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General Pathology > Endocrine Pathology > Flashcards

Endocrine Pathology Flashcards

1
Q

Describe the pituitary gland

A

-Small bean-shaped gland at base of brain within sella turcica
-Composed of two distinct lobes:
1. Anterior pituitary = “Adenohyphysis”
2. Posterior pituitary = “Neurohypophysis”

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2
Q

Describe the two lobes of the pituitary gland

A
  1. Anterior pituitary =
    Adenohypophysis”
    -Develops from epithelial cells derived embryologically from oral cavity (posterior pharynx)
  2. Posterior pituitary = “Neurohypophysis”
    -Develops from the floor of the diencephalon
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3
Q

What is the function of the pituitary gland?

A

Regulation of most endocrine glands via hormones (hypothalamus controls the release of hormones from pituitary gland)

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4
Q

List the anterior pituitary hormones

A
  1. TSH - thyroid stimulating hormone
  2. FSH - follicle stimulating hormone
  3. LH - luteinizing hormone
  4. GH - growth hormone
  5. ACTH - adrenocorticotrophic hormone
  6. PRL - prolactin

*produced in anterior pituitary cells –> Hypothalamic control

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5
Q

Describe TSH

A

Thyroid stimulating hormone –> thyroid gland
-stimulatory; controlled by TRH thyrotropin releasing hormone

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6
Q

Describe FSH

A

Follicle stimulating hormone –> ovaries/testes
-stimulatory; controlled by GnRH gonadotropin releasing hormone

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7
Q

Describe LH

A

Luteinizing hormone –> ovaries/testes
-stimulatory; controlled by GnRH gonadotropin releasing hormone

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8
Q

Describe GH

A

Growth hormone –> fat/muscle/bones/liver
-stimulatory; controlled by GHRH growth hormone releasing hormone
-inhibitory; controlled by GIH growth hormone inhibitory hormone

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9
Q

Describe ACTH

A

Adrenocorticotrophic hormone –> adrenal cortex
-stimulatory; controlled by CRH corticotropin releasing hormone

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10
Q

Describe PRL

A

Prolactin –> mammary glands
-inhibitory; controlled by PIF prolactin inhibitory hormone/dopamine

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11
Q

List the posterior pituitary hormone

A
  1. ADH
  2. Oxytocin

*Produced in hypothalamic neurons and stored in posterior pituitary –> hypothalamic control

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12
Q

Describe ADH

A

Anti-diuretic hormone –> kidney
-promotes resorption of water in collecting tubules of kidney

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13
Q

Describe Oxytocin

A

Oxytocin hormone –> uterus
-promotes contraction of smooth muscle in pregnant uterus

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14
Q

What is the most common cause of hyperpituitarism?

A

Functional Adenoma

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15
Q

What is the pathogenesis and histology of Adenomas?

A

Pathogenesis: G-protein mutations (most common)

Histology: Single cell type - producing single hormone

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16
Q

Describe Craniopharyngioma

A

Most common pituitary tumor of CHILDHOOD (rare)

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17
Q

What is the pathogenesis of Craniopharyngioma? The histology? The clinical/oral signs?

A

Pathogenesis: Neoplasia

Histology: resembles odontogenic tumors

Clinical/Oral:
-usually in children*
-trouble walking*
-loss of balance
-slow growth
-fatigue
-vomiting

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18
Q

Describe Giantism

A

Enormous growth in stature before bone epipyhses close

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19
Q

What is the pathogenesis of Giantism? The histology? The clinical/oral signs?

A

Pathogenesis: Functioning adenoma in CHILDHOOD

Histology:
-pituitary cell type = somatotroph
-hormone produced = increased GH

Clinical/Oral:
-short lifespan (increased cardiac output/heart failure)*
-height usually over 7ft*
-proportionally large

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20
Q

Describe Acromegaly

A

Increase in appositional bone growth and organ growth (changes occur very slowly - bony epiphyses are closed)

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21
Q

What is the pathogenesis of Acromegaly? The histology? The clinical/oral signs?

A

Pathogenesis: Functioning adenoma in ADULTHOOD

Histology:
-pituitary cell type = somatotroph
-hormone produced = increased growth hormone

Clinical/Oral:
-mandibular prognathism*
-macroglossia*
-new diastema develops*
-large hands/feet - normal height*
-patients develop hypertension and CHF

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22
Q

Describe Pituitary Dwarfism

A

Proportionate decrease in height when bony epiphyses are open

(Don’t confuse this with achondroplastic dwarfs (genetic))

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23
Q

What is the pathogenesis of Pituitary Dwarfism? The histology? The oral/clinical signs?

A

Pathogenesis: Pituitary hyposecretion of GH in CHILDREN (trauma; autoimmune; congenital; tumor)

Histology: decreased hormone produced = decreased growth hormone

Oral/Clinical:
-small teeth and jaws *
-proportionate small stature *

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24
Q

Describe Simmond Disease

A

Atrophy or decreased function of pituitary gland target organs

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25
Q

What is the pathogenesis of Simmond Disease? The histology? Clinical/Oral signs?

A

Pathogenesis: Anterior pituitary failure

Histology: Decreased GH, LH, FSH, TSH, ACTH, PRL

Clinical/Oral:
-anorexia and muscle weakness*
-hypotension; hypoglycemia; anemia*
-dry skin and loss of pubic/axillary hair*
-atrophy of genitalia and breasts*
-premature senility*

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26
Q

Describe Sheehan Postpartum Necrosis

A

Atrophy or decreased function of pituitary gland target organs after birth

*most common form of anterior pituitary necrosis

(progresses to symptoms of simmond disease is not treated)

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27
Q

What is the pathogenesis of Postpartum Necrosis? The histology? The clinical/oral signs?

A

Pathogenesis: excessive blood loss during delivery –> ischemia = necrosis of anterior pituitary

Histology: Decreased GH, LH, FSH, TSH, ACTH, PRL

Oral/Clinical:
-no lactation*
-amenorrhea*
-weakness and lethargy*
-anemia*

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28
Q

Describe Diabetes Insipidus

A

Imbalance of fluids with defect in urine concentration (nothing to do with blood sugar!)

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29
Q

What are the oral/clinical signs of Diabetes Insipidus? The histology?

A

Clinical/Oral:
-Polyuria* (increased urination - 5 L/day vs 2 L/day)
-Polydipsia* (increased thirst)
-Hypotension

Histology: Decreased ADH secretion or decreased kidney response

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30
Q

What is Diabetes Insipidus associated with? (4)

A

1) Craniopharyngioma
2) Langerhans cell disease
3) Head trauma
4) Idiopathic

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31
Q

What are the 3 distinct cell types seen in the anterior pituitary

A

1) Cells with basophilic cytoplasm
2) Cells with eosinophilic cytoplasm
3) Cells with chromophobic cytoplasm

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32
Q

Describe the histology of the posterior pituitary

A

-Modified glial cells = pituicytes
-Axon processes extending from hypothalamus

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33
Q

Describe Thyroid Gland development

A

-Located below and anterior to larynx
-Develops from tubular evagination of pharyngeal epithelium descending from foramen cecum
-Thyroglossal duct disappears or may remain as a central pyramidal lobe
-Ectopic thyroid tissue is most often located at base of tongue = lingual thyroid

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34
Q

What is the function of the thyroid gland?

A

-Regulation of metabolism and basic body functions
-Controlled by hypothalamus and anterior pituitary gland

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35
Q

Describe the histology of the thyroid gland

A

-Thyroid is developed into lobules –> each with 20-40 follicles
-Follicular cells produce and store thyroglobulin as a colloid
-Clusters of C-cells are between follicles and produce calcitonin

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36
Q

Thyroid follicles are filled with ______ and lined with _______

A

colloid; simple cuboidal epithelial cells (follicular cells)

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37
Q

Describe the thyroid hormones produced by follicular cells and stored as Thyroglobulin

A

1) T4 - Thyroxine –> brain, bone, heart, muscles
-Hypothalamus releases TRH = stimulates anterior pituitary to release TSH = activates G-protein –> cAMP released –> stimulates thyroglobulin conversion to T4 (iodine is needed!)

2) T3 - Triiodothyronine –> brain, bone, heart, muscles
-Hypothalamus releases TRH = stimulates anterior pituitary to release TSH = activates G-protein –> cAMP released –> stimulates thyroglobulin conversion to T3 (iodine is needed!)

*Negative feedback control

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38
Q

Describe the thyroid hormones produced by parafollicular C-cells

A

Calcitonin –> bone
-Controlled by increase in blood calcium

*Negative feedback control

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39
Q

What are the functions of T3 and T4?

A

Increased:
-Metabolic rate
-Heat production
-Appetite
-GH secretion
-Alterness/reflexes

-After release –> most free T4 is de-iodinated to T3
-T3 binds to receptors in target tissues with 10x greater affinity

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40
Q

What are the functions of Calcitonin

A

-Increased bone formation
-Stimulates osteoblastic activity
-Produced and released when blood calcium levels are high

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41
Q

Describe Thyroglossal Duct Cyst

A

Developmental cyst located anterior and superior to larynx

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42
Q

What is the pathogenesis of a Thyroglossal Duct Cyst? The histology? The oral/clinical signs?

A

Pathogenesis: Remnants of thyroglossal duct form cyst

Histology: Thyroid follicle is cyst wall

Oral/Clinical:
-soft/fluctuant mass at midline
-elevates when swallowing
-elevates when tongue protruded
-usually in children; under 20 yrs

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43
Q

Describe diagnostic thyroglossal duct cyst

A

Thyroid follicles in cyst wall

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44
Q

Describe a Lingual Thyroid

A

Ectopic thyroid tissue at base of tongue near foramen cecum

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45
Q

What is the pathogenesis of lingual thyroid? The histology? The oral/clinical signs?

A

Pathology: possible hypothyroidism

Histology: normal thyroid tissue

Oral/Clinical:
-vascular swelling near foramen cecum
-women (4:1) - any age
-dysphasia, sore throat
-bleeding possible

**Must refer for testing before biopsy

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46
Q

Describe Cretinism

A

Hypothyroidism in infants/children

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47
Q

What is the histology of Cretinism? The oral/clinical signs?

A

Histology: Decreased T3 and T4

Oral/Clinical:
-macroglossia
-delayed tooth eruption
-short stature; round face
-wide-spaced eyes; flat nasal bridge

** causes permanent mental retardation if not treated

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48
Q

What is Cretinism associated with?

A

-Iodine deficiency
-Aplasia
-Genetic mutation

49
Q

Describe Myxedema

A

Hypothyroidism in adults

50
Q

What is the histology of Myxedema? Oral/clinical signs?

A

Histology: Decreased T3 and T4 or decreased TSH

Oral/Clinical:
-macroglossia
-decreased metabolic rate - decreased RR and HR
-weight gain; cold intolerance; dry skin

**Causes reversible mental sluggishness and apathy

51
Q

What is Myxedema associated with?

A

-Hashimoto disease
-Iodine deficiency
-Goiter
-Surgical ablation

52
Q

Describe Hashimoto Disease

A

Progressive hypothyroidism in adults

53
Q

What is the pathogenesis of Hashimoto disease? The histology? The oral/clinical signs?

A

Pathogenesis: Autoimmune disease –> auto-antibodies induce T-cells to destroy thyroid tissue = loss of follicular cells

Histology:
-Lymphocytic infiltrate; loss of follicular cells
- Decreased T3/T4 levels

Oral/Clinical:
-Symmetrical enlargement of thyroid
-Females (10:1) - 45-65yrs
-Genetic predisposition

**Associated with other auto-immune diseases

54
Q

Describe Adenoma

A

Benign, encapsulated neoplasm of follicular epithelium

**Most are non-functional (don’t produce hormone)

*Toxic adenoma produces increased T3/T4 (rare)

55
Q

What is the pathogenesis of adenoma? The histology? The oral/clinical signs?

