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Block 2 PATH Exam -- (Neuro)Endocrine Neoplasia Lecture


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Primary hyperparathyroidism
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
Usually high serum calcium level
30% will have mets
Therapy is usually en bloc resection
Death is usually from hypercalcemia
Primary chronic insufficiency -- Addison's disease (auto-immune)
Primary acute insufficiency -- crisis in Addison's
Conn's Syndrome
10% have hyperplasia of zona glomerulosa
90% have adenoma
Cushing's syndrome
Excess corticotropin
Can be from pituitary or within adrenal gland or iatrogenic
Iatrogenic (too much steroid medication)
Adrenal Adenoma
May secrete aldo, cortisol or sex steroids
Solitary, small and bright yellow
Secretes Epi and NE
10% are bilateral, 10% are extra-adrenal
40-70% of familial are bilateral
10% are malignant
Most common malignant congenital tumor
Increased urinary homovanillic acid, vanillymandelic acid
Carotid and aorticopulmonary paragangliomas
Zellballen -- distinctive cell nests
Z-E Syndrome
Pancreatic, but can occur elsewhere (duodenum)
High gastric acid secretion, high plasma gastrin
65% are malignant
Menin tumor suppressor gene
Chromosome 11q
Neoplasms/Hyperplasias of PT gland, adrenal cortex, pituitary, and pancreas
Peptic ulcers
Gastric hypersecretion
A.K.A. Sipple Syndrome
Multiple pheochromocytomas
Medullary carcinoma of the thyroid
PT hyperplasia/adenoma
Same as IIa, WITHOUT PT abnormality, but WITH mucucutaneous neuromas
RET proto-oncogene
Chromosome 10q11

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