Curriculum for Specialty Certificate Examination in Gastroenterology

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Sunday 18 November 2012

Cystic Pancreatic tumours

1- Serous Cystadenoma:
10% of all cystic lesions of the pancreas
Large, palpable asymptomatic upper abdominal mass.
occur equaly in males and females
CT scan: shows a lobulated cystic mass with a central scar, microcytic cluster of small cysts. 20-30% have stellate scar "sunburst".
EUS: shows multiple anechoic cystic cavities 
Histologically consist of multiple tiny cysts that contain watery fluid.
While SCAs rarely have malignant potential, surgical resection for symptomatic lesions or lesions greater than 4 cm is recommended in good operative candidates.





2- Mucinous Cystadenoma:
Almost exclusively in women 40-60 years old
Usually have thick fibrotic walls and can be multiple
Cysts do not communicate with the main pancreatic duct
1-2% of pancreatic endocrine tumours
Histologically consist of multiple cysts containing sticky mucous
The stroma is refered to as 'ovarian like'
Treatment is surgical resection in approperiate patients.




3-Intraductal Papillary Mucinous Tumour IPMT:
Consist of intraductal papillary growth of mucin-producing columnar epithelium.
obstructive pancreatits frequently occurs
CT: dilated main or side ducts
Main duct IPMN: Because of the high risk of invasive cancer in the main duct, surgical resection is recommended ( Whipple's )
Side chain IPMN: still high risk of malignancy. advice resection or survay
At ERCP, half of the patients have the diagnostic finding of a patulous papilla extruding mucous.


9 comments:

  1. A 68-year-old Caucasian male was admitted with acute onset of severe epigastric abdominal pain associated with nausea and vomiting. The patient denied alcohol use and his fasting triglycerides checked during recent annual physical examination were normal. Patient had a similar episode 3 months ago which required hospital admission. He has lost 10 lbs in the last 3 months. Clinical examination revealed an anicteric male who had low-grade pyrexia and epigastric tenderness. Serum amylase was 1800 U/L (normal < 130 U/L) and lipase was >3000 U/L (normal < 95 U/L). CT scan of abdomen with oral and intravenous contrast shows a dilated main pancreatic duct in the head of pancreas. The endoscopic view during ERCP is shown in the figure. The appropriate next step in management of this case is:

    A. Check IgG4 level

    B. Pancreatic enzyme supplement

    C. Refer for pancreaticoduodenectomy (Whipple procedure)

    D. Follow-up with MRCP in 3 months

    correct answer C,
    The endoscopic view shows a patulous ampulla with extruding mucus typical of intraductal papillary mucinous tumor (IPMT). Pancreaticoduodenectomy is the treatment of choice for patients with symptomatic IPMT in the head of pancreas. IPMT can present with abdominal pain or recurrent pancreatitis. The disease is commonly localized to the head of pancreas and can mimic mucinous cystic neoplasm on the CT scan. Autoimmune pancreatitis does not present with mucous extruding from the papilla nor does it usually present with a dilated pancreatic duct. The other two choices are not appropriate for management of symptomatic IPMT.

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  2. A 66-year-old man is evaluated for a 6 cm cystic lesion in the body of the pancreas that was found incidentally on CT scan (sse fig 1), which was performed 6 days ago to evaluate nephrolithiasis [see figure]. The lesion does not appear to communicate with the main pancreatic duct. The patient does not have a personal or family history of acute pancreatitis, chronic pancreatitis, or pancreatic cancer. On physical examination, abdominal palpation discloses no abdominal tenderness, masses, or hepatosplenomegaly. Laboratory studies, including a complete blood count, complete metabolic profile, amylase, and lipase, are normal. Endoscopic ultrasound confirms the CT scan findings, and fine needle aspiration is performed. Analysis of the cyst fluid reveals a carcinoembryonic antigen level of less than 5 micrograms/L (normal ≤ 5 ng/mL). Which of the following is the most likely diagnosis?

    A. Intraductal papillary mucinous neoplasm

    B. Mucinous cystic lesion

    C. Pancreatic pseudocyst

    D. Serous cystadenoma

    correct answer D,
    The patient likely has a serous cystadenoma. Cystic neoplasms of the pancreas are rare tumors comprising only 10% of pancreatic cysts and 1% of pancreatic cancers. However, with the increased use of CT and MRI imaging, incidental pancreatic cysts have been detected in up to 20% of the population. If an inflammatory benign pseudocyst can be ruled out, the differential diagnosis of pancreatic cystic neoplasms includes the benign serous cystadenomas; the premalignant mucinous cystadenomas and frankly malignant mucinous cystadenocarcinoma; and main branch and side branch intraductal papillary mucinous neoplasms. Morphologic features on imaging and cytologic examination of cyst fluid obtained by fine-needle aspiration as well as analysis for amylase and tumor markers, such as carcinoembryonic antigen, can aid in detecting malignant lesions. The morphologic features of microcysts and a central scar are pathognomonic for a serous cystadenoma. The diagnosis is supported by the cyst fluid carcinoembryonic antigen (CEA) level of less than 5 micrograms/L. In addition, the cyst does not communicate with the main pancreatic duct. The lesion is not a mucinous cystic neoplasm nor an intraductal papillary mucinous neoplasm (IPMN) because of its morphologic appearance (honeycomb appearance) and its low CEA level. IPMN and mucinous cystic neoplasms generally have few compartments. Pancreatic pseudocysts are the most common complication of acute pancreatitis, usually appearing several weeks following pancreatic injury or inflammation. This patient has no history of pancreatitis; therefore, a pancreatic pseudocyst would be an unlikely diagnosis.