A

Pathogenesis: neoplasia

Histology: resembles normal thyroid parenchyma

Oral/Clinical:
-usually solitary tumor
-rarely turns malignant

*Difficult to distinguish adenoma from goiter or malignancy

56
Q

Describe Papillary Carcinoma

A

-Most common thyroid malignancy
-Grows slowly
-Spreads to cervical nodes

57
Q

What is the pathogenesis of Papillary Carcinoma? Histology? Oral/clinical signs?

A

Pathogenesis: non-functional malignant neoplasia

Histology: Orphan Annie nuclei (clear nuclei, well formed papillae)

Oral/Clinical:
-painless mass in thyroid
-painless cervical node swelling
-any age
-history of previous radiation exposure

*good prognosis –> 95% 10yr survival

58
Q

Describe Medullary Carcinoma

A

Thyroid malignancy of C-cells

59
Q

What is the pathogenesis of Medullary Carcinoma? Histology? Oral/Clinical signs?

A

Pathogenesis: Functional malignant neoplasia, secretes calcitonin

Histology: Amyloid deposits

Oral/Clinical:
-painless mass in neck
-dysphagia; hoarseness
-adults 50-60yrs

**children/young adults if MEN 2A/2B (an inherited syndrome)

60
Q

What is Medullary Carcinoma associated with?

A

Hereditary –> MEN 2A/MEN 2B

61
Q

Describe Multinodular Goiter

A

Nodular enlargement of thyroid gland

*Can present as: euthyroid - hypothyroid - hyperthyroid

62
Q

What is the pathogenesis of Multinodular Goiter? Histology? Oral/Clinical signs?

A

Pathogenesis: Increased TSH secretion

Histology: Follicular cell hypertrophy

Oral/Clinical:
-asymmetrical large mass on thyroid
-dysphasia
-possible airway obstruction

63
Q

Describes Graves Disease

A

Most common form of hyperthyroidism

64
Q

What is the pathogenesis of Graves Disease? Histology? Oral/Clinical signs?

A

Pathology: Autoimmune disease –> auto-antibodies mimic TSH = increased T3 and T4

Histology: Hyperplastic follicular cells - watery colloid

Oral/Clinical:
-symmetric enlargement of thyroid
-females (7:1) - 20-40yrs
-exophthalmos
-increased metabolism - increased HR, RR and BP, weight loss
-nervousness, diarrhea; sweating

65
Q

Describe Goiter

A

Refers to any enlargement of thyroid tissue. Associated with:
-hypothyroidism
-hyperthyroidism
-normal thyroid function

66
Q

Describe Aplasia

A

Developmental failure of thyroid to form associated with hypothyroidism

67
Q

Describe Atrophy

A

Shrinkage of thyroid gland. Associated with:
-Simmond disease
-surgery
-hypothyroidism

68
Q

Describe parathyroid gland development

A

-Developmentally derived from pharyngeal pouches
-Typically, 4 tiny tan glands located near left and right thyroid lobes

69
Q

Describe parathyroid gland function

A

-Regulation of calcium homeostasis
-Controlled by blood levels of free (ionized) calcium

**Not controlled by hypothalamus/pituitary hormones

70
Q

Describe parathyroid gland histology

A

-Primarily composed of chief cells with secretory granules containing PTH (parathyroid hormone)
-Adipocytes are found throughout the disease

71
Q

Describe parathyroid hormone (PTH)

A

*Produced and secreted by chief cells

PTH –> kidneys and bone

-Decreased free calcium levels in the blood stimulate the chief cells to produce and secrete PTH
-Increased free calcium levels in the blood inhibit the secretion of PTH

72
Q

PTH functions by:

A

Increased…
-calcium reabsorption in kidney
-active Vit D conversion in kidney
-calcium absorption in GI
-phosphate excretion in kidney
-osteoclastic activity = bone releases Ca

**NO hypothalamic or pituitary gland control of parathyroid

73
Q

Describe Primary Hyperparathyroidism

A

Increased secretion of PTH from parathyroid glands

**Causes increased blood calcium levels and decreased blood phosphate levels

74
Q

What is the pathogenesis of Primary Hyperparathyroidism? Histology? Oral/Clinical signs?

