Pathology (dr.

Yabut)
Endocrine Pathology ( from Book)
09 January 08

Clinical Features of Diabetes

Type I Type II
- Occur at any age - Older than age 40
- Honeymoon Period: in the initial 1-2 years following manifestation of - With polydipsia, polyuria, obesity
overt type 1 diabetes the exogenous insulin requirement may be - Metabolic derangements are usually mild
minimal to none - Dx is made after routine blood or urine testing in asymptomatic
- Dominated by signs of altered metabolism: polyuria, polydipsia, persons
polyphagia - Non-ketotic Hyperosmolar Coma: elderly individuals who become
- Despite increased appetite, catabolic effects prevail  weight loss and dehydrated secondary to osmotic dieresis and lack adequate water
intake
weakness
- Chemical indices: ketoacidosis, low or absent plasma insulin,
elevated plasma glucose
- Metabolic derangement and insulin need are directly related to
physiologic stress

Complications of Both Types of Diabetes

Cardiovascular Disease Diabetic Nephropathy Diabetic Retinopathy Infections
- MI, renal vascular insufficiency, - Leading cause of end-stage renal - 60-80% of pxs develop this - Enhanced susceptibility to
CVA are the most common cause disease 15-20 years after diagnosis infections of the skin and
of death - Earliest manifestation is the - 4th leading cause of acquired to TB, pneumonia, and
- Appear ≈ 15-20 years after appearance of low amounts of blindness pyelonephritis
hypoglycaemic onset albumin in the urine (≥30mg/day) - Have a predisposition to
- Accounts for 80% of deaths in type or microalbuminuria cataracts and glaucoma
2 diabetes - 80% of type1 and 20-40% of type
2 develop overt nephropathy
with macroalbuminuria
(≥300mg/day) over the next 10-15
years usually accompanied by the
appearance of hypertension
Pancreatic Endocrine Neoplasms
Incidence Clinical Features Morphology Pathogenesis/Etiol Lab Treatment
ogy Finduings
Insulinoma (β-cell - clinical triad: - generally benign - Diffuse - high - Surgical
tumor) - Attacks of - solitary lesions, hyperplasia of circulatin removal of
- most common hypoglycemia although the islets. g levels the tumor
of pancreatic occur with multiple tumors - in neonates of insulin
endocrine blood glucose or tumors and infants. and a
neoplasms levels below ectopic to the - previously high
- responsible 50 mg/dL of pancreas may be insulin-
known as
for the serum encountered glucose
nesidioblasto

2b1 (joy, shar, cams, yna) at goldie 1 of 6

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elaboration of - the attacks - small (often less sis ratio

sufficient consist than 2 cm in - may result from
insulin to principally of diameter) maternal
induce such central - encapsulated diabetes,
clinically nervous - pale to red- Beckwith-
significant system brown nodules Wiedemann
hypoglycemia manifestations located syndrome, and
as confusion, anywhere in the rare metabolic
stupor, and pancreas disorders.
loss of - Histo: look
consciousness remarkably like
; giant islets, with
- the attacks preservation of
are the regular cords
precipitated of monotonous
by fasting or cells and their
exercise and orientation to
are promptly the vasculature.
relieved by - Immuno: insulin
feeding or
can be localized
parenteral
in the tumor
administration
cells
of glucose
- EM: neoplastic β
cells display
distinctive round
granules that
contain
polygonal or
rectangular
dense crystals
separated from
the enclosing
membrane by a
distinct halo.
Zollinger-Ellison - Triad of: - Arise in the - Surgical
Syndrome - Recalcitrant duodenum and removal
(Gastrinomas) peptic ulcer peripancreatic but with
disease soft tissues, e.g., recurrence
- Gastric pancreas
hypersecretion (gastrinoma
- Endocrine cell triangle)
tumor - 25% arises in
elaborating
conjunction with
gastrin
other endocrine
- Ulcers:
tumors  MEN-
- Duodenal and 1 syndrome
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gastric ulcers - multifocal

are often - histology and
multiple ultrastructure
- Intractable similar to normal
to usual intestinal and
modalities of gastric G cells
therapy
- Occur in
unusual
locations,
e.g., jejunum
- Diarrhea 
presenting
symptom in 30%
of px
- 60% 
malignant; 40%
 benign
α cell tumors - Peri- and - Extremely high
(Glucagonomas) postmenopausal plasma glucagon
women levels
- Mild features of
DM
- Migratory
necrotizing skin
erythema
- anemia
δ cell tumors - High plasma
somatostatin
levels
- Features of DM,
cholelithiasis,
steatorrhea, and
hypochlorhydia
VIPoma - Associated with - Watery diarrhea
(Diarrheogenic Islet neural crest - Hypokalemia
Tumor Cell) tumors - achlorhydia
Pancreatic - rare - serotonin-
Carcinoid Tumors producing
Pancreatic - rare - asymptomatic
Polypeptide-
Secreting Islet Cell
Tumors

