Podcast
Questions and Answers
What is the most common type of adrenal incidentaloma?
What is the most common type of adrenal incidentaloma?
All adrenal incidentalomas are functional tumors.
All adrenal incidentalomas are functional tumors.
False
Which test can help rule out malignancy in an adrenal incidentaloma diagnosis?
Which test can help rule out malignancy in an adrenal incidentaloma diagnosis?
FNAC
An adrenal tumor with a diameter greater than ______ cm is suggestive of malignancy.
An adrenal tumor with a diameter greater than ______ cm is suggestive of malignancy.
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Match the following adrenal evaluation methods with their purpose:
Match the following adrenal evaluation methods with their purpose:
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Which of the following is a symptom of hypercalcemic crisis?
Which of the following is a symptom of hypercalcemic crisis?
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An age of 45 years is an indication for surgery in asymptomatic hyperparathyroidism.
An age of 45 years is an indication for surgery in asymptomatic hyperparathyroidism.
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What is the first step in the management of hypercalcemic crisis?
What is the first step in the management of hypercalcemic crisis?
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Post-PTH gland removal, bone influx of $Ca^{2+}$ leads to __________.
Post-PTH gland removal, bone influx of $Ca^{2+}$ leads to __________.
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Match the following conditions with their features:
Match the following conditions with their features:
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What imaging technique is used in the case of discordant imaging for localization of adenoma?
What imaging technique is used in the case of discordant imaging for localization of adenoma?
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The Miami protocol involves checking the PTH level before surgery.
The Miami protocol involves checking the PTH level before surgery.
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What is the primary goal of minimally invasive parathyroidectomy (MIP)?
What is the primary goal of minimally invasive parathyroidectomy (MIP)?
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In open removal of glands, a half gland is auto-transplanted in the __________ of the non-dominant hand.
In open removal of glands, a half gland is auto-transplanted in the __________ of the non-dominant hand.
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Match the surgical procedures with their corresponding descriptions:
Match the surgical procedures with their corresponding descriptions:
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What is the most common cause of secondary hyperparathyroidism?
What is the most common cause of secondary hyperparathyroidism?
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Tertiary hyperparathyroidism occurs after kidney transplantation.
Tertiary hyperparathyroidism occurs after kidney transplantation.
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What is the mainstay treatment for tertiary hyperparathyroidism?
What is the mainstay treatment for tertiary hyperparathyroidism?
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Secondary hyperparathyroidism is commonly associated with _____ hyperplasia.
Secondary hyperparathyroidism is commonly associated with _____ hyperplasia.
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Match the following conditions with their associated findings:
Match the following conditions with their associated findings:
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Which condition is most commonly associated with hypercalcemia in the context of pregnancy?
Which condition is most commonly associated with hypercalcemia in the context of pregnancy?
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DiGeorge syndrome is characterized by a deletion of the 22q12 chromosome.
DiGeorge syndrome is characterized by a deletion of the 22q12 chromosome.
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Name one risk factor associated with parathyroid cancer.
Name one risk factor associated with parathyroid cancer.
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In cases of hypercalcemia, confusion, abdominal pain, and vomiting are considered __________.
In cases of hypercalcemia, confusion, abdominal pain, and vomiting are considered __________.
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Match the following diseases with their corresponding characteristics:
Match the following diseases with their corresponding characteristics:
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Which feature indicates a higher suspicion of malignancy in an adrenal mass?
Which feature indicates a higher suspicion of malignancy in an adrenal mass?
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Pheochromocytoma is more common in males than females.
Pheochromocytoma is more common in males than females.
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What is the most common syndrome associated with familial pheochromocytomas?
What is the most common syndrome associated with familial pheochromocytomas?
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In cases of pheochromocytoma, 10% of tumors are ___ and extra adrenal.
In cases of pheochromocytoma, 10% of tumors are ___ and extra adrenal.
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Match the tumor types with their characteristics:
Match the tumor types with their characteristics:
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What is the most common type of renal stone?
What is the most common type of renal stone?
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Hyperplasia refers to the enlargement of only one parathyroid gland.
Hyperplasia refers to the enlargement of only one parathyroid gland.
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What biochemical changes are observed in primary hyperparathyroidism?
What biochemical changes are observed in primary hyperparathyroidism?
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A patient with primary hyperparathyroidism may have __________ renal stones due to increased calcium levels.
A patient with primary hyperparathyroidism may have __________ renal stones due to increased calcium levels.
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Match the following investigation methods with their purposes:
Match the following investigation methods with their purposes:
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Which gene is associated with multiple endocrine neoplasia type 2A (MEN-2A)?
Which gene is associated with multiple endocrine neoplasia type 2A (MEN-2A)?
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Von Hippel-Lindau (VHL) syndrome is associated with paragangliomas.
Von Hippel-Lindau (VHL) syndrome is associated with paragangliomas.
