Surgery Marrow Pg 111-120 (Endocrine)
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Surgery Marrow Pg 111-120 (Endocrine)

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Questions and Answers

What is the most common type of adrenal incidentaloma?

  • Carcinoma
  • Metastases (correct)
  • Cushing's Syndrome
  • Pheochromocytoma

    • All adrenal incidentalomas are functional tumors.

    False

    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.

    <p>4</p> Signup and view all the answers

    Match the following adrenal evaluation methods with their purpose:

    <p>Serum cortisol = Evaluate for Cushing's Syndrome Plasma free metanephrines = Evaluate for pheochromocytoma Dexamethasone suppression test = Differentiate between Cushing's and non-Cushing's Urinary cortisol = Assess cortisol production</p> Signup and view all the answers

    Which of the following is a symptom of hypercalcemic crisis?

    <p>Nausea and vomiting</p> Signup and view all the answers

    An age of 45 years is an indication for surgery in asymptomatic hyperparathyroidism.

    <p>True</p> Signup and view all the answers

    What is the first step in the management of hypercalcemic crisis?

    <p>Aggressive IV fluid therapy</p> Signup and view all the answers

    Post-PTH gland removal, bone influx of $Ca^{2+}$ leads to __________.

    <p>hypocalcemia</p> Signup and view all the answers

    Match the following conditions with their features:

    <p>Primary hyperparathyroidism = Causes hypercalcemia Hypocalcemia = Can occur after PTH removal Hypercalcemia of malignancy = Often associated with certain cancers Hungry Bone Syndrome = Post-operative calcium influx into bones</p> Signup and view all the answers

    What imaging technique is used in the case of discordant imaging for localization of adenoma?

    <p>4D CT</p> Signup and view all the answers

    The Miami protocol involves checking the PTH level before surgery.

    <p>True</p> Signup and view all the answers

    What is the primary goal of minimally invasive parathyroidectomy (MIP)?

    <p>Removal of the adenomatous gland.</p> Signup and view all the answers

    In open removal of glands, a half gland is auto-transplanted in the __________ of the non-dominant hand.

    <p>brachioradialis</p> Signup and view all the answers

    Match the surgical procedures with their corresponding descriptions:

    <p>Minimally Invasive Surgery (MIS) = Removes 3 1/2 glands and retains 1/2 gland in neck Thymectomy = Indicated for involvement of lower PTH gland Open Removal = All 4 glands are removed Minimally Invasive Parathyroidectomy (MIP) = Checks PTH level post gland removal</p> Signup and view all the answers

    What is the most common cause of secondary hyperparathyroidism?

    <p>Chronic renal failure</p> Signup and view all the answers

    Tertiary hyperparathyroidism occurs after kidney transplantation.

    <p>True</p> Signup and view all the answers

    What is the mainstay treatment for tertiary hyperparathyroidism?

    <p>Subtotal parathyroidectomy</p> Signup and view all the answers

    Secondary hyperparathyroidism is commonly associated with _____ hyperplasia.

    <p>PTH</p> Signup and view all the answers

    Match the following conditions with their associated findings:

    <p>Secondary Hyperparathyroidism = ↑ PTH, N/↓ S. Ca²⁺ Tertiary Hyperparathyroidism = ↑ PTH, ↑ S. Ca²⁺ Calciphylaxis = Gangrene, painful purpura Vitamin D Deficiency = Defective intestinal absorption</p> Signup and view all the answers

    Which condition is most commonly associated with hypercalcemia in the context of pregnancy?

    <p>Pseudohyperparathyroidism</p> Signup and view all the answers

    DiGeorge syndrome is characterized by a deletion of the 22q12 chromosome.

    <p>False</p> Signup and view all the answers

    Name one risk factor associated with parathyroid cancer.

    <p>Radiation exposure or HGPRT2 gene</p> Signup and view all the answers

    In cases of hypercalcemia, confusion, abdominal pain, and vomiting are considered __________.

    <p>clinical features</p> Signup and view all the answers

    Match the following diseases with their corresponding characteristics:

    <p>Pseudohyperparathyroidism = PTH related peptide mediated Hypoparathyroidism = Iatrogenic cause is most common Parathyroid cancer = Inactivation of parafibromin DiGeorge syndrome = CATCH 22</p> Signup and view all the answers

    Which feature indicates a higher suspicion of malignancy in an adrenal mass?

