Hepatobiliary and Pancreatic Neoplasms Flashcards

1
Q

What are the types of hepatobiliary neoplasms?

A

Primary:

Benign:

Haemangiomas (most common)

Hepatocellular adenoma (secretory)

Bile duct adenoma

Malignant:

Most commonly a metastatic tumour from the GI tract. (Portal circulation)

Primary:

Most commonly hepatocellular carcinoma (Misnomer is hepatoma)

Cholangiocarcinoma

Hepatoblastoma - Most common liver tumour in the young

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2
Q

Who most commonly gets liver cell adenoma? What should be tested for when these are seen?

A

Most commonly seen in young women on oral contraceptives.

Should be differentiated from hepatocellular carcinoma

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3
Q

What are the possible complications that can arise from liver cell adenomas?

A

Rupture: sub-capsular

Rarely progresses to HCC (certain subtypes)

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4
Q

What do liver cell adenomas look like? What do they look like on histology?

A

Pale, yellow/tan

Bile stained

Well demarcated usually

On histology:

Well demarcated

Form sheets of hepatocytes

Absent portal tracts

Prominent arterial vessels and draining vessels.

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5
Q

Where are hepato-cellular carcinomas most common?

A

Highest incidence in Asia, Less in NA and Western Europe.

High incidence in countries with high HBV and HCV infection +/- cirrhosis

20 - 40 years

In the West it is only common with cirrhosis and rarely before 60 years of age.

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6
Q

What causes hepatocellular carcinomas?

A

HBV, HCV

Chronic alcoholism

Food contamination by aflatoxins

Haemachromatosis

Other

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7
Q

What are the morphological features of hepatocellular carcinomas?

A

Liver enlarged

Single, multiple or diffusely infiltrative

Usually paler than the surrounding liver

Form satellite nodules which are strong small metastases which are microtumours that have spread from original tumour into surrounding liver tissue.

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8
Q

What are the morphological features of malignant hepatocytes? What are the variations in types of malignant hepatocytes?

A

Large nuclei, nucleoli, mitoses and thick liver cell plates.

Variations:

Well differentiated (acinar/pseudo glandular formations, solid

Poor to undifferentiated (anaplastic)

Fibrolamellar variant - young

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9
Q

How can metastases from the liver be identified on histology?

A

Due to energy rich nature of hepatocytes, the cytoplasm will appear pink and have lots of mitochondria.

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10
Q

How can a diagnosis be made of a hepatocellular carcinoma prior to operating on it?

A

Fine needle aspirate/biopsy

Cytology

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11
Q

How is hepatocellular carcinoma treated?

A

Resection

Transplantation

Chemo-radiation

Newer targeted therapies coming to fruition

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12
Q

What is a cholangiocarcinoma?

A

Malignancy of the biliary epithelium (intrahepatic and extrahepatic, intrapancreatic or extrapancreatic)

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13
Q

What is cholangiocarcinoma associated with?

A

Primary Sclerosin Cholangitis (PSC)

Congenital diseases (Carolis disease)

Exposure to thorotrast (radiological examination no longer used)

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14
Q

Where is cholangiocarcinoma most common?

A

SE Asia: Chronic infection of the biliary tree by liver flukes

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15
Q

What kind of neoplasms are cholangiocarcinomas?

A

Adenocarcinomas with a markedly desmoplastic stroma

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16
Q

What kind of spread do cholangiocarcinomas have? Where do they metastasize to most commonly?

A

Haematogenous spread after vascular invasion. Metastasize to lungs, bone, and brain.

Can also track along the portal tracts to the intestine.

17
Q

How do metastatic tumours present in the liver?

A

Usually multiple and come from the GIT, breast, lung, and most other places.

18
Q

What are the most common pancreatic tumours?

A

Exocrine:

Benign: Serous cyst adenoma

Pre-malignant: Cystic neoplasms; mucinous cystic neoplasm (MCN)

Intraductal papillary mucinous neoplasm - low and high grade (+/- invasive carcinoma)

Malignant: Pancreatic adenocarcinoma:

Acinar cell carcinoma (5%) Solid pseudopapillary tumour (SPN)

Endocrine: Pancreatic neuroendocrine neoplasm (PEN) - low and high grade)

19
Q

What is the most common tumour of the pancreas?

A

Pancreatic neuroendocrine neoplasms (PEN) - islet cell tumours

20
Q

What is prognosis like for pancreatic neuroendocrine neoplasms?

A

5 year survival rate is ~6%

85% of people that have this present with advanced/unresectable lesions.

21
Q

Who most commonly gets mucinous cystic neoplasms (MCNs)?

A

MCNs are mostly in women, rarely reported in men.

22
Q

Where in the pancreas are MCNs located?

A

In the body/tail

23
Q

What are the morphological features of MCNs?

A

Filled with thick mucin

Lined by mucinous epithelium and the wall contains ovarian stroma

24
Q

Where do intraductal papillary mucinous neoplasms (IPMNs) originate?

A

Originate in the pancreatic duct system

25
Q

What are the morphological features of IPMNs?

A

Filled with thick mucin

Lined by mucinous epithelium with dysplasia + papillary formation

Solid areas/mural nodules

26
Q

What are MCNs and IPMNs both associated with?

A

Both can be associated with invasive carcinoma

27
Q

How are pre-malignant and malignant cells different?

A

The malignant cells are invading the stