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Part 2: Advanced-Level MCQs

Below are the 5 Advanced-Level MCQs, followed by the Key Takeaways for the Entire Topic as requested.

Part 2: Advanced-Level MCQs

MCQ 6

  1. Question

    In patients with a known mucin-producing pancreatic cyst (e.g., IPMN or MCN), which of the following molecular markers is most strongly associated with progression to invasive carcinoma?

  2. Answer Choices

    A. Amylase

    B. VHL gene mutation

    C. KRAS and GNAS mutations

    D. Low cyst fluid CEA (< 5 ng/mL)

  3. Correct Answer

    C. KRAS and GNAS mutations

  4. Explanation

    • Why C is correct: In IPMNs, especially the intestinal subtype, GNAS and KRAS mutations frequently drive neoplastic progression. GNAS mutations are quite specific to IPMNs (as opposed to MCNs), whereas KRAS mutations appear in various pancreatic tumors.
    • Why A, B, and D are incorrect:
      • A. Amylase levels can be elevated in many pancreatic cystic conditions and are not specifically tied to malignant progression.
      • B. VHL gene mutations are primarily associated with serous cystadenomas.
      • D. A low cyst fluid CEA generally argues against mucinous pathology.
  5. Key Takeaways
    • Molecular analysis (e.g., KRAS, GNAS, RNF43) is increasingly used to differentiate cyst types.
    • GNAS mutations are uniquely common in IPMNs.
    • MCNs may share KRAS mutations with IPMNs but typically lack GNAS mutations.
    • Identifying these markers can help predict the risk of malignant transformation and guide management.
Reference:
Blumgart's Surgery of the Liver, Biliary Tract and Pancreas, 7th edition,
Chapter 60: "Cystic neoplasms of the pancreas: Epidemiology, clinical features, assessment, and management",
Subtopic: "Genetics of Intraductal Papillary Mucinous Neoplasms" (pp. 873–874)

MCQ 7

(All are true EXCEPT)

  1. Question

    Regarding solid pseudopapillary tumors (SPTs) of the pancreas, all of the following statements are true EXCEPT:

  2. Answer Choices

    A. They predominantly affect young females in their 20s and 30s.

    B. They are often well-encapsulated and can show mixed solid-cystic architecture.

    C. They typically demonstrate KRAS and TP53 gene alterations in the majority of cases.

    D. Complete surgical extirpation offers an excellent long-term prognosis in most patients.

  3. Correct Answer

    C. They typically demonstrate KRAS and TP53 gene alterations in the majority of cases.

  4. Explanation

    • Why C is correct (the EXCEPT statement): Solid pseudopapillary tumors (SPTs) commonly exhibit aberrant β-catenin signaling rather than KRAS or TP53 alterations, which are more characteristic of ductal adenocarcinoma or other pancreatic neoplasms.
    • Why A, B, and D are true:
      • A. SPTs have a strong female predominance and frequently present in younger women.
      • B. They often appear encapsulated and display both solid and cystic components.
      • D. Surgical resection is curative for most patients, leading to excellent survival outcomes even if minimal metastases are present.
  5. Key Takeaways
    • SPTs are rare pancreatic tumors, often discovered in younger females.
    • Complete surgical resection typically confers a favorable prognosis.
    • Their molecular profile is typically characterized by β-catenin pathway dysregulation.
    • Early detection and resection are paramount, as these tumors can become locally invasive or metastasize if neglected.
Reference:
Blumgart's Surgery of the Liver, Biliary Tract and Pancreas, 7th edition,
Chapter 60: "Cystic neoplasms of the pancreas: Epidemiology, clinical features, assessment, and management",
Subtopic: "Solid Pseudopapillary Tumor (SPT)" (pp. 874–875)

MCQ 8

  1. Question

    Mixed-type intraductal papillary mucinous neoplasms (IPMNs) involve which of the following ductal structures?

  2. Answer Choices

    A. Common bile duct plus main pancreatic duct

    B. Diffuse areas of the branch ducts only

    C. Both the main pancreatic duct and one or more branch ducts

    D. Minor papilla ductal system exclusively

  3. Correct Answer

    C. Both the main pancreatic duct and one or more branch ducts

  4. Explanation

    • Why C is correct: Mixed-type IPMNs, by definition, display combined involvement of the main pancreatic duct (≥5 mm dilatation) and associated branch duct(s).
    • Why A, B, and D are incorrect:
      • A. The common bile duct is an extrahepatic biliary structure, unrelated to IPMN classification.
      • B. Branch-duct IPMNs do not involve the main duct; they are confined to side branches.
      • D. Exclusive involvement of the minor papilla is rare and not termed “mixed-type.”
  5. Key Takeaways
    • IPMNs are subdivided into main-duct, branch-duct, or mixed subtypes based on ductal involvement.
    • The extent of ductal involvement correlates with malignancy risk; main-duct and mixed variants carry higher risk than pure branch-duct.
    • Accurate imaging (CT, MRI/MRCP, EUS) is crucial to identify ductal dilation and potential invasive features.
    • Management often hinges on identifying these subtypes to determine resectability and follow-up intervals.
Reference:
Blumgart's Surgery of the Liver, Biliary Tract and Pancreas, 7th edition,
Chapter 60: "Cystic neoplasms of the pancreas: Epidemiology, clinical features, assessment, and management",
Subtopic: "IPMN Classification and Subtypes" (pp. 872–873)

MCQ 9

  1. Question

    In a patient with suspected main-duct IPMN (diffuse dilation of the main pancreatic duct >10 mm) but no radiologic evidence of an invasive mass, which of the following best describes a key surgical management controversy?

  2. Answer Choices

    A. Whether to perform an extended lymph node dissection for prophylaxis

    B. Whether to attempt a total pancreatectomy versus a segmental resection

    C. Whether to administer neoadjuvant chemotherapy before resection

    D. Whether to resect the lesion endoscopically rather than surgically

  3. Correct Answer

    B. Whether to attempt a total pancreatectomy versus a segmental resection

  4. Explanation

    • Why B is correct: When the main duct is diffusely involved, the dilemma is balancing the oncologic benefit of total pancreatectomy (removing all at-risk tissue) against its significant morbidity (exocrine and endocrine insufficiency). Some prefer a segmental approach plus close surveillance of the remnant.
    • Why A, C, and D are incorrect:
      • A. A standard lymph node dissection is done if invasive disease is identified, but this is not the central dilemma in noninvasive IPMN.
      • C. Neoadjuvant chemotherapy is not routinely used for IPMN without confirmed invasive cancer.
      • D. Endoscopic resection is not typical for IPMNs that involve large portions of the pancreatic duct.
  5. Key Takeaways
    • The extent of resection for main-duct IPMNs remains a challenging decision.
    • Total pancreatectomy eliminates the entire ductal system but carries lifelong insulin dependence and digestive enzyme supplementation.
    • Intraoperative frozen-section analysis helps determine the presence of high-grade dysplasia near resection margins.
    • Individualized decisions consider patient comorbidities, age, and risk of missed or future malignancy in residual gland.
Reference:
Blumgart's Surgery of the Liver, Biliary Tract and Pancreas, 7th edition,
Chapter 60: "Cystic neoplasms of the pancreas: Epidemiology, clinical features, assessment, and management",
Subtopic: "Main-Duct IPMN: Indication and Extent of Resection" (pp. 873–875)

MCQ 10

  1. Question

    A 58-year-old patient with a branch-duct IPMN (2.8 cm cyst) exhibits worrisome features on imaging but no high-risk stigmata. An endoscopic ultrasound confirms the absence of a solid mural nodule ≥5 mm. Current guidelines would most likely recommend which next step?

  2. Answer Choices

    A. Immediate total pancreatectomy

    B. Close radiologic surveillance with repeat EUS in 3–6 months

    C. Pancreaticoduodenectomy without lymph node dissection

    D. Liver MRI to rule out metastatic disease

  3. Correct Answer

    B. Close radiologic surveillance with repeat EUS in 3–6 months

  4. Explanation

    • Why B is correct: Current guidelines (IAP, European) recommend surveillance for branch-duct IPMNs that lack “high-risk stigmata” (e.g., obstructive jaundice, duct ≥ 10 mm, enhancing mural nodule ≥5 mm). “Worrisome features” justify close follow-up rather than immediate surgery if clearly invasive signs are absent.
    • Why A, C, and D are incorrect:
      • A. Total pancreatectomy is excessive for a lesion of uncertain aggressiveness without high-risk signs.
      • C. Pancreaticoduodenectomy is indicated for head lesions with high-risk features; it also typically includes lymph node dissection.
      • D. Liver MRI is not standard unless there is suspicion of metastatic disease, which is not indicated by the scenario described.
  5. Key Takeaways
    • Branch-duct IPMN management depends on the presence of “worrisome” vs. “high-risk” features.
    • Guidelines help avoid overtreatment of benign or low-risk cysts.
    • Surveillance intervals often vary, but 3–6 months is typical initially for suspicious but not definitively malignant lesions.
    • EUS plays a key role in detecting subtle mural nodules or changes over time.
Reference:
Blumgart's Surgery of the Liver, Biliary Tract and Pancreas, 7th edition,
Chapter 60: "Cystic neoplasms of the pancreas: Epidemiology, clinical features, assessment, and management",
Subtopic: "Branch-Duct IPMN and Guidelines" (pp. 873–875)

Key Takeaways for the Entire Topic

  1. Rising Incidence of Pancreatic Cysts
    • With better imaging modalities (CT, MRI/MRCP) and an aging population, more asymptomatic cystic pancreatic lesions are being discovered.
  2. Types and Malignant Potential
    • Common neoplastic cysts include IPMNs, MCNs, SCAs, and SPTs, each with distinct risk profiles.
    • Mucinous lesions (MCN, IPMN) carry a higher risk of malignant transformation.
  3. Diagnostic Workup
    • Cross-sectional imaging (CT, MRI/MRCP) combined with EUS-FNA (cyst fluid CEA, cytology, molecular markers) aids in accurate classification and malignancy risk assessment.
  4. Guideline-Driven Management
    • Important factors: cyst size, presence of mural nodules, main duct dilation, and solid components.
    • High-risk stigmata typically prompt surgical resection, while smaller or “worrisome” cysts often warrant surveillance.
  5. Surgical Considerations
    • For main-duct IPMN or large MCN, resection is often recommended due to higher malignant potential.
    • Extent of resection (segmental vs. total pancreatectomy) must balance complete removal of at-risk tissue against postoperative morbidity (insulin-dependent diabetes, exocrine insufficiency).

End of Advanced MCQs and Overall Key Takeaways