A

Pathogenesis:
-Adenoma - secretes PTH
-Primary hyperplasia - increased PTH

Histology: benign tumor/hyperplasia of parathyroids

Oral/Clinical: “bones-stones-groans-moans”
-painful bones
-kidney stones
-GI pain/peptic ulcers/pancreatitis
-psychic moans/fatigue-depression
-adults- over 50yrs
**loss of lamina dura
**radiolucencies in jaws

75
Q

Describe Parathyroid Adenoma

A

-Solitary, benign
-Single parathyroid gland
-Histology: proliferation of chief cells and decreased adipose tissue

76
Q

Describe Parathyroid Primary Hyperplasia

A

-Enlargement of multiple parathyroid glands
-Histology: chief cell hyperplasia and decreased adipose tissue

77
Q

What is the pathogenesis of Secondary Hyperparathyroidism? Histology? Oral/Clinical signs?

A

Pathogenesis: Renal failure causes phosphate retention = decreased blood calcium = increased PTH production/secretion

Histology: Chief cell hyperplasia and decreased adipose tissue

Oral/Clinical:
-bones-stones-groans-moans
**loss of lamina dura
**radiolucencies in jaws

*Hyperphosphatemia leads to metastatic calcification of blood vessels = ischemic necrosis of skin and other organs (calciphylaxis)

78
Q

Describe Hypoparathyroidism?

A

Decreased production and secretion of PTH –> usually develops secondary to hypocalcemia

79
Q

Decreased blood calcium leads to:

A

1) neuromuscular excitability
2) cardiac arrhythmias –> life threatening

80
Q

What is the pathogenesis of hypoparathyroidism? The clinical/oral signs?

A

Pathogenesis:
1) surgery
2) aplasia
3) autoimmune disease

Oral/Clinical:
-hypoplasia in developing teeth
-perioral paresthesia; seuizures
-Trousseau sign (wrist/hand)
-Chvostek sign (facial nerve)

81
Q

Describe Hypocalcemia

A

Decreased blood calcium levels –> usually the underlying cause of hypoparathyroidism

82
Q

Hypocalcemia presents as:

A

1) Paresthesia
2) Neuromuscular excitability
*Chvostic sign
*Trousseau sign
3) Dental defects
4) Cardiac arrhythmias

83
Q

Describe Hypercalcemia

A

Increased blood calcium levels –> leads to precipitation of calcium in organs (metastatic calcification)

84
Q

What are the causes of Hypercalcemia that increase PTH

A

1) Parathyroid adenoma
2) Renal disease = secondary hyperparathyroidism

85
Q

What are the causes of Hypercalcemia that decrease PTH

A

1) Bone malignancies
2) Malignancies producing increased calcium
3) Granulomatous disease
4) Vitamin D toxicity

86
Q

Describe adrenal gland development

A

-Above and slightly medial to the kidneys
-Develops from 2 distinct embryologic tissues:
1) mesoderm –> forms the adrenal cortex
2) neuroectoderm –> forms the adrenal medulla

87
Q

What controls the adrenal glands?

