain islet-like aggregates of
endocrine cells (chromogranin A positive) ●● Exocrine acini are poorly developed
PERLMAN SYNDROME
gigantism
WOLCOTT-RALLISON SYNDROME
LEPRECHAUNISM
muscle tissue