    <p>All of the above</p> Signup and view all the answers

    Pheochromocytoma is more common in males than females.

    <p>False</p> Signup and view all the answers

    What is the most common syndrome associated with familial pheochromocytomas?

    <p>MEN 2 Syndrome</p> Signup and view all the answers

    In cases of pheochromocytoma, 10% of tumors are ___ and extra adrenal.

    <p>malignant</p> Signup and view all the answers

    Match the tumor types with their characteristics:

    <p>Pheochromocytoma = Tumor of Adrenal Medulla Paraganglioma = Extra Adrenal Tumors</p> Signup and view all the answers

    What is the most common type of renal stone?

    <p>Calcium oxalate stone</p> Signup and view all the answers

    Hyperplasia refers to the enlargement of only one parathyroid gland.

    <p>False</p> Signup and view all the answers

    What biochemical changes are observed in primary hyperparathyroidism?

    <p>Increased PTH, Increased Serum Calcium, Increased Urine Calcium, Decreased Phosphate</p> Signup and view all the answers

    A patient with primary hyperparathyroidism may have __________ renal stones due to increased calcium levels.

    <p>multiple</p> Signup and view all the answers

    Match the following investigation methods with their purposes:

    <p>USG neck = Identify parathyroid adenoma Tc99m sestamibi scan = Localize PTH adenoma Biochemical test = Evaluate hormone and electrolyte levels SPECT = Visualize tissue perfusion and localization</p> Signup and view all the answers

    Which gene is associated with multiple endocrine neoplasia type 2A (MEN-2A)?

    <p>RET</p> Signup and view all the answers

    Von Hippel-Lindau (VHL) syndrome is associated with paragangliomas.

    <p>True</p> Signup and view all the answers

    What is the histopathological appearance of pheochromocytoma?

    <p>Reddish-brown color with areas showing tan-brown, necrosis, and hemorrhage.</p> Signup and view all the answers

    Familial paraganglioma 1 is associated with the gene ______.

    <p>SDHD</p> Signup and view all the answers

    Match the following syndromes with their associated lesions:

    <p>Multiple Endocrine Neoplasia Type 2A = Pheochromocytoma Neurofibromatosis, type 1 = Pheochromocytoma Von Hippel-Lindau (VHL) = Pheochromocytoma and Paraganglioma Familial Paraganglioma 3 = Paraganglioma</p> Signup and view all the answers

    What is the most common symptom associated with pheochromocytoma?

    <p>Headache</p> Signup and view all the answers

    Pheochromocytomas predominantly release adrenaline over noradrenaline.

    <p>False</p> Signup and view all the answers

    Name one confirmatory sign of malignant pheochromocytoma.

    <p>Metastases</p> Signup and view all the answers

    The primary screening test for pheochromocytoma is a 24-hour urinary _______.

    <p>VMA</p> Signup and view all the answers

    Match the following substances with the conditions they are primarily associated with:

    <p>Pheochromocytoma = Noradrenaline &gt; Adrenaline Paraganglioma = Noradrenaline Malignant Pheochromocytoma = Dopamine Adrenaline = Not primarily released in pheochromocytoma</p> Signup and view all the answers

    What is the primary function of parathyroid hormone (PTH)?

    <p>Increase plasma Ca2+ concentration</p> Signup and view all the answers

    The thymus is derived from the 4th pharyngeal pouch.

    <p>False</p> Signup and view all the answers

    What is the mnemonic to remember the clinical features of hyperparathyroidism?

    <p>Bones, stones, abdominal groans, psychiatric overtones</p> Signup and view all the answers

    The inferior parathyroid gland originates from the __________ pharyngeal pouch.

    <p>3rd</p> Signup and view all the answers

    Match the following conditions with their associated features:

    <p>Pathological fractures = Bones Osteitis fibrosa cystica = Bones Diminished renal function = Stones Psychiatric manifestations = Psychiatric overtones</p> Signup and view all the answers

    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.

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