A

Hypothalamus and Anterior Pituitary gland, renal pathway, stress/fear

88
Q

Describe the histology of the adrenal gland cortex

A

3 layers:
1) Zona glomerulosa –> produces aldosterone
2) Zona fasciculta –> produces cortisol
3) Zona reticularis –> produces steroid sex hormones

89
Q

Describe the histology of the adrenal gland medulla

A

Composed of chromaffin cells (pheochromocytes) –> produce catecholamines

90
Q

List and describe and adrenal cortex hormones

A

1) Aldosterone –> kidney
2) Cortisol –> body organs/tissues
-controlled by ACTH/CRH
3) Androgens/Estrogens –> ovaries/testes
-controlled by ACTH/CRH

91
Q

List and describe adrenal medulla hormones

A

Epinephrine/Norepi –> body organs/tissues

92
Q

Describe Cushing Syndrome

A

Excess cortisol levels

93
Q

What is the pathogenesis of Cushing Syndrome? The histology?

A

Pathogenesis:
*Exogenous –> glucocorticoids administered (most common)
*Endogenous:
-hypersecretion of ACTH
-ectopic secretion of ACTH
-ACTH-producing malignant tumor

Histology:
*Exogenous –> bilateral cortical atrophy
*Endogenous –> bilateral hyperplasia or neoplasia

94
Q

What is an adrenal crisis?

A

Adrenal gland is unresponsive to ACTH

*Pt’s on exogenous steroids develop adrenal atrophy –> if discontinued suddenly –> possible adrenal crisis
*Most pt’s regain adequate Cortisol response to stress w/in 2 weeks after cessation of steroid therapy
**Long term steroids taken every other day = not at risk for adrenal crisis

95
Q

What do the sign/symptoms of cushing syndrome reflect?

A

increased glucose and decreased protein

96
Q

What are the oral/clinical signs of cushing syndrome?

A

-HTN, weight gain
-Truncal obesity; ‘moon facies’, ‘buffalo hump’
-Thin skin, bruising, cutaneous striae
-Decreased muscle mass
-Hypergycemia - secondary diabetes
-Gastric ulcers; GI bleeds
-Poor wound healing; increased infections
-Osteoporosis/compression fractures
-Mood swings/psychosis

97
Q

Describe Conn Syndrome

A

Chronic increased aldosterone levels with decreased renin

98
Q

What is the pathogenesis of Conn syndrome? The histology? The oral/clinical signs?

A

Pathogenesis:
1) Bilateral hyperplasia of adrenal cortex
2) Aldosterone-producing adenoma

Histology:
1) Diffuse hyperplasia
2) Adenoma –> spironolactone bodies

Oral/Clinical:
-HTN
-Hypokalemia –> weakness, paresthesia
-Children/young adults

99
Q

Describe Secondary Hyperaldosteronism

A

Increased aldosterone and increased renin

100
Q

Describe 21-Hydroxylase Deficiency

A

Chronic increased androgen levels

101
Q

What is the pathogenesis of 21-Hydroxylase Deficiency? The histology?

A

Pathogenesis: Congenital adrenal hyperplasia

Histology: Bilateral hyperplasia of the adrenal cortex

**21-hydroxylase is needed to make mineralocorticoids and glucocorticoids. Deficiency = decreased cortisol and decreased aldosterone production = cortisol/aldosterone deficiency. Substances for cortisol/aldosterone will build up –> forms excessive androgen

102
Q

What are the oral/clinical signs of 21-Hydroxylase Deficiency?

A

-Masculinization in females (clitoral hypertrophy/hirsutism)
-Pseudo-hermaphroditism in female infants
-Enlargement of external genitalia in young men
-Oligospermia in men - but they are fertile
-Cortisol deficiency and aldosterone deficiency

103
Q

Describe Bilateral Hemorrhagic Necrosis

A

Acute adrenocortical insufficiency = acute adrenal crisis

*No glucocorticoids/no mineralocorticoids/no catecholamines

104
Q

What is the pathogenesis of Bilateral Hemorrhagic Necrosis? The histology? The oral/clinical signs?