Adrenal Cortex
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A. Adrenocortical Hyperfunction (Hyperadrenalism)

- Dx: measuring serum level of serum corticotrophin and • Zona glomerulosa – intact
determining urinary steroid excretion after administration of - Clinical Features:
dexamethasone to suppress corticotrophin levels • Central obesity
- Morphology: depends on the cause • Moon facies
• Pituitary – Crooke hyaline change with basophils caused by • Weakness and fatigability
elevated glucocorticoid levels • Hirsutism
• Diffuse Adrenal Cortical Hyperplasia – glands are • Hypertension
enlarged and affected bilaterally • Plethora
• Nodular Adrenal Cortical Hyperplasia – cortex between • Glucose intolerance/diabetes
nodules is identical to diffuse hyperplasia • Osteoporosis
• Adenomas – small, well circumscribed • Neuropsychiatric abnormalities
• Menstrual abnormalities
• Carcinomas – larger, unencapsulated
• Cutaneous striae
• Zona reticularis and fasciculate – atrophic (both the • Delayed wound healing/bruisability
adjacent and contralateral)
Incidence Clinical Features Morphology Pathogenesis/Etiol Lab Treatment
ogy Finduings

Primary Adrenal Neoplasms (Adrenal adenoma, CA, primary cortical hyperplasia)

- 10-20% of - Independent of - Px with unilateral
endogenous corticotrophin neoplasm:
Cushing because the contralateral
syndrome adrenals fxn adrenal cortex is
- Adults: autonomously atrophic vecause
adenomas and of
carcinomas costicotropin
- Children: suppression
Hypercortisolism carcinomas and low levels of
(Cushing corticotropin
Syndrome) Ectopic Costicotropin Secretion by Non-Pituitary Tumors
- 10% of - Associated with - Adrenals are
endogenous small cell CA bilateral and
Cushing of the lungs hyoerplastic
syndrome - Rarely
- Men, 40-60 y/o
associated with
ectopic
secretion of
corticotrophin
-releasing
factor
Cushing Dse - Young adults - 70-80% of - Adrenals are - - Elevated
- F:M  5:1 endogenous bilaterally serum
hypercortisolism hyperplastic corticotro
 Pathology – Endocrine Pathology by Dr. Yabut Page 5 of 6

- Asoociated with pin

a corticotrophin-
producing
pituitary
adenoma
Primary - Women aged 30- - Hypokalemia  - Solitary, small, - Autonomous -
Hyperaldosteronis 50 y/o weakness, encapsulated overproduction of
m paresthesias, adenomas aldosterone
- Characterized by visual occurring more caused by:
chronic excess disturbances, commonly on the - Conn
aldosterone tetany left side syndrome
secretion  Na - Na retention  - May be buried - Idiopathic
retention and K w/in the adrenal hyperaldoster
increases total
excretion  HPN gland and not onism
body Na and
and hypokalemia apparent - Glucocorticoid
expands ECF
externally -remediable
volume 
- Cut surface is hyperaldoster
hypertension
bright yellow  onism
high lipid content
- Lipid laden cells
resemble cells of
zona fasciculate
- Presence of PAS-
reactive,
eosinophilic,
laminated,
cytoplasmic
inclusions known
as
spironolactone
bodies
o 3 distinct syndromes:
Adrenogenital Syndromes  Salt-wasting adrenogenitalism
- Androgen-secreting adrenal cortical neoplasms: androgen-  Simple virilizing adrenogenitalism without
secreting adrenal CA salt-wasting
- Congenital adrenal hyperplasia (CAH):  Nonclassic adrenogoenitalism
o autosomal recessive
o metabolic errors characterized by a deficiency or total lack of a
particular enzyme involved in the biosynthesis of cortical
steroids, i.e., cortisol
o steroidogenesis is channelled into other pathways  increased
production of androgens  virilization - Morphology:
- 21-hydroxylase deficiency o Substantial, bilateral adrenal enlargement
o Defective conversion of progesterone to 11- o Widened cortex and nodular
deoxycorticosterone by 21-hydroxylase (CYP21B) o Appears brown because of lipid depletion
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o Adrenomedullary dysplasia – incomplete migration

of the chromaffin cells to the center of the gland
- Clinical features
o Determined by specific enzyme deficiency
o Abnormalities related to androgen excess vs.
aldosterone and glucocorticoid deficiency
o CAH – any neonate with ambiguous genitalia
o Severe enzyme deficiency can be life-threatening
with vomiting, dehydration, and salt-wasting
o Women may present with delayed menarche,
oligomenorrhea, or hirsutism