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Part 2: 5 Advanced-Level MCQs (With More Challenging Options)

MCQ 6 (Critical Analysis / Genetics)

Question

A young patient with a newly diagnosed Solid Pseudopapillary Epithelial Neoplasm (SPEN) undergoes tumor genetic testing. Which of the following specific mutations is most commonly associated with SPEN?

Answer Choices

A. Beta-catenin (CTNNB1) exon 2 mutation

B. KRAS G12D mutation

C. GNAS R201C mutation

D. Beta-catenin (CTNNB1) exon 3 mutation

Correct Answer

D. Beta-catenin (CTNNB1) exon 3 mutation

Explanation

  • SPENs almost universally harbor alterations in the APC/β-catenin pathway, specifically involving CTNNB1 (exon 3). This leads to abnormal β-catenin expression and tumorigenesis.
  • KRAS mutations (option B) are commonly seen in pancreatic ductal adenocarcinoma, and GNAS mutations (option C) are typical in IPMN. CTNNB1 exon 2 mutations (option A) are not characteristic of SPEN.

Key Takeaways

  • SPEN’s unique molecular fingerprint sets it apart from other pancreatic tumors.
  • Detection of CTNNB1 mutation supports the diagnosis and differentiates SPEN from adenocarcinoma or IPMN.
  • Understanding the genetic profile aids in accurate histopathological classification and potentially future targeted therapies.

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: Genetic Alterations in SPEN (pp. 874–875)

MCQ 7 (All Are True EXCEPT / Management Nuances)

Question

All of the following statements about the management of Solid Pseudopapillary Epithelial Neoplasm (SPEN) are true EXCEPT:

Answer Choices

A. Even large tumors with local vascular encasement can often be resected completely.

B. Approximately 15% of SPEN cases present with metastatic disease.

C. Definitive surgery is contraindicated in any patient with liver metastases.

D. Complete (R0) resection typically confers excellent long-term survival.

Correct Answer

C. Definitive surgery is contraindicated in any patient with liver metastases.

Explanation

  • Although metastatic disease to the liver occurs in around 15% of SPEN patients, surgery may still be indicated if resection of both the primary tumor and metastases is feasible, potentially offering prolonged survival.
  • The other statements are accurate: large or vascularly invasive SPENs can often be resected (option A), metastases do occur in ~15% (option B), and complete resection is associated with excellent outcomes (option D).

Key Takeaways

  • SPEN has an indolent course but can be locally invasive or metastatic.
  • Metastases, particularly to the liver, do not universally rule out surgery. Selective resection can yield good survival.
  • Aggressive surgical approaches (when feasible) often lead to long-term disease control.

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: Surgical Considerations for SPEN (pp. 874–875)

MCQ 8 (Scenario-Based / Postoperative Follow-up)

Question

A 28-year-old male underwent a Whipple procedure (pancreaticoduodenectomy) for a SPEN in the pancreatic head. Histopathology shows negative surgical margins, but one of the sampled lymph nodes is positive. Which statement regarding his prognosis and follow-up is most accurate?

Answer Choices

A. Lymph node positivity reduces 5-year survival below 20%, making additional surgery mandatory.

B. Adjuvant chemotherapy (e.g., gemcitabine) is universally recommended for all nodal-positive SPENs.

C. Long-term follow-up imaging is advised because recurrences can occur despite an overall favorable prognosis.

D. Stereotactic body radiotherapy (SBRT) is the primary treatment after R0 resection for nodal involvement.

Correct Answer

C. Long-term follow-up imaging is advised because recurrences can occur despite an overall favorable prognosis.

Explanation

  • SPEN generally carries a good prognosis even with some adverse features such as lymph node involvement; however, close surveillance is crucial because late recurrences can happen.
  • A: While nodal involvement can be a negative prognostic factor, 5-year survival can still be quite high.
  • B and D: There is no uniform recommendation for adjuvant chemotherapy or radiotherapy in SPEN; complete resection remains the key.

