Part 1: 5 Basic-Level MCQs (Revised: Numbering Removed)

MCQ 1 (Image-Based / Scenario)

Question

A 35-year-old female presents with vague abdominal pain. A contrast-enhanced CT scan (see provided image) reveals an 8 × 9 cm solid-cystic mass in the head of the pancreas. Which of the following is the most likely diagnosis?

Answer Choices

A. Mucinous Cystic Neoplasm (MCN)

B. Intraductal Papillary Mucinous Neoplasm (IPMN)

C. Serous Cystadenoma (SCN)

D. Solid Pseudopapillary Epithelial Neoplasm (SPEN)

Correct Answer

D. Solid Pseudopapillary Epithelial Neoplasm (SPEN)

Explanation

SPEN (Frantz tumor) typically presents in young females (9:1 ratio) around the second to third decade of life. It often manifests as a large encapsulated lesion with both solid and cystic components.
MCN more commonly occurs in middle-aged women in the body or tail of the pancreas and lacks the classic hemorrhagic or necrotic features.
IPMN shows ductal communication and mucin production.
SCN usually presents as a microcystic lesion (“honeycomb” appearance), more often in older women, and does not typically have hemorrhagic or solid components.

Key Takeaways

SPEN predominantly affects younger females.
The tumor commonly appears as a solid-cystic mass that may contain hemorrhagic or necrotic areas.
Ductal dilatation is rare in SPEN.
Surgical resection offers an excellent prognosis.

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 Epithelial Neoplasm (pp. 874–875)

MCQ 2 (Factual / One-Liner)

Question

Which of the following best describes the typical epidemiology of Solid Pseudopapillary Epithelial Neoplasm (SPEN) of the pancreas?

Answer Choices

A. Predominantly affects men in their 60s

B. Predominantly affects young women (mean age ~29 years)

C. Equal incidence in men and women in their 40s

D. Most common in elderly individuals with a history of chronic pancreatitis

Correct Answer

B. Predominantly affects young women (mean age ~29 years)

Explanation

SPEN shows a marked female predominance (9:1) and occurs in young patients (mean age of 29 years).
Options A, C, and D contradict the well-established demographic data.

Key Takeaways

SPEN is a rare tumor but commonly affects younger females.
It may be asymptomatic or present with vague abdominal pain.
Recognition and early surgical management are vital.

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: Epidemiology of SPEN (pp. 874–875)

MCQ 3 (All Are True EXCEPT)

Question

All of the following are typical imaging findings for Solid Pseudopapillary Epithelial Neoplasm (SPEN) EXCEPT:

Answer Choices

A. Well-encapsulated lesion

B. Solid and cystic components with areas of hemorrhage

C. Possible calcifications within the lesion

D. Marked main pancreatic duct dilation

Correct Answer

D. Marked main pancreatic duct dilation

Explanation

SPEN rarely causes significant pancreatic ductal dilation.
A, B, and C are classic imaging features: an encapsulated lesion, solid-cystic appearance (often with hemorrhage or necrosis), and occasional calcifications.

Key Takeaways

SPEN is typically encapsulated and contains variable solid and cystic areas.
Hemorrhage or necrosis within the lesion is a common finding.
Absence of ductal involvement helps differentiate SPEN from certain other pancreatic tumors.

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: Imaging Findings of SPEN (pp. 874–875)

MCQ 4 (Descriptive / Knowledge-Based)

Question

Immunohistochemistry for Solid Pseudopapillary Epithelial Neoplasm (SPEN) of the pancreas typically shows negativity for which one of the following markers?

Answer Choices

A. Neuron-Specific Enolase (NSE)

B. Vimentin

C. AMACR (Alpha-methylacyl-CoA racemase)

D. Chromogranin

Correct Answer

D. Chromogranin

Explanation

SPEN is usually chromogranin-negative, distinguishing it from pancreatic neuroendocrine tumors (which are often chromogranin-positive).
NSE, vimentin, and AMACR are frequently positive in SPEN.

Key Takeaways

SPEN has a unique immunophenotype, commonly expressing vimentin, keratin, NSE, and AMACR.
It is chromogranin-negative, helping rule out neuroendocrine origin.
Accurate immunohistochemical profiling is essential for correct diagnosis.

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: Pathology of SPEN (pp. 874–875)

MCQ 5 (Scenario-Based / Application)

Question

A 25-year-old female is found to have a 6 cm Solid Pseudopapillary Epithelial Neoplasm (SPEN) in the tail of the pancreas. She is otherwise asymptomatic. What is the recommended management?

Answer Choices

A. Observation with serial imaging

B. Endoscopic ultrasound-guided cyst ablation

C. Surgical resection (distal pancreatectomy)

D. Chemotherapy and radiation therapy

Correct Answer

C. Surgical resection (distal pancreatectomy)

Explanation

Complete surgical removal is indicated even if asymptomatic because SPEN has malignant potential (~15% may metastasize).
Ablation, observation, or chemotherapy/radiation are not considered definitive treatments for SPEN.

Key Takeaways

Surgery is curative in most cases and is the standard of care for localized SPEN.
Distal pancreatectomy is typically performed for lesions in the body or tail.
Surgical resection confers an excellent prognosis for most patients.

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: Management of SPEN (pp. 874–875)

Part 2: 5 Advanced-Level MCQs

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 genetic alterations is most commonly associated with SPEN?

Answer Choices

A. KRAS mutation

B. GNAS mutation

C. SMAD4 deletion

D. CTNNB1 exon 3 mutation

Correct Answer

D. CTNNB1 exon 3 mutation

Explanation

Almost all SPENs harbor alterations in the APC/β-catenin pathway, specifically involving CTNNB1 (exon 3).
KRAS, GNAS, and SMAD4 mutations are frequently observed in other pancreatic tumors (e.g., IPMN, adenocarcinoma), but not in SPEN.

Key Takeaways

SPEN has a distinct molecular profile compared to other pancreatic neoplasms.
CTNNB1 mutations lead to abnormal β-catenin expression, driving tumorigenesis.
Molecular testing can help differentiate SPEN from other cystic pancreatic lesions.

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 very large tumors can often be resected completely.

B. Metastatic disease is present in about 15% of patients.

C. Presence of metastatic disease universally precludes surgical intervention.

D. Complete surgical resection usually confers long-term survival.

Correct Answer

C. Presence of metastatic disease universally precludes surgical intervention.

Explanation

Although metastases occur in about 15% of SPEN patients, surgery may still be offered if a complete resection (including metastasectomy) is feasible. Long-term survival can be achieved despite metastatic disease in selected cases.
The other statements (A, B, D) reflect established facts: large tumors are often amenable to en bloc resection, approximately 15% have metastases at presentation, and complete surgical removal typically yields excellent survival outcomes.

Key Takeaways

SPEN is an indolent tumor with the potential for local invasion and metastasis, but complete resection remains the cornerstone of treatment.
The presence of metastases should not automatically exclude a patient from surgery if resectable.
Proper patient selection is crucial to maximize survival benefits.

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 / Application – Postoperative Follow-up)

Question

A 28-year-old male underwent a Whipple procedure (pancreaticoduodenectomy) for a SPEN in the pancreatic head. Histopathology confirmed negative surgical margins; however, one peripancreatic lymph node was positive. Which statement about his prognosis and follow-up is most accurate?

Answer Choices

A. Positive lymph nodes mean no chance of long-term survival.

B. Adjuvant chemotherapy is mandatory for all SPEN cases.

C. Long-term surveillance is recommended despite excellent overall prognosis.

D. Radiotherapy is curative for any lymph node-positive disease.

Correct Answer

C. Long-term surveillance is recommended despite excellent overall prognosis.

Explanation

Even with some negative prognostic factors (e.g., positive lymph node), SPEN often carries a favorable prognosis if resected. However, careful follow-up is advised since recurrence can occur.
A: Long-term survival is still possible despite nodal positivity.
B and D: There is no standardized role for adjuvant chemo or radiotherapy in SPEN, as surgical resection is typically curative.

Key Takeaways

SPEN outcomes can remain good even in the presence of some “high-risk” features.
Surgical clearance is the key determinant of prognosis; residual disease is the main risk factor for recurrence.
Follow-up imaging and clinical evaluation help detect any late recurrences or metastases.

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 with a large SPEN in the tail of the pancreas abutting the splenic hilum has involvement of the splenic vein. What is the most appropriate surgical approach if the lesion is deemed resectable?

Answer Choices

A. Simple enucleation of the tumor

B. Distal pancreatectomy with possible splenectomy

C. Total pancreatectomy

D. Bypass palliation without resection

Correct Answer

B. Distal pancreatectomy with possible splenectomy

Explanation

For SPEN located in the body or tail, distal pancreatectomy is the standard surgical procedure. Due to vascular involvement (especially the splenic vessels), splenectomy is often performed en bloc to achieve negative margins.
Enucleation is typically not feasible for large lesions that invade or abut major vessels.
Bypass palliation is inappropriate when complete resection is possible.
Total pancreatectomy is generally reserved for multifocal or extensive disease not amenable to partial resection.

Key Takeaways

SPEN often remains surgically resectable despite local vascular involvement.
Distal pancreatectomy with splenectomy is commonly performed for tail lesions invading the splenic vessels.
Complete resection is crucial for optimal long-term outcomes.

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 distinguishes Solid Pseudopapillary Epithelial Neoplasm (SPEN) most clearly from a Pancreatic Neuroendocrine Tumor (PNET)?

Answer Choices

A. Location in the pancreas

B. Typical young female predominance

C. Chromogranin negativity

D. CT appearance of well-defined lesion

Correct Answer

C. Chromogranin negativity

Explanation

SPEN does not express chromogranin, whereas PNETs are typically chromogranin-positive.
SPEN does tend to affect young females (option B), but age and gender alone are not always definitive. Both SPEN and PNET can appear as well-defined lesions on imaging (option D). The distribution across pancreatic locations can overlap (option A).

Key Takeaways

Immunohistochemistry remains a key tool in distinguishing SPEN from other pancreatic tumors.
Clinical demographics and imaging features can overlap among different tumor types, but chromogranin status is a more reliable discriminator in this context.
Accurate histopathological diagnosis is essential for appropriate treatment planning.

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 with a large, well-encapsulated pancreatic mass containing solid and cystic components.
Characteristic immunohistochemical profile includes positivity for NSE, vimentin, and AMACR, but negativity for chromogranin.
SPEN has a distinct genetic mutation (CTNNB1), differentiating it from other pancreatic neoplasms such as IPMN or adenocarcinoma.
Complete surgical resection is the mainstay of treatment, offering an excellent long-term prognosis even in some cases with metastatic disease.
Ongoing surveillance is recommended due to the potential for local recurrence or metastasis, though overall progression rates are low.