Pancreatic endocrine tumors US Medical PG Practice Questions and MCQs
Practice US Medical PG questions for Pancreatic endocrine tumors. These multiple choice questions (MCQs) cover important concepts and help you prepare for your exams.
Pancreatic endocrine tumors US Medical PG Question 1: A previously healthy 52-year-old man comes to the physician because of a 4-month history of recurrent abdominal pain, foul-smelling, greasy stools, and a 5-kg (11-lb) weight loss despite no change in appetite. Physical examination shows pain on palpation of the right upper quadrant. His fasting serum glucose concentration is 186 mg/dL. Abdominal ultrasound shows multiple round, echogenic foci within the gallbladder lumen with prominent posterior acoustic shadowing. The serum concentration of which of the following substances is most likely to be increased in this patient?
- A. Glucagon
- B. Serotonin
- C. Insulin
- D. Somatostatin (Correct Answer)
- E. Vasoactive intestinal peptide
Pancreatic endocrine tumors Explanation: ***Somatostatin***
- Elevated **somatostatin** levels can inhibit the release of various gastrointestinal hormones and enzymes, leading to malabsorption, **steatorrhea (foul-smelling, greasy stools)**, and **weight loss**, as seen in this patient.
- The abdominal pain, gallstones (echogenic foci with posterior acoustic shadowing), and elevated fasting glucose suggest a possible **somatostatinoma**, a rare neuroendocrine tumor producing somatostatin.
*Glucagon*
- **Glucagon** primarily increases blood glucose, which is elevated here, but its excess typically presents with a characteristic **necrolytic migratory erythema** and severe weight loss, not malabsorption as the primary GI symptom.
- While high blood glucose is present, malabsorption symptoms and gallstones are not typical features of a glucagonoma.
*Insulin*
- **Insulin** lowers blood glucose, so an increased concentration would lead to **hypoglycemia**, not the hyperglycemia (186 mg/dL) observed in this patient.
- Symptoms of insulin excess (e.g., insulinoma) would include neuroglycopenic symptoms and weight gain, not malabsorption and weight loss.
*Serotonin*
- Increased **serotonin** levels are associated with **carcinoid syndrome**, which typically presents with flushing, diarrhea, bronchospasm, and valvular heart disease.
- Although diarrhea can be a symptom, the specific features of malabsorption, recurrent abdominal pain, gallstones, and hyperglycemia do not align well with carcinoid syndrome.
*Vasoactive intestinal peptide*
- Elevated **vasoactive intestinal peptide (VIP)**, as in **VIPoma**, causes severe watery diarrhea, hypokalemia, and achlorhydria (WDHA syndrome).
- While weight loss can occur due to fluid loss, the presence of steatorrhea, gallstones, and hyperglycemia makes VIPoma a less likely diagnosis.
Pancreatic endocrine tumors US Medical PG Question 2: A 53-year-old patient presents to his primary care provider with a 1-week history of abdominal pain at night and between meals. He has attempted taking antacids, which help briefly, but then the pain returns. The patient has not noticed any changes to the color of his stool but states that he has been having some loose bowel movements. The patient reports that he has had duodenal ulcers in the past and is concerned that this is a recurrence. On exam, his temperature is 98.4°F (36.9°C), blood pressure is 130/84 mmHg, pulse is 64/min, and respirations are 12/min. The abdomen is soft, nontender, and nondistended in clinic today. A fecal occult blood test is positive for blood in the stool. During outpatient workup, H. pylori stool antigen is negative, endoscopy demonstrates duodenal ulcers, and gastrin levels are elevated after a secretin stimulation test. Which of the following should also be examined in this patient?
- A. Parathyroid hormone (Correct Answer)
- B. Plasma metanephrines
- C. Vasoactive intestinal peptide
- D. Calcitonin
- E. Thyroid stimulating hormone
Pancreatic endocrine tumors Explanation: ***Parathyroid hormone***
- Elevated gastrin levels after a secretin stimulation test and recurrent duodenal ulcers are characteristic of **Zollinger-Ellison syndrome (ZES)**, which is often associated with **Multiple Endocrine Neoplasia type 1 (MEN1)**.
- MEN1 involves tumors of the **parathyroid glands**, **pituitary gland**, and **pancreatic islet cells**. Therefore, parathyroid hormone levels should be checked to screen for **primary hyperparathyroidism**, a common component of MEN1.
*Plasma metanephrines*
- **Plasma metanephrines** are used to screen for **pheochromocytoma**, a tumor of the adrenal medulla which is associated with **MEN2**.
- This patient's presentation is consistent with ZES, which is linked to MEN1, not MEN2.
*Vasoactive intestinal peptide*
- **Vasoactive intestinal peptide (VIP)** levels are elevated in **VIPomas**, which cause **watery diarrhea, hypokalemia, and achlorhydria (WDHA syndrome)**.
- While VIPomas are pancreatic tumors, the patient's symptoms (abdominal pain, duodenal ulcers, elevated gastrin) point towards ZES, not a VIPoma.
*Calcitonin*
- **Calcitonin** is a marker for **medullary thyroid carcinoma**, which is a component of **MEN2**.
- Given the classic presentation of ZES, screening for MEN1 components is appropriate, not MEN2.
*Thyroid stimulating hormone*
- **Thyroid stimulating hormone (TSH)** is used to assess thyroid function. While thyroid disorders can present with various symptoms, they are not directly linked to ZES or MEN1 in the same way parathyroid disease is.
- There is no specific indication from the patient's symptoms (abdominal pain, ulcers, elevated gastrin) that warrants TSH evaluation as the next step in this context.
Pancreatic endocrine tumors US Medical PG Question 3: A 42-year-old man presents to his primary care provider for abdominal pain. He reports that for several months he has been experiencing a stabbing pain above the umbilicus during meals. He denies associated symptoms of nausea, vomiting, or diarrhea. The patient’s past medical history is significant for hypertension and hyperlipidemia for which he takes amlodipine and atorvastatin. His family history is significant for lung cancer in his father. The patient is a current smoker with a 20 pack-year smoking history and drinks 3-5 beers per week. Initial laboratory testing is as follows:
Serum:
Na+: 141 mEq/L
K+: 4.6 mEq/L
Cl-: 102 mEq/L
HCO3-: 25 mEq/L
Urea nitrogen: 14 mg/dL
Creatinine: 1.1 mg/dL
Glucose: 120 mg/dL
Calcium: 8.4 mg/dL
Alkaline phosphatase: 66 U/L
Aspartate aminotransferase (AST): 40 U/L
Alanine aminotransferase (ALT): 52 U/L
Gastrin: 96 pg/mL (<100 pg/mL)
Lipase: 90 U/L (<160 U/L)
The patient is started on a proton pump inhibitor without symptomatic improvement after 6 weeks. He is referred for an upper endoscopy, which demonstrates erosive gastritis, three ulcers in the duodenum, and one ulcer in the jejunum. Biopsy of the gastric mucosa is negative for H. pylori. Which of the following is the best next step in management?
- A. Calcium infusion study
- B. Serum prolactin level
- C. Serum chromogranin A level
- D. Empiric triple therapy
- E. Secretin stimulation test (Correct Answer)
Pancreatic endocrine tumors Explanation: ***Secretin stimulation test***
- The presence of multiple ulcers in unusual locations (duodenum and jejunum), **erosive gastritis**, despite PPI therapy and negative *H. pylori* test, is highly suggestive of **Zollinger-Ellison syndrome (ZES)**.
- A secretin stimulation test is the most accurate diagnostic test for ZES. It assesses the paradoxical increase in gastrin levels after secretin administration, which is characteristic of **gastrinomas**.
*Calcium infusion study*
- While sometimes used in ZES diagnosis, especially if secretin stimulation test is inconclusive, a **calcium infusion study** is less specific and sensitive than the secretin stimulation test.
- It involves infusing calcium and monitoring gastrin levels; however, it has a higher incidence of side effects.
*Serum prolactin level*
- **Serum prolactin level** is used to diagnose **prolactinomas**, a type of pituitary adenoma, which causes symptoms like galactorrhea, amenorrhea, or erectile dysfunction.