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  3. A 43-year-old woman is evaluated in the emergency department for an 8 hour history of epigastric abdominal pain that radiates to the back. The pain is accompanied by nausea, vomiting, and anorexia. She has no history of alcohol use, gallbladder disease, abdominal trauma, or acute pancreatitis. Up until this time, she has been well and has no other medical problems and takes no medications. On physical examination, temperature is 38.4°C (101.2°F), blood pressure is 143/76 mm Hg, pulse rate is 96/min, and respiration rate is 22/min. BMI is 26. The oral pharynx is dry. Abdominal examination discloses hypoactive bowel sounds, a distended abdomen, tenderness with palpation, and guarding. Laboratory studies indicate normal liver tests but a lipase of 987 units/L (normal < 95 units/L). A contrast-enhanced CT scan is performed [see fig 2]. Which of the following is the most likely cause for this patient’s acute pancreatitis?

    A. Mucinous cystadenoma

    B. Pancreas divisum

    C. Pancreatic fistula

    D. Pancreatic pseudocyst

    correct answer A,
    This patient most likely has a mucinous cystic lesion. Based on the clinical presentation and laboratory studies, this patient has acute pancreatitis. It is important to determine the cause of the pancreatitis. A middle-aged woman without risk factors for pancreatitis who presents with a cystic lesion in the body of the pancreas may have a mucinous cystic neoplasm. Once her pancreatitis resolves, the lesion should be resected if further evaluation, such as with endoscopic ultrasound, demonstrates this to be a mucinous lesion. Pancreas divisum is a congenital pancreatic ductal anomaly found in approximately 7% of patients; its prevalence is much higher in patients with idiopathic acute pancreatitis. Diagnosis of pancreas divisum is made with either endoscopic retrograde cholangiopancreatography (ERCP) or magnetic resonance cholangiopancreatography (MRCP); pancreas divisum does not present as a cystic pancreatic mass. An uncommon complication of acute pancreatitis is a pancreatic fistula. Injury to a pancreatic duct can result in leakage of pancreatic secretions into the peritoneal cavity, resulting in pancreatic ascites. Pancreatic fistulas, like pseudocysts, occur many weeks following an episode of acute pancreatitis and would not be the initial presentation of a patient without a history of previous pancreatitis or injury. Pancreatic pseudocysts are the most common complication of acute pancreatitis, usually appearing several weeks following pancreatic injury or inflammation. This patient’s cyst is unlikely to be a pancreatic pseudocyst because this patient has no previous history of acute pancreatitis.

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  4. A 25-year-old fit female tennis star had abdominal computerized tomography (CT) scan after a trauma with the tennis ball. Her CT scan was normal except for a cyst in the pancreas. This had features of a serous cystadenoma in the body.
    What will be the next step:-
    1-Resection of the tail of the pancreas
    2-Resection of the cyst alone.
    3-Resection of the whole of pancreas.
    4-Annual CT scan.
    5-Do nothing.

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  5. Which of the following cystic pancreatic masses does not require surgical resection?

    1-Intraductal papillary mucinous neoplasm (IPMN)
    2-Mucinous cystadenoma
    3-Serous cystadenoma
    4-Solid pseudopapillary neoplasm

    Mucinous cystadenoma, IPMN, and solid pseudopapillary neoplasm all have the potential to behave in a malignant fashion. Serous cystadenoma is considered a benign entity; there have only been isolated reports of a serous cystadenoma behaving in an aggressive fashion and invading local regional structures. The diagnosis of a serous cystadenoma does not require surgical removal of the lesion.

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  6. A 32-year-old woman presents to your office after having been diagnosed with her third episode of acute pancreatitis. She denies history of alcohol use and has no history of hypocalcemia or hypertriglyceridemia. There is no family history of pancreatitis. She is otherwise healthy. A CT scan is performed that demonstrates mild pancreatic ductal dilation and a cyst in the region of the head of the pancreas. ERCP is performed that demonstrates a saccular dilation with a central lucency communicating with the main pancreatic duct. Globules of clear viscous material were seen to extrude through the pancreatic os. Which of the following statements regarding this finding is most correct?

    1-The next appropriate step for this patient would be EUS-guided FNA of the dilated segment.
    2-The patient should be counseled that she has a premalignant lesion and should consider surgical resection.
    3-This finding is diagnostic of a mucinous cystadenoma and requires resection.
    4-This finding is diagnostic of a solid pseudopapillary tumor that may be observed radiographically.
    5-This image demonstrates a pancreatic pseudocyst resulting from recurrent pancreatitis and can be managed with transpapillary drainage.

    The pancreatogram demonstrates a saccular dilation communicating with the main pancreatic duct. A central lucency is seen within the dilated duct segment. These findings, coupled with the presence of mucin extruding through the pancreatic os, are diagnostic of an IPMN. Because IPMN is a premalignant lesion, it should be considered for surgical resection in this healthy young individual. EUS-guided FNA is unlikely to yield additional valuable information. A mucinous cystadenoma does not communicate with the main pancreatic duct and is more commonly found in the tail of the pancreas. Solid pseudopapillary tumors are commonly found in young women, although they do not communicate with the main pancreatic duct. Although a pancreatic pseudocyst is a consideration, the presence of a central lucency within the cyst and mucin seen to extrude from the pancreatic os are diagnostic of IPMN.

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