A

Pathogenesis:
1) Septicemia
2) Anticoagulant therapy
3) Complication of pregnancy
4) DIC

Histology:
-Extensive necrosis of all 3 cortical layer cells
-Extensive necrosis of medullary cells

Oral/Clinical: Initially non-specific symptoms
-abdominal/thoracic pain; fatigue; anorexia; nausea
-leads to acute adrenal crisis

105
Q

Describe the signs and symptoms of Acute Adrenal Crisis

A

-Significant decreased BP
-Hypoglycemia
-Coma
-Death w/o immediate steroid replacement

106
Q

Describe Waterhouse-Friderichsen Syndrome

A

Massive bilateral adrenal hemorrhage specifically associated with overwhelming sepsis causing acute adrenal crisis

107
Q

What is the pathogenesis of Waterhouse-Friderichsen Syndrome? The histology? The oral/clinical signs?

A

Pathogenesis: Neisseria meningitidis bacterial sepsis

Histology: Extensive necrosis

Oral/Clinical:
-children (mimics child abuse)
-acute hypotension
-skin purpura
-coma and death with 48 hours
*60% mortality

108
Q

Describe Addison Disease

A

Primary Chronic Adrenal Insufficiency
-Chronic loss of all cortical hormones and increased ACTH
-Causes decreased BP; increased K; decreased Na; dehydration

109
Q

What is the pathogenesis of Addison Disease? Histology? Oral/Clinical signs?

A

Pathogenesis: Autoimmune adrenalitis

Histology: Atrophy of adrenal cortex

Oral/Clinical:
-oral/skin hyperpigmentation
-hypotension
-salt craving
-anorexia; fatigue; weakness; diarrhea

**Treatment is hormone replacement; high risk for acute adrenal crisis

110
Q

Describe Secondary Chronic Adrenal Insufficiency

A

Chronic decreased ACTH secretion = leads to decreased cortisol and decreased androgens

**NO deficiency of aldosterone / NO hyperpigmentation

111
Q

What is the pathogenesis of Secondary Chronic Adrenal Insufficiency? The histology? The oral/clinical signs?

A

Pathogenesis:
-Metastasis to hypothalamus/pituitary gland
-Infection
-Radiation

Histology: Atrophy of adrenal cortex

Oral/Clinical:
-Anorexia
-Fatigue; weaknes
-Diarrhea

*Tx = hormone replacement; high risk for acute adrenal crisis

112
Q

Describe Pheochromocytoma

A

Functioning tumor of adrenal medulla = increased catecholamines
-Can be benign or malignant; unilateral or bilateral
-25% of pheochromocytomas are associated with syndromes

113
Q

What is the pathogenesis of Pheochromocytoma? The histology? The oral/clinical signs?

A

Pathogenesis: Neoplasia

Histology: Chromaffin cells

Oral/Clinical:
-HTN - 90%
-Episodes of “fight-or-flight” symptoms

*Contraindication for local anesthetic with vasoconstrictor

114
Q

Describe Neuroblastoma

A

Malignant tumor developing from immature nerve cells
-Most often arises in medulla of adrenal glands
-Second most common childhood cancer

115
Q

What is the pathogenesis of a Neuroblastoma? The histology? The oral/clinical signs?

A

Pathogenesis: Malignant neoplasia

Histology: Poorly differentiated neuroblasts

Oral/Clinical:
-Children under 5yrs
-Large abdominal mass
-Fever; weight loss

116
Q

Describe Multiple Endocrine Neoplasia Syndromes

A

Group of autosomal-dominant inherited diseases caused by proliferative lesions of multiple endocrine organs

117
Q

Describe MEN 2B - Multiple Mucosal Neuroma Syndrome

A

Autosomal dominant; single amino acid change in RET gene

118
Q

What are the clinical signs of MEN 2B? Oral?

A

Clinical:
1) Mucosal neuromas
2) Pheochromocytomas
3) Medullary carcinoma of thyroid - 100%

Oral:
-Multiple oral neuromas = 1st manifestation of sx
-Lips/tongue most common sites
-Large thick lips

**Refer to physician

119
Q
A

General Pathology (32 decks)

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