Key Takeaways

  • A positive lymph node does not preclude a favorable outcome in SPEN.
  • The indolent nature of this tumor allows for good long-term survival if resected completely.
  • Regular follow-up is critical for early detection of potential recurrence or metastasis.

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: Postoperative Outcomes and Follow-up (pp. 874–875)

MCQ 9 (Interpretation / Problem-Solving)

Question

A 32-year-old female presents with a large SPEN in the pancreatic tail, abutting the splenic hilum and involving the splenic vein. Imaging suggests resectability. Which surgical approach is most appropriate?

Answer Choices

A. Distal pancreatectomy with preservation of the spleen using the Warshaw technique

B. Distal pancreatectomy with en bloc splenectomy

C. Simple enucleation of the tumor without splenic vessel ligation

D. Total pancreatectomy to ensure negative margins

Correct Answer

B. Distal pancreatectomy with en bloc splenectomy

Explanation

  • Involvement of the splenic vein (and often the splenic hilum) typically necessitates a distal pancreatectomy with splenectomy to achieve clear margins and manage vascular involvement.
  • The Warshaw technique (option A) can sometimes preserve the spleen, but direct vein involvement at the hilum often makes splenic preservation difficult.
  • Simple enucleation (option C) is inadequate for large, invasive tumors.
  • A total pancreatectomy (option D) is not routinely indicated when a distal resection can achieve R0.

Key Takeaways

  • Distal pancreatectomy is the standard for lesions in the body/tail of the pancreas; splenectomy is often required if there is vascular or hilar involvement.
  • SPEN can invade adjacent structures, but complete resection usually confers an excellent prognosis.
  • Splenic preservation might be attempted in select cases, but not typically when there is direct splenic vessel involvement.

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: Surgical Approach to Pancreatic Body/Tail SPEN (pp. 874–875)

MCQ 10 (Controversies / Comparative Insight)

Question

Which of the following features best distinguishes Solid Pseudopapillary Epithelial Neoplasm (SPEN) from a Pancreatic Neuroendocrine Tumor (PNET)?

Answer Choices

A. SPEN typically shows focal positivity for synaptophysin, whereas PNET is universally negative

B. SPEN is almost always positive for chromogranin, whereas PNET is chromogranin-negative

C. SPEN is chromogranin-negative, whereas PNET usually demonstrates chromogranin positivity

D. SPEN arises only in the tail, whereas PNET predominantly arises in the head

Correct Answer

C. SPEN is chromogranin-negative, whereas PNET usually demonstrates chromogranin positivity

Explanation

  • SPEN generally lacks chromogranin expression, helping differentiate it from PNET, which is typically chromogranin-positive (and often positive for synaptophysin as well).
  • Options A and B reverse known immunohistochemical findings. Option D is an oversimplification; both tumors can arise in different regions of the pancreas.

Key Takeaways

  • Chromogranin expression is a key immunomarker to distinguish a neuroendocrine origin (PNET) from SPEN.
  • While clinical presentation and imaging features may overlap, IHC remains definitive in ambiguous cases.
  • SPEN and PNET can occur in any pancreatic region; location alone is not diagnostic.

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: Distinguishing SPEN from Other Pancreatic Tumors (pp. 874–875)

Key Takeaways for the Entire Topic

  • Solid Pseudopapillary Epithelial Neoplasm (SPEN) predominantly occurs in young females and often presents as a large, well-demarcated lesion with both solid and cystic components.
  • Immunohistochemistry is central to diagnosis: SPEN commonly expresses vimentin, NSE, and AMACR but is chromogranin-negative, contrasting it with neuroendocrine tumors.
  • Nearly all SPENs feature a mutation in the CTNNB1 (exon 3) gene, setting them apart genetically from other cystic neoplasms (e.g., MCN, IPMN).
  • Complete surgical resection (R0) remains the cornerstone of treatment and offers excellent long-term survival, even with occasional metastatic disease.
  • Continued surveillance is advised due to the potential, albeit low, risk of late recurrence.