Podcast
Questions and Answers
Which adrenal gland zone is primarily responsible for the production of aldosterone?
Which adrenal gland zone is primarily responsible for the production of aldosterone?
In the context of adrenocortical tumors, what condition is most indicative of high cortisol levels?
In the context of adrenocortical tumors, what condition is most indicative of high cortisol levels?
What laboratory finding is indicative of Conn's Syndrome?
What laboratory finding is indicative of Conn's Syndrome?
What is a primary clinical manifestation of pheochromocytoma?
What is a primary clinical manifestation of pheochromocytoma?
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Which of the following conditions is associated with the RET proto-oncogene mutation?
Which of the following conditions is associated with the RET proto-oncogene mutation?
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What percentage of pheochromocytomas is found to be bilateral or multicentric?
What percentage of pheochromocytomas is found to be bilateral or multicentric?
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What imaging technique is considered to have high diagnostic sensitivity for adrenal tumors?
What imaging technique is considered to have high diagnostic sensitivity for adrenal tumors?
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What is the perioperative mortality rate associated with surgery for adrenal tumors?
What is the perioperative mortality rate associated with surgery for adrenal tumors?
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Which of the following symptoms is NOT typically associated with pheochromocytoma?
Which of the following symptoms is NOT typically associated with pheochromocytoma?
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What value indicates a significant aldosterone/renin ratio in Conn's Syndrome?
What value indicates a significant aldosterone/renin ratio in Conn's Syndrome?
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Study Notes
Adrenal Tumors
- Adrenal tumors are commonly not considered.
- Adrenal glands are located above kidneys.
- Adrenal glands have cortex and medulla.
Adrenal Cortex
- Zona Glomerulosa produces aldosterone.
- Zona Fasiculata produces cortisol.
- Zona Reticularis produces DHEA and androstenedione.
Adrenal Medulla
- Contains chromaffin cells, producing epinephrine and norepinephrine.
Adrenocortical Tumors
- 50% of adults, 90% of children are hormonally active.
- Clinical presentation varies depending on secreted hormone.
- Cushing's syndrome can be caused by tumors.
- Conn's syndrome is also caused by tumors.
- Virilism or feminization can occur.
Cushing's
- Incidence is 5 in a million, and F/M ration is 9.
- In 1 out of 5 cases, adrenal gland is the origin.
- Unresponsive to dexamethasone suppression test, especially in low ACTH levels.
- In 95% of cases, the origin is adrenal.
Conn's Syndrome
- No significant phenotypic appearance.
- 70% are adenomas, 30% hyperplasia, and 1% malignancies.
- Hypertension is unresponsive.
- Hypokalemia and hypernatremia commonly occur.
- Plasma aldosterone : renin activity ratio is usually greater than 20.
- Aldosterone level is above 15ng/dL.
Adrenal Medulla Tumors
- Pheochromocytoma
Pheochromocytoma
- 90% are of adrenal origin, 10% are extra-adrenal.
- Extra-adrenal location can be aorta-sympathetic chain.
- Main presenting sign is unresponsive hypertension.
- Other symptoms include headache, fever, palpitation, sweating, nausea, polyuria and weight loss.
Pheo's 10% Rules
- 10% are familial.
- 10% are malignant.
- 10% are of extra-adrenal origin.
- 10% occur in children.
- 10% are bilateral or multicentric.
Pheochromocytoma Syndromes
- Pheochromocytoma can be part of some syndromes like Type 2 multiple endocrine neoplasia (MEN-2), von Hippel-Lindau syndrome, type 1 neurofibromatosis.
- MEN-2 is an autosomal dominant disease caused by a mutation in RET proto-oncogene (on chromosome 10).
- With MEN-2, there is a 50% probability of medullary thyroid cancer and bilateral benign pheochromocytomas.
- A mutation in the VHL gene on chromosome 3 can cause VHL syndrome, leading to retinal angiomas, hemangioblastomas in the CNS, renal cysts and tumors, and pancreatic cysts.
Imaging
- Contrast enhanced CT scan and MRI.
- If there is any doubt, MIBG (Metaiodobenzylguanidine scintigraphy) scan should be considered to improve diagnostic sensitivity.
Management
- Surgery is the definitive treatment.
- Perioperative mortality is around 10%.
- Blood pressure monitoring and control are crucial.
- Alpha-blockers are used as pre-medication.
Persistent Hypertension
- Persistent hypertension after surgery warrants a search for alternative causes.
- Metastatic regions should be assessed if the pathology shows malignancy.
Adrenal Venous Sampling
- Used to identify the side of bilateral adrenal adenoma.
- Used to select predominant side in hormonally active adrenal diseases.
Adrenal Myelopoma
- Autopsy incidence is 0.08-0.4%.
- MRI shows fat density.
- Hormonally inactive.
- Size-related risk of sarcoma component is greater than 6cm, which indicates surgery consideration.
Management of Adrenal Gland Tumors
- Clinical approach is needed for tumors like Cushing's and Conn's.
- If adrenal mass is found incidentally, the nature of the mass must be evaluated.
Management of Adrenal Masses
- If hormonally active, pre-op medication and surgery are indicated.
- If malignancy suspicion is present, pre-op staging and surgery are indicated.
- If size is greater than 4cm, surgery is indicated.
Adrenalectomy
- Open adrenalectomy
- Laparoscopic adrenalectomy
- Robotic adrenalectomy.
Adenomectomy
- Selected cases (no suspicion of malignancy).
- Smaller than 2-2.5 cm.
- Previous contralateral adrenalectomy(VHL).
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