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Tumors of Pancreas & Ampulla

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MCQ - Pancreatic Cancer

Explanation:

  • Cigarette smoking has been definitively identified as a causative agent in 25% to 35% of the cases of pancreatic ductal adenocarcinoma (PDAC) and is the most consistently reported risk factor.
  • There is a dose-response relationship between PDAC and the number of cigarettes consumed.
  • Diabetes and prediabetes are risk factors for pancreatic cancer and may also be a consequence of pancreatic cancer.
  • The chronic hyperinsulinemia and hyperglycemia associated with type 2 diabetes has been proposed as the underlying mechanism for pancreatic cancer. Every 0.56 mmol/L increase in fasting blood glucose is associated with a 14% increase in the rate of pancreatic cancer.
  • Helicobacter pylori has emerged as a moderate risk factor for PDAC.
  • Lower levels of Neisseria elongate and Streptococcus mitis and high levels of Porphyromonas gingivalis and Granulicatella adiacens in the gut are associated with an increased risk of pancreatic cancer.
  • New-onset insulin-dependent diabetes after the age of 60 has a 1% to 2% incidence of a previously undiagnosed pancreas cancer.
  • CA19-9 is a carbohydrate antigen linked to the Lewis blood group antigens.
  • 15% to 20% of patients are Lewis antigen negative and are incapable of synthesizing CA19-9.

Chronic hypoinsulinemia is not an underlying mechanism for pancreatic cancer, making statement D false.

MCQ - Hereditary Syndromes and Duodenal Adenoma

Explanation:

  • The most important hereditary syndrome contributing to the development of duodenal adenoma is familial adenomatous polyposis (FAP).
  • Duodenal adenomas develop in nearly all patients with FAP.
  • The most common site for these adenomas is the periampullary segment of the duodenum.
  • Duodenal adenoma (DA) and desmoid tumors are the leading causes of death in patients with FAP who have undergone colectomy.

Conditions associated with an increased risk of duodenal adenoma include:

  • Familial adenomatous polyposis (FAP)
  • Lynch syndrome
  • Peutz-Jeghers syndrome
  • Inflammatory bowel disease
  • Celiac disease

MCQ - Lymph Node Removal in Small Bowel and Duodenal Cancer

Explanation:

  • The NCCN guidelines recommend that at least eight lymph nodes be evaluated for adequate staging of small bowel and duodenal cancer.

Additional Information

Indications for Adjuvant Therapy in Duodenal Adenocarcinoma:

  • Positive lymph nodes (Stage III disease)
  • Stage II disease with high-risk tumor features such as:
    • T4 tumors
    • Poorly differentiated histology
    • Lymphovascular or perineural invasion
    • Tumor perforation

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MCQ - Pancreatic Cancer Characteristics

Explanation:

  • The most common neoplasm of the pancreas is pancreatic ductal adenocarcinoma (PDAC), not pancreatic metastases. PDAC accounts for more than 90% of pancreatic neoplasms.
  • The median age at diagnosis for PDAC is approximately 70 years.
  • Patients with untreated PDAC, or with metastatic disease, have a mean survival of less than 6 months.
  • A desmoplastic reaction surrounding the malignant epithelium is typical in PDAC.
  • The tumor cells of typical PDAC are positive for CK7 and negative for CK20 and CDX2.
  • They also express carcinoma biomarkers such as MUC1, CEA, CA125, and B72.3.

