Block 2 PATH Exam -- (Neuro)Endocrine Neoplasia Lecture
Terms
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- Primary hyperparathyroidism
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Excess PTH in absence of stimulus
Adenoma is cause of 80% of this (single gland involved)
Hyperplasia involves all 4 glands (weight is very important)
Therapy is to remove parathyroid tissue - Secondary hyperparathyroidism
- Hypocalcemic stimulation of excess PTH
- Parathyroid Carcinoma
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Usually high serum calcium level
30% will have mets
Therapy is usually en bloc resection
Death is usually from hypercalcemia - Hypoadrenalism
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Primary chronic insufficiency -- Addison's disease (auto-immune)
Primary acute insufficiency -- crisis in Addison's - Conn's Syndrome
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Hyperaldosteronism
10% have hyperplasia of zona glomerulosa
90% have adenoma - Cushing's syndrome
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Excess corticotropin
Can be from pituitary or within adrenal gland or iatrogenic
Iatrogenic (too much steroid medication) - Adrenal Adenoma
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May secrete aldo, cortisol or sex steroids
Solitary, small and bright yellow - Pheochromocytoma
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Secretes Epi and NE
10% are bilateral, 10% are extra-adrenal
40-70% of familial are bilateral
10% are malignant - Neuroblastoma
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Most common malignant congenital tumor
Increased urinary homovanillic acid, vanillymandelic acid - Chemodectomas
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Carotid and aorticopulmonary paragangliomas
Zellballen -- distinctive cell nests - Z-E Syndrome
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Pancreatic, but can occur elsewhere (duodenum)
High gastric acid secretion, high plasma gastrin
65% are malignant - MEN-I
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Menin tumor suppressor gene
Chromosome 11q
Neoplasms/Hyperplasias of PT gland, adrenal cortex, pituitary, and pancreas
Peptic ulcers
Gastric hypersecretion - MEN-IIa
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A.K.A. Sipple Syndrome
Multiple pheochromocytomas
Medullary carcinoma of the thyroid
PT hyperplasia/adenoma - MEN-IIb
- Same as IIa, WITHOUT PT abnormality, but WITH mucucutaneous neuromas
- MEN-II
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RET proto-oncogene
Chromosome 10q11