Adrenal gland Flashcards
3 layers of adrenal (outside to inside)?
Some syndromes associated with adrenocortical tumors?
Syndromes w/ pheochromocytomas?
Glomerulosa (aldosterone, salt), Fasiculata (coritsol/sugar), Reticularis (androgens/sex), Medulla (epinephrine)
Bechwith-Wiedmann, Li-Fraumeni- MEN1, McCune Albright
VHL, NF1, MEN2
Myolipoma findings?
Age and associations?
Treatment?
Benign tumor of fat and hematopoietic cells (see all 3 cell lines); gross see fat
Mostly on right side, ~50 years
Right side, HTN, atherosclerosis, diabetes
Observation
Adrenocortical adenoma (gross and microscopic findings?)
Is nuclear atypia a sign of malignancy, what about pigment?
Treatment?
IHC?
Circumscribed yellow mass, Fat laden cells (resemble fasciulata).
NO!; atypia can be seen as can pigment
Observation vs removal
Melan-A/MART-1 +, Synaptophysin +, Inhibin +
Pheochromocytoma (gross, microscopic) findings?
Cell of origins and symptoms?
Maliganancy risk–sites it mets to?
Treatment?
IHC?
Circumscribed brown-red mass, Medulla cells (blue nested cells, zellballin)?
Chromaffin: HTN, headaches, palpitations, and sweating
2-26% malignant (size >5, invasion, necrosis, atypia, high mitotic rate, LN, bone , liver lung
Adrenalectomy
Chromogranin A, S-100 (sustentacular cells)
Pheochromocytoma, familiar variant findings?
Other associations (3 and their findings)?!
Yes, younger males, bilateral, muilticentric
VHL: Auto dom, Chromo 3, Pheochromocytomas, Clear cell RCC, retinal angioma, pancreatic cysts, CNS hemangioblastomas, and epidiymal cystadenomas
NF1: Chromo 17, Auto dom, Pheo, neurofibromas, cafe au lait, Lisch nodules (iris nodules), Optic brain gliomas, MPNST, schwanoma, menigioma
MEN2: RET2 proto-onogene, AD
2a: Pheo, medullary thyroid, parathyroid hyperplasia
2b: Pheo, Medulary thyroid, mucosal neuromas, ganglioneuromas
VHL?
VHL: Auto dom, Chromo 3, Pheochromocytomas, Clear cell RCC, retinal angioma, pancreatic cysts, CNS hemangioblastomas, and epidiymal cystadenomas
NF1: Chromo 17, Auto dom, Pheo, neurofibromas, cafe au lait, Lisch nodules (iris nodules), Optic brain gliomas, MPNST, schwanoma, menigioma
MEN2: RET2 proto-onogene, AD
2a: Pheo, medullary thyroid, parathyroid hyperplasia
2b: Pheo, Medulary thyroid, mucosal neuromas, ganglioneuromas
NF1 (von Recklinghausen’s disease)
NF1: Chromo 17, Auto dom, Pheo, neurofibromas, cafe au lait, Lisch nodules (iris nodules), Optic brain gliomas, MPNST, schwanoma, menigioma
MEN2 (A and B)
MEN2: RET2 proto-onogene, AD
2a: Pheo, medullary thyroid, parathyroid hyperplasia
2b: Pheo, Medulary thyroid, mucosal neuromas, ganglioneuromas
Adrenocortical carcinoma (maligant or benign, gross and micro findings”?
Who gets this?
How to seperate from adrenalcortical adenoma?
IHC
Gross tan-yellow to orange mass with necrosis, micro: eosiniophilic cytoplasm, pleomorphic cells/atypia)
Rare; F>M, ~45 yrs old; Mets Common
Hard/impossible without invasion
Melan-A/MART-1 +, Synanaptophysin +, Inhibin +
Treatment: adrenalectomy
Adrenocortical carcinoma associated syndromes:
MEN-1
Li-Fraumeni
Bechwith-Wiedemann
MEN-1: AD, Pituitary, parathyroid, and pancreas neuroendocrine tumors
Li-Fraumeni: p53 mutation, AD, Acute leukemia, premenopausal breast carcinoma, brain tumors, adrenocortical tumors, bone and soft-tissue sarcomas
Bechwith-Wiedemann: IGF2 gene, Wilms, macroglossia, abdominal wall defects, visceromegaly, hemihyperplasia, ear lobe creases, high birth weight/ length
