Adrenal Histology Flashcards
causes of hypercortisolism
bilateral adrenal hyperplasia (pituitiary acth, ectopic acth, primary, idiopathic), adenoma, carcinoma
_____ cancer is the most important cause of ectopic acth production
small cell lung cancer
gross features of adrenal corticocarcinoma
large, necrosis, hemorrhage, invasion
poor prognostic factors for adrenal corticocarcinoma
mitotic activity, venous invasion, p53+, Ki67+, large size
Weiss scoring
tumors with a score > 3 are malignant (scored based on histological features)
LiFraumeni
p53
Beckwith-Wiedemann
11p15 –> Wilm’s tumor
familial adenomatous polyposis coli
APC
Multiple endocrine neoplasia type 1
Menin (11q13)
An adenoma that is REALLY big is probably a ___
tumor of uncertain malignant potential : UMP
Female/Male: simple diffuse adrenal hyperplasia
females
Female/Male: bilateral nodular hyperplasia
females
Female/Male: ectopic adrenal hyperplasia
males
Primary cause of hypercortisolism in adults
hyperplasia > adenoma/carcinoma
Primary cause of hypercortisolism in children
carcinoma > hyperplasia > adenoma
Causes of hyperaldosteronism
adenoma> bilateral hyperplasia // carcinoma RARE
Causes of hypoaldosteronism
autoimmune destruction, replacement (tb, fungi, amyloid, tumor)
Causes of adrenal medulla hyperfunction
pheochromocytoma, hyperplasia, neuroblastoma
Clinical triad for pheochromocytoma
paroxysmal hypertension, headache, diapheresis
T/F pheochromocytoma is rare in children
T
Genes associated with familial pheochromocytoma
VHL,RET, NF1, SDHB,SDHD
Rule of ten
pheochromocytomas: 10% bilateral, 10% extraadrenal, 10% malignant
Familial pheochromocytomas are uni/multicentric
multicentric
Most malignant familial pheochromocytomas are of what gene type?
SDHB
__________ lesions of pheochromocytoma or association with medullary hyperplasia suggest genetically linked syndrome and hence lesions in other organs.
bilaterality/multifocal –> especially true if pt <40 //must consider family disease
Pheochromocytoma malignancy is only defined by
metastasis
T/F necrosis, mitoses, and vascular invasion indicate malignancy of pheochromocytoma
F
MEN Syndromes
genetically defined and involve multiple endocrine organs –> may also have lesions in non endocrine systems
3 main MENS
MEN 1, 2A, 2B
Werner syndrome
MEN1: multifocal hyperplasia and neoplasia of Pancreatic islets, Pituitary, Parathyroid (also adrenal cortex, lung, thymus) –> 3Ps
Sipple syndrome
MEN 2A: neoplasia and hyperplasia of thyroid C cells, adrenal medulla, and parathyroids
Gene involved in MEN1
menin
Clinical prognosis of MEN1 is related to
pancreatic lesions –> usually malignant
MEN 2A and 2B involve mutations in ___
ret protooncogene
Most prognostically important lesion in MEN 2A/B
medullary thyroid carcinoma
MEN 2B
neoplasia and hyperplasia of thyroid C cells and adrenal AND neural tissue of oral/GI, skeletal/eye lens abnormalities
MEN Syndromes
genetically defined and involve multiple endocrine organs –> may also have lesions in non endocrine systems
3 main MENS
MEN 1, 2A, 2B
Werner syndrome
MEN1: multifocal hyperplasia and neoplasia of Pancreatic islets, Pituitary, Parathyroid (also adrenal cortex, lung, thymus) –> 3Ps
Sipple syndrome
MEN 2A: neoplasia and hyperplasia of thyroid C cells, adrenal medulla, and parathyroids
Gene involved in MEN1
menin
Clinical prognosis of MEN1 is related to
pancreatic lesions –> usually malignant
MEN 2A and 2B involve mutations in ___
ret protooncogene
Most prognostically important lesion in MEN 2A/B
medullary thyroid carcinoma
MEN 2B
neoplasia and hyperplasia of thyroid C cells and adrenal AND neural tissue of oral/GI, skeletal/eye lens abnormalities
96% of MEN 2B have mutation in codon ___ of ret
918 (intracellular part of ret)