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What is the histopathological appearance of pheochromocytoma?
What is the histopathological appearance of pheochromocytoma?
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Familial paraganglioma 1 is associated with the gene ______.
Familial paraganglioma 1 is associated with the gene ______.
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Match the following syndromes with their associated lesions:
Match the following syndromes with their associated lesions:
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What is the most common symptom associated with pheochromocytoma?
What is the most common symptom associated with pheochromocytoma?
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Pheochromocytomas predominantly release adrenaline over noradrenaline.
Pheochromocytomas predominantly release adrenaline over noradrenaline.
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Name one confirmatory sign of malignant pheochromocytoma.
Name one confirmatory sign of malignant pheochromocytoma.
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The primary screening test for pheochromocytoma is a 24-hour urinary _______.
The primary screening test for pheochromocytoma is a 24-hour urinary _______.
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Match the following substances with the conditions they are primarily associated with:
Match the following substances with the conditions they are primarily associated with:
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What is the primary function of parathyroid hormone (PTH)?
What is the primary function of parathyroid hormone (PTH)?
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The thymus is derived from the 4th pharyngeal pouch.
The thymus is derived from the 4th pharyngeal pouch.
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What is the mnemonic to remember the clinical features of hyperparathyroidism?
What is the mnemonic to remember the clinical features of hyperparathyroidism?
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The inferior parathyroid gland originates from the __________ pharyngeal pouch.
The inferior parathyroid gland originates from the __________ pharyngeal pouch.
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Match the following conditions with their associated features:
Match the following conditions with their associated features:
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Study Notes
Adrenal Incidentaloma
- Most common cause of adrenal lesions are non-functional tumors
- Metastases from cancers such as breast cancer, lung cancer and renal cancer are possible
- Functional tumors can lead to Cushing's syndrome or carcinoma
- Evaluation of functional tumors include serum cortisol, plasma free metanephrines, serum DHEA, dexamethasone suppression and urinary cortisol
- FNAC is used to rule out malignancy after ruling out pheochromocytoma
- Radiological features suggestive of adrenal malignancy include diameter greater than 4cm and greater than 10 HU density
Hypercalcemic Crisis
- Seen in primary hyperparathyroidism and hypercalcemia of malignancy
- Hypercalcemia of malignancy is a paraneoplastic syndrome seen in cancers such as squamous cell cancer, breast cancer, and prostate cancer
- Symptoms include confusion, nausea and vomiting, anuria, dehydration, and abdominal pain
- Treatment include aggressive IV fluid therapy, diuretics, bisphosphonates, and calcitonin
- Dialysis should be considered if other measures fail
Hyperparathyroidism
- Indication for surgery in asymptomatic hyperparathyroidism include serum calcium greater than 1mg/dL above reference range, complications such as nephrolithiasis, severe hypercalcuria, decreased bone mass, and age less than 50 years
Parathyroid Surgery
- Localization of adenoma is achieved using sestamibi scan and USG
- If imaging is discordant, 4D CT can be used
- If imaging is concordant, pre-op marking with USG can be used
- Minimally invasive parathyroidectomy (MIP) is a surgical procedure to remove the adenomatous gland
- Miami protocol involves checking pre-op PTH level and comparing it with post-op check 10-15 mins after gland removal
- For hyperplasia, minimally invasive surgery (MIS) is employed
- MIS involves removal of 3.5 glands and retaining half the gland in the neck
- Thymectomy is indicated for lower PTH gland involvement, secondary hyperparathyroidism, and familial syndromes
- Open removal involves removal of all four glands and auto-transplantation of half the gland in the brachioradialis
- Management of adenoma includes minimally invasive parathyroidectomy (MIP)
Secondary Hyperparathyroidism
- Associated with PTH hyperplasia
- Most common cause is chronic renal failure
- Investigations include N/↓ S.Ca (Total & ionized), ↑ S.phosphorus, ↓ Calcitriol, ↑ PTH
- Management includes correction of chronic renal failure, vitamin D3 supplementation, low phosphate diet, cinacalcet, and renal transplantation
- Surgery is not required for localization of gland
Tertiary Hyperparathyroidism
- Cause is persistent autonomous hypercalcemic hyperPTH after kidney transplant
- Investigations include ↓ S.phosphate, ↑ PTH, USG neck, ↑ S.Ca²⁺
- Treatment includes subtotal parathyroidectomy and embolization of gland
Calciphylaxis
- Seen in chronic dialysis
- Hypercalcemic uremic arteriopathy leads to calcium deposition in skin, blood vessels, and soft tissues
- Leads to gangrene, painful purpura, and calcification in breast
Pseudohyperparathyroidism
- Also known as hypercalcemia of pregnancy
- Paraneoplastic syndrome
- Mediated by PTH related peptide (PTHrp)
- Seen in squamous cell carcinoma of lung, breast cancer, and prostate cancer
- Clinical features include confusion, abdominal pain, vomiting, dehydration, and features of hypercalcemia
Parathyroid Cancer
- Rare cancer
- Risk factors include radiation exposure and HGPRT2 gene
- Clinical features include features of hypercalcemia and hypercalcemic crisis (arrhythmias)
- Investigations include increased serum calcium, normal serum phosphorus, N/↓ Calcitriol and PTH, and increased PTHrp
- Management is same as Hypercalcemic crisis
- Investigations include HPE (fibrous bands and vascular invasion) and IHC (inactivation of parafibromin)
- Management includes surgery, octreotide, and cinacalcet
Hypoparathyroidism
- Most common cause is iatrogenic
- DiGeorge syndrome is also a cause, associated with deletion of 22q11
- Symptoms include cardiac anomalies, atypical facies, thymic hypoplasia, cleft lip and palate hypocalcemia
Management of Unilateral Adrenal Mass
- Adrenalectomy is indicated for small (less than 4cm) or malignant features
- Laparoscopic adrenalectomy is indicated if the mass has functional tumor, local invasion, or tumor diameter greater than 5cm
- For non-functional tumors, surgery is indicated if the tumor is greater than 4cm or has malignant features
Pheochromoytoma
- Tumor of adrenal medulla
- Incidence is equal in males and females
- Paraganglioma is an extra-adrenal tumor, common sites include sympathetic and parasympathetic chain
- Follows the rule of 10%: 10% familial, 10% bilateral, 10% malignant, 10% extra adrenal, 10% children
- Syndrome associated with familial Pheochromocytomas include MEN 2 Syndrome, Neurofibromatosis type 1, Von Hippel-Lindau (VHL), Familial paraganglioma 1, Familial paraganglioma 3, and Familial paraganglioma 4
Stones
- Most common cause of recurrent and multiple renal stones is calcium oxalate stones
- Calcium oxalate stones are radiopaque
- Symptoms include abdominal colic and pancreatitis
Primary Hyperparathyroidism
- Causes include adenoma (enlargement of one gland) and hyperplasia (enlargement of all four glands)
- Risk factors include radiation exposure, MEN syndrome, familial syndromes and multiglandular disease
- Biochemical tests reveal increased PTH, increased serum calcium, increased urine calcium, and decreased phosphate
- USG neck and Tc99m sestamibi scan are used for investigations.
- Tc99m sestamibi scan localizes in PTH adenoma due to the presence of mitochondria, false positive can occur with Warthin tumor.
- TYPES of Tc99m sestamibi scan include Single isotope dual phase, Dual isotope subtraction imaging, and SPECT (most common)
Renal stones
- Calcium oxalate stone: Most common type of renal stone
- Radio-opaque.
- Can cause abdominal colic.
Abdominal groans
- Indicate abdominal colic or pancreatitis.
Endocrine System
- Pheochromocytoma is a tumor of adrenal medulla
- Zellballen pattern and salt and pepper nuclei are seen microscopically
- IHC reveal positive staining for synaptophysin, neuron specific enolase, and chromogranin
- Malignant pheochromocytoma is confirmed by metastases and passing score on Ki-67 (proliferation marker), vascular invasion, and capsular invasion.
- Compounds released from pheochromocytoma include noradrenaline, adrenaline, and dopamine
- Clinical features include headache, sweating, palpitations, weight loss, and nausea
- 24 hour urinary VMA is a screening test, plasma free metanephrines are most sensitive
- MRI findings include light bulb sign and swiss cheese appearance
- Extra adrenal pheochromocytoma or metastasis is investigated using a gallium dotatate scan
Parathyroid
- Superior parathyroid originates from the 4th pharyngeal pouch and is located at the posterior aspect of thyroid lobe
- Inferior parathyroid originates from the 3rd pharyngeal pouch and has variable location
- Thymus is a key structure during parathyroid surgery as it can be removed along with the inferior parathyroid gland
- Sentinel pad of fat is a key identification factor for the parathyroid gland
- Parathyroid gland secretes parathyroid hormones (PTH) which regulates plasma Ca2+ concentration
Clinical Features of Hyperparathyroidism
- Bones, stones, abdominal groans, and psychiatric overtones
- Bones: Pathological fracture, subperiosteal bone resorption, salt & pepper skull, brown tumors
- Stones: kidney stones, renal colic, abdominal groans
- Groans: GI motility, pancreatitis
- Psy: Depression, memory impairment, personality changes
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Description
Test your knowledge on adrenal incidentalomas and hypercalcemic crises, including their causes, evaluation methods, and treatment options. Understand the implications of non-functional and functional tumors, as well as the complications of hypercalcemia in cancer. This quiz covers essential concepts related to these critical endocrine disorders.