- These symptoms are unrelated to the patient’s presentation of severe dyspepsia and refractory ulcers.
*Serum chromogranin A level*
- **Chromogranin A** is a tumor marker for neuroendocrine tumors, including gastrinomas. However, its specificity is low, as levels can be elevated in conditions like chronic atrophic gastritis, chronic renal failure, and during PPI use.
- It is often used for monitoring treatment response or recurrence rather than initial diagnosis of ZES.
*Empiric triple therapy*
- **Empiric triple therapy** is the standard treatment for *H. pylori* infection, consisting of a PPI, amoxicillin, and clarithromycin (or metronidazole).
- The patient's gastric biopsy was already **negative for *H. pylori***, and his ulcers are refractory to standard PPI therapy, indicating that *H. pylori* is not the cause and further empiric treatment would be ineffective.
Pancreatic endocrine tumors US Medical PG Question 4: A 28-year-old man is brought in by ambulance to the ER, barely conscious, after feeling drowsy and falling to the floor during a presentation several hours ago. His colleague who accompanied him says he has had similar episodes 5 times in the past 3 months. No significant past medical history. His blood pressure is 110/80 mm Hg and pulse is 114/min. His capillary blood glucose is 15 mg/dL. Immediate IV dextrose with thiamine is started, and he rapidly regains consciousness. A contrast CT of the abdomen is performed which reveals a tumor in the pancreas. Which of the following relative laboratory findings would you most likely expect to find in this patient?
- A. Glucose: ↑, Insulin: ↓, C-Peptide: ↓, Ketoacidosis: Present
- B. Glucose: Normal, Insulin: Normal, C-Peptide: Normal, Ketoacidosis: Absent
- C. Glucose: ↓, Insulin: ↑, C-Peptide: ↑, Ketoacidosis: Absent (Correct Answer)
- D. Glucose: ↓, Insulin: ↑, C-Peptide: ↓, Ketoacidosis: Absent
- E. Glucose: ↑, Insulin: ↑/Normal, C-Peptide: ↑/Normal, Ketoacidosis: Absent
Pancreatic endocrine tumors Explanation: ***Glucose: ↓, Insulin: ↑, C-Peptide: ↑, Ketoacidosis: Absent***
- The patient's **hypoglycemia (15 mg/dL)**, coupled with a pancreatic tumor and recurrent episodes, strongly suggests an **insulinoma**.
- An **insulinoma** is an insulin-secreting tumor, leading to **high insulin** and **C-peptide** levels in the presence of low glucose, and typically no ketoacidosis because insulin inhibits ketogenesis.
*Glucose: ↑, Insulin: ↓, C-Peptide: ↓, Ketoacidosis: Present*
- This profile describes **Type 1 Diabetes Mellitus** or severe insulin deficiency, where high glucose is due to lack of insulin production and subsequent diabetic ketoacidosis.
- The patient's symptoms (hypoglycemia) and the presence of a pancreatic tumor producing insulin are contradictory to this profile.
*Glucose: Normal, Insulin: Normal, C-Peptide: Normal, Ketoacidosis: Absent*
- This profile represents a **healthy individual** with normal metabolic function, which is inconsistent with the patient's severe hypoglycemia and recurrent collapses.
- It would not explain the patient's symptoms or the pancreatic tumor's function.
*Glucose: ↓, Insulin: ↑, C-Peptide: ↓, Ketoacidosis: Absent*
- This finding would be typical of **exogenous insulin administration** (e.g., insulin overdose) where insulin levels are high, but C-peptide (which is co-secreted with endogenous insulin) is low.
- While hypoglycemia is present, the low C-peptide contradicts the presence of an endogenous insulin-secreting pancreatic tumor.
*Glucose: ↑, Insulin: ↑/Normal, C-Peptide: ↑/Normal, Ketoacidosis: Absent*
- These findings could be seen in conditions like **Type 2 Diabetes** with **insulin resistance** or Cushing's syndrome where glucose and insulin might be elevated, but the patient's primary presentation is severe hypoglycemia.
- This profile does not align with the patient's profound hypoglycemia and clinical picture of an insulinoma.
Pancreatic endocrine tumors US Medical PG Question 5: A 53-year-old man comes to the physician because of fatigue, recurrent diarrhea, and an 8-kg (17.6-lb) weight loss over the past 6 months. He has a 4-month history of recurrent blistering rashes on different parts of his body that grow and develop into pruritic, crusty lesions before resolving spontaneously. Physical examination shows scaly lesions in different phases of healing with central, bronze-colored induration around the mouth, perineum, and lower extremities. Laboratory studies show:
Hemoglobin 10.1 mg/dL
Mean corpuscular volume 85 μm3
Mean corpuscular hemoglobin 30.0 pg/cell
Serum
Glucose 236 mg/dL
Abdominal ultrasonography shows a 3-cm, solid mass located in the upper abdomen. This patient's mass is most likely derived from which of the following types of cells?
- A. Gastrointestinal enterochromaffin cells
- B. Pancreatic β-cells
- C. Pancreatic δ-cells
- D. Pancreatic α-cells (Correct Answer)
- E. Gastric G-cells
Pancreatic endocrine tumors Explanation: ***Pancreatic α-cells***
- The patient's symptoms of **fatigue, recurrent diarrhea, weight loss, blistering rash (necrolytic migratory erythema)**, and **hyperglycemia** are classic features of a **glucagonoma**.
- A **glucagonoma** is a tumor of the pancreatic α-cells that **secretes excessive glucagon**, leading to these characteristic signs and symptoms, supported by the presence of an **upper abdominal mass**.
*Gastrointestinal enterochromaffin cells*
- Tumors of gastrointestinal enterochromaffin cells (carcinoid tumors) typically produce **serotonin** and present with flushing, diarrhea, bronchospasm, and valvular heart disease, not the skin rash or hyperglycemia seen here.
- While carcinoid tumors can cause diarrhea, the additional symptoms of **necrolytic migratory erythema** and **diabetes** are not characteristic.
*Pancreatic β-cells*
- Tumors of pancreatic β-cells (**insulinomas**) produce excessive insulin, leading to **hypoglycemia**, not the hyperglycemia observed in this patient.
- Insulinomas cause symptoms like sweating, tremors, confusion, and palpitations, which are inconsistent with the patient's presentation.
*Pancreatic δ-cells*
- Pancreatic δ-cell tumors (**somatostatinomas**) secrete **somatostatin**, which can cause **diabetes mellitus**, steatorrhea, and gallstones.
- While diabetes is present, the characteristic **necrolytic migratory erythema** and severe diarrhea are less common with somatostatinomas.
*Gastric G-cells*
- Tumors of gastric G-cells (**gastrinomas**) secrete **gastrin**, leading to **Zollinger-Ellison syndrome**, characterized by severe peptic ulcers, abdominal pain, and chronic diarrhea.
- Gastrinomas do not typically cause **necrolytic migratory erythema** or significant hyperglycemia.
Pancreatic endocrine tumors US Medical PG Question 6: A 47-year-old male presents to his primary care physician complaining of upper abdominal pain. He reports a four-month history of gnawing epigastric discomfort that improves with meals. He has lost 10 pounds over that same period. His past medical history is significant for a prolactinoma for which he underwent transphenoidal resection. He does not smoke or drink alcohol. His family history is notable for a paternal uncle and paternal grandmother with parathyroid neoplasms. His temperature is 99°F (37.2°C), blood pressure is 115/80 mmHg, pulse is 80/min, and respirations are 18/min. Upon further diagnostic workup, which of the following sets of laboratory findings is most likely?
- A. Elevated fasting serum gastrin that increases with secretin administration (Correct Answer)
- B. Elevated fasting serum gastrin that decreases with secretin administration
- C. Elevated fasting serum gastrin that decreases with cholecystokinin administration
- D. Elevated fasting serum gastrin that increases with somatostatin administration
- E. Normal fasting serum gastrin
Pancreatic endocrine tumors Explanation: ***Elevated fasting serum gastrin that increases with secretin administration***
- The patient's presentation with **gnawing epigastric discomfort improving with meals**, weight loss, and a history of **prolactinoma** (suggesting MEN1) points strongly to **Zollinger-Ellison syndrome (ZES)**, caused by a gastrinoma.
- In ZES, gastrinomas uniquely have **ectopic secretin receptors**, leading to a paradoxical increase in gastrin levels after secretin administration, which is a diagnostic hallmark.
*Elevated fasting serum gastrin that decreases with secretin administration*
- This response is typical for **non-gastrinoma hypergastrinemia**, such as that seen in **atrophic gastritis** with low gastric acid production.
- Secretin normally inhibits gastrin release by parietal cells, but gastrinomas are autonomous and respond paradoxically.
*Elevated fasting serum gastrin that decreases with cholecystokinin administration*
- **Cholecystokinin (CCK)** primarily stimulates pancreatic enzyme and gallbladder contraction, and its administration is not a standard diagnostic test for gastrinoma or ZES gastrin response.
- Gastrinomas are typically **unresponsive to CCK** in a predictable manner that would aid in diagnosis.
*Elevated fasting serum gastrin that increases with somatostatin administration*
- **Somatostatin** is a potent inhibitor of gastrin release from both normal G cells and gastrinomas.
- An **increase in gastrin with somatostatin** administration would be highly unusual and not characteristic of any known gastrinopathy.
*Normal fasting serum gastrin*
- Given the patient's symptoms (severe, chronic ulcer-like pain, weight loss) and his family history and personal history of prolactinoma suggestive of **MEN1**, a **normal fasting gastrin level** would make Zollinger-Ellison syndrome much less likely.
- **Hypergastrinemia** is a prerequisite for diagnosing ZES and would be expected in this clinical context.
Pancreatic endocrine tumors US Medical PG Question 7: A previously healthy 41-year-old woman comes to the physician for the evaluation of recurrent episodes of palpitations and sweating over the past month. Her symptoms typically start after swimming practice and improve after drinking ice tea and eating some candy. She has also had a 5-kg (11-lb) weight gain over the past 3 months. She works as a nurse. Physical examination shows no abnormalities. Fasting serum studies show:
Glucose 38 mg/dL
Insulin 260 μU/mL (N=11–240)
Proinsulin 65 μU/mL (N <20% of total insulin)
C-peptide 5.0 ng/mL (N=0.8–3.1)
Insulin secretagogues absent
Which of the following is the most likely cause of her symptoms?
- A. Pancreatic β-cell tumor (Correct Answer)
- B. Exogenous administration of insulin
- C. Binge eating disorder
- D. Factitious use of sulfonylureas
- E. Peripheral resistance to insulin
Pancreatic endocrine tumors Explanation: ***Pancreatic β-cell tumor***
- The patient presents with **Whipple's triad**: symptoms of hypoglycemia (palpitations, sweating), low plasma glucose (<50 mg/dL), and symptom relief with glucose intake (ice tea, candy).
- Elevated **C-peptide** and **proinsulin** levels, along with inappropriately high insulin relative to the low glucose, indicate endogenous hyperinsulinism, strongly suggesting an **insulinoma (pancreatic β-cell tumor)**.
*Exogenous administration of insulin*
- Injected insulin would lead to high insulin levels and low glucose, but **C-peptide** levels would be **low** because exogenous insulin suppresses endogenous insulin secretion.
- The patient's **high C-peptide** rules out exogenous insulin administration.
*Binge eating disorder*
- Binge eating disorder does not directly explain the recurrent episodes of documented **hypoglycemia** and the specific hormonal profile of high insulin and C-peptide.
- While weight gain might be associated, it does not account for the **biochemical abnormalities**.
*Factitious use of sulfonylureas*
- Sulfonylureas stimulate endogenous insulin secretion, leading to hypoglycemia, high insulin, and **high C-peptide**.
- However, the lab results explicitly state **insulin secretagogues absent**, ruling out sulfonylurea use.
*Peripheral resistance to insulin*
- Insulin resistance is characterized by **high insulin levels** due to the pancreas overproducing insulin to compensate for tissue insensitivity, but it typically causes **hyperglycemia**, not hypoglycemia.
- The patient's **hypoglycemia** is inconsistent with peripheral insulin resistance.
Pancreatic endocrine tumors US Medical PG Question 8: A 45-year-old woman comes to see you for a second opinion regarding an upcoming surgery for pancreatic insulinoma. While taking a surgical history, she tells you she previously had a pituitary tumor resected. For which additional neoplasms might you consider testing her?
- A. Parathyroid adenoma (Correct Answer)
- B. Medullary thyroid carcinoma
- C. Mucosal neuroma
- D. Multiple myeloma
- E. Pheochromocytoma
Pancreatic endocrine tumors Explanation: ***Parathyroid adenoma***
- The patient's history of a **pituitary tumor** and a **pancreatic insulinoma** strongly suggests **Multiple Endocrine Neoplasia Type 1 (MEN1)**.
- **Parathyroid adenomas** are the most common manifestation of MEN1, affecting over 90% of patients, and should be screened for.
*Medullary thyroid carcinoma*
- This is a characteristic component of **Multiple Endocrine Neoplasia Type 2 (MEN2)**.
- MEN2 includes medullary thyroid carcinoma, pheochromocytoma, and parathyroid hyperplasia, which is distinct from the patient's presentation.
*Mucosal neuroma*
- **Mucosal neuromas** are associated with **Multiple Endocrine Neoplasia Type 2B (MEN2B)**, a subtype of MEN2.
- MEN2B also typically involves medullary thyroid carcinoma and pheochromocytoma, which do not align with the patient's current tumors.
*Multiple myeloma*
- This is a **hematologic malignancy** involving plasma cells and is not typically associated with the type of endocrine tumors seen in this patient.
- There is no direct link between multiple myeloma and pituitary or pancreatic neuroendocrine tumors.
*Pheochromocytoma*
- **Pheochromocytoma** is a tumor of the adrenal medulla and is a hallmark of **Multiple Endocrine Neoplasia Type 2 (MEN2)**.
- While it is an endocrine tumor, it is not part of the triad seen in MEN1 (pituitary, parathyroid, and pancreatic tumors).
Pancreatic endocrine tumors US Medical PG Question 9: A 36-year-old man is admitted to the hospital because of a 1-day history of epigastric pain and vomiting. He has had similar episodes of epigastric pain in the past. He drinks 8 oz of vodka daily. Five days after admission, the patient develops aspiration pneumonia and sepsis. Despite appropriate therapy, the patient dies. At autopsy, the pancreas appears gray, enlarged, and nodular. Microscopic examination of the pancreas shows localized deposits of calcium. This finding is most similar to an adaptive change that can occur in which of the following conditions?
- A. Primary hyperparathyroidism
- B. Sarcoidosis
- C. Multiple myeloma
- D. Congenital CMV infection
- E. Chronic kidney disease (Correct Answer)
Pancreatic endocrine tumors Explanation: ***Chronic kidney disease***
- The pancreatic finding represents **dystrophic calcification** (calcium deposition in damaged tissue with normal serum calcium levels), a consequence of chronic pancreatitis.
- **Chronic kidney disease** is the most similar condition because it also involves pathologic calcification as an adaptive/pathologic change, though through a different mechanism called **metastatic calcification**.
- In CKD, **hyperphosphatemia** and secondary **hyperparathyroidism** elevate the serum calcium-phosphate product, leading to calcium deposition in **normal tissues** (blood vessels, kidneys, lungs, gastric mucosa).
- Both conditions demonstrate **pathologic calcification as a tissue response** to metabolic derangement, making CKD the best answer among the options provided.
*Primary hyperparathyroidism*
- Causes **hypercalcemia** and **hypophosphatemia** due to excess PTH secretion.
- Can lead to **metastatic calcification** in normal tissues, but is less commonly associated with widespread tissue calcification compared to CKD.
- The mineral imbalance pattern differs from CKD (high calcium, low phosphate vs. high phosphate, variable calcium).
*Sarcoidosis*
- Causes **hypercalcemia** due to increased 1,25-dihydroxyvitamin D production by activated macrophages in granulomas.
- Can result in **metastatic calcification**, particularly nephrocalcinosis.
- However, this is less common and less extensive than the calcification seen in CKD.
*Multiple myeloma*
- Produces **hypercalcemia** through osteolytic bone destruction and cytokine-mediated bone resorption.
- Can theoretically cause **metastatic calcification**, but this is not a typical or prominent feature of the disease.
- The hypercalcemia is usually addressed before significant tissue calcification occurs.
*Congenital CMV infection*
- Causes **dystrophic calcification** in damaged tissues, typically **periventricular intracranial calcifications**.
- While this involves the same type of calcification (dystrophic), CMV infection is not characterized by systemic or progressive calcification as an adaptive metabolic response.
- The calcifications are focal sequelae of viral tissue damage, not a widespread metabolic derangement.
Pancreatic endocrine tumors US Medical PG Question 10: A 38-year-old woman with type 1 diabetes for 20 years presents with diabetic ketoacidosis. She is treated and recovers. Six months later, she develops progressive fatigue, nausea, and hyperpigmentation. Laboratory studies show morning cortisol 3 μg/dL, ACTH 180 pg/mL, TSH 8.2 mIU/L, free T4 0.6 ng/dL, and positive anti-thyroid peroxidase antibodies. She also has positive 21-hydroxylase antibodies. Her 12-year-old daughter was recently diagnosed with type 1 diabetes. Evaluate the pathologic process and most critical monitoring recommendation for the daughter.
- A. Multiple endocrine neoplasia syndrome; screen for pheochromocytoma and medullary thyroid cancer
- B. Autoimmune polyglandular syndrome type 2; screen daughter for adrenal antibodies and thyroid function annually (Correct Answer)
- C. Schmidt syndrome with isolated sporatic occurrence; routine diabetes management only for daughter
- D. Autoimmune polyglandular syndrome type 1; screen for mucocutaneous candidiasis and hypoparathyroidism
- E. Secondary endocrine failure from diabetes complications; optimize glycemic control in daughter
Pancreatic endocrine tumors Explanation: ***Autoimmune polyglandular syndrome type 2; screen daughter for adrenal antibodies and thyroid function annually***
- The patient presents with **Schmidt syndrome (APS type 2)**, defined by the triad of **Addison's disease** (low cortisol, high ACTH, 21-hydroxylase antibodies), **type 1 diabetes**, and **autoimmune thyroid disease** (Hashimoto's).
- Because APS-2 is **polygenic** and associated with **HLA-DR3/DR4**, first-degree relatives with one component (like the daughter) require screening for other silent autoimmune conditions to prevent **adrenal crisis**.
*Multiple endocrine neoplasia syndrome; screen for pheochromocytoma and medullary thyroid cancer*
- **MEN syndromes** are characterized by **neoplastic** growths (like medullary thyroid cancer or parathyroid adenomas) rather than the **autoimmune destruction** of glands seen here.
- The absence of hypertension or neck masses and the presence of **autoantibodies** directly contradict a diagnosis of MEN.
*Schmidt syndrome with isolated sporatic occurrence; routine diabetes management only for daughter*
- While the patient does have **Schmidt syndrome**, it is incorrect to label it as purely sporadic because it has a strong **familial clustering** component.
- Managing only diabetes in the daughter is insufficient and dangerous, as it ignores her increased risk for life-threatening **primary adrenal insufficiency**.
*Autoimmune polyglandular syndrome type 1; screen for mucocutaneous candidiasis and hypoparathyroidism*
- **APS-1** typically presents in childhood with the triad of **chronic mucocutaneous candidiasis**, **hypoparathyroidism**, and adrenal failure, which does not match this adult patient’s profile.
- APS-1 is caused by a single gene mutation in **AIRE**, whereas this patient's presentation and family history are classic for the polygenic **APS-2**.
*Secondary endocrine failure from diabetes complications; optimize glycemic control in daughter*
- Diabetic complications lead to **microvascular** or **macrovascular** damage, not the **primary gland failure** (indicated by high ACTH and TSH) seen in this patient.
- **Hyperpigmentation** and the presence of **organ-specific antibodies** (21-hydroxylase) confirm an **autoimmune primary failure**, not a secondary metabolic complication.
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