MCQ - Imaging Findings in Pancreatic Ductal Adenocarcinoma (PDAC)

Explanation:

  • The duct-penetrating sign is visualization of the normal or non-obstructed duct in the focally abnormal pancreatic segment, which is characteristic of focal pancreatitis, not PDAC.
  • CECT findings of pancreatic ductal adenocarcinoma include:
    • Negative duct-penetrating sign
    • Double duct sign: Peripapillary obstruction (specificity, 63%-80%; sensitivity, 50%-76%)
    • Dilated peripancreatic veins
    • Tear drop sign: Deformed shape of the superior mesenteric vein as a result of tumoral involvement

Summary of Imaging Findings from the Attached Table:

  • Duct-penetrating sign: Typically absent in PDAC, may be present in mass-forming pancreatitis (reliable sign of a benign abnormality, specificity 96%, sensitivity 85%).
  • Collateral duct dilatation: Typically absent in PDAC, may be present in mass-forming pancreatitis.
  • Duct-to-parenchyma ratio >0.34: May be present in PDAC, typically absent in mass-forming pancreatitis (reliable sign of malignancy, specificity 97%, sensitivity 94%).
  • Displaced calcifications: May be present in PDAC, typically absent in mass-forming pancreatitis.
  • Double duct sign: May be present in PDAC, typically absent in mass-forming pancreatitis.
  • SMA-to-SMV ratio >1: May be present in PDAC, typically absent in mass-forming pancreatitis (indicative of peritumoral fatty infiltration leading to deformity and decreased caliber of the SMV).
  • Vessel encasement or deformity: May be present in PDAC, typically absent in mass-forming pancreatitis (occasionally seen in autoimmune pancreatitis).

Autoimmune pancreatitis: Characterized by a diffuse sausage-shaped pancreas.

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MCQ - Periampullary Cancer

The most common periampullary cancer is:

  • A. Pancreatic head ductal adenocarcinoma
  • B. Distal cholangiocarcinoma
  • C. Duodenal adenocarcinoma
  • D. Ampullary adenocarcinoma

Answer: A

Explanation:

  • The most common periampullary malignancy is pancreatic ductal adenocarcinoma (PDAC), followed by ampullary adenocarcinoma, distal cholangiocarcinoma, and duodenal adenocarcinoma.
  • Duodenal adenocarcinoma of the periampullary region is the least common of the main periampullary cancers.

MCQ - Adenosquamous Carcinoma of Pancreas

To define adenosquamous carcinoma of the pancreas, the squamous component should be:

  • A. More than 30%
  • B. More than 0%
  • C. More than 90%
  • D. More than 10%

Answer: A

Explanation:

  • The classification of an adenosquamous carcinoma requires the presence of greater than or equal to 30% squamous component.

MCQ - Colloid Carcinoma of Pancreas

Which of the following statements regarding colloid carcinoma of the pancreas is true?

  • A. Usually arises from pancreaticobiliary type of IPMN
  • B. Colloid carcinoma tumor cells can have focal poorly differentiated signet cell morphology
  • C. Colloid carcinoma has worse prognosis than pancreatic ductal adenocarcinoma
  • D. All the above statements are true

Answer: B

Explanation:

  • Colloid carcinoma of the pancreas is usually derived from intestinal type intraductal papillary mucinous neoplasia (IPMN).
  • The defining feature of colloid carcinoma is that at least 80% of the neoplastic epithelium of the tumor is suspended in mucin.
  • The tumor cells can have focal poorly differentiated signet ring cell morphology.
  • Most are well or moderately differentiated.
  • The tumor is immunoreactive for CK20, CDX2, and MUC2.
  • Patients with colloid carcinoma have more favorable outcomes than those with conventional PDAC, with a 5-year survival of greater than 55%.

MCQ - Location of Acinar Cell Carcinoma of Pancreas

Most common location of acinar cell carcinoma of the pancreas is:

  • A. Pancreatic head
  • B. Uncinate process
  • C. Pancreatic body and tail
  • D. Neck of the pancreas

Answer: A

Explanation:

  • Acinar cell carcinoma (ACC) is most commonly located in the pancreatic head (47%), followed by the tail (45%), neck (3%), and uncinate process (3%).
  • When neuroendocrine cells comprise greater than 35% of the tumor, it qualifies as a mixed acinar-neuroendocrine carcinoma.
  • ACCs express BCL10, which is absent in pancreatic ductal adenocarcinomas and neuroendocrine tumors.

MCQ - Acinar Cell Carcinoma of Pancreas

Which of the following statements is true regarding acinar cell carcinoma of the pancreas?

  • A. More common in females
  • B. Age of onset 20 to 30 years
  • C. Most commonly associated with FAP and Carney's complex
  • D. Metastatic acinar cell carcinoma can present with subcutaneous fat necrosis and polyarthralgia

Answer: D

Explanation:

  • Acinar cell carcinoma has a male sex predominance (2:1).
  • The age of onset is typically 60 to 70 years (age range 2-88 years).
  • The majority of cases are sporadic, but rare cases have been diagnosed in association with familial adenomatous polyposis (FAP), Lynch syndrome, and Carney's complex.
  • When associated with disseminated metastasis, patients may present with subcutaneous fat necrosis and polyarthralgia due to hypersecretion of pancreatic enzymes (particularly lipase) from the tumor cells.
  • Alpha-fetoprotein may be present at increased levels in young patients.
  • Immunohistochemical staining of trypsin and/or chymotrypsin has the highest degree of sensitivity.

MCQ - Genetic Mutation in Pancreatic Ductal Adenocarcinoma

Explanation:

  • Somatic genetic drivers of pancreatic ductal adenocarcinoma (PDAC) include:
    • Oncogenic mutations of KRAS (>90%)
    • Mutation or deletion of TP53 (75%)
    • Mutation or deletion of CDKN2A (80%)
    • Mutation or deletion of SMAD4 (55%)

MCQ - Time to Development of Clinically Visible Pancreatic Tumor

Explanation:

  • An average of 11.7 years is required from tumor initiation to the development of a clinically visible primary pancreatic tumor. It takes an additional 6.8 years for the development of metastatic disease.

MCQ - Pancreatoblastoma

Explanation:

  • Pancreatoblastoma is associated with Beckwith-Wiedemann syndrome and familial adenomatous polyposis, but it is not most commonly associated with Beckwith-Wiedemann syndrome.
  • The essential feature for diagnosis is the presence of squamoid nests.
  • It can cause Cushing syndrome in rare cases due to inappropriate ACTH secretion.
  • Pancreatoblastoma associated with Beckwith-Wiedemann syndrome may be predominantly cystic.

Additional Information:

  • Pancreatoblastoma prognosis is more favorable in children than adults.
  • Markedly elevated serum alpha-fetoprotein (AFP) is common in two-thirds of cases, particularly in children.
  • The loss of chromosome 11p occurs in more than 80% of both sporadic pancreatoblastomas and cases of children with Beckwith-Wiedemann syndrome.

MCQ - Perivascular Epithelioid Cell Tumor (PEComa) of Pancreas

Explanation:

  • Perivascular epithelioid cell tumor (PEComa), also known as angiomyolipoma.
  • PEComa of the pancreas has a strong female predominance.
  • Tumors are usually located in the head and body of the pancreas.
  • Leiomyosarcoma is the most common primary sarcoma of the pancreas, not PEComa.

MCQ - Most Common Primary Sarcoma of the Pancreas

Explanation:

  • Leiomyosarcoma is the most common primary sarcoma of the pancreas.
  • Solitary fibrous tumor can rarely present with Doege-Potter syndrome (hypoglycemia).
  • Inflammatory myofibroblastic tumor: Immunoreactivity with anaplastic lymphoma kinase 1 (ALK-1) is a diagnostic hallmark.

MCQ - Primary Pancreatic Lymphoma

Explanation:

  • Primary Pancreatic Lymphoma (PPL):
    • Diffuse large B-cell lymphoma (DLBCL) is the most common histologic subtype of PPL, followed by follicular lymphoma.
    • The most common location of PPL is the head of the pancreas.
    • Jaundice is not a common symptom of PPL; the most common symptoms are typically related to abdominal pain or a palpable mass.

Thus, the statement "most common symptom - jaundice" is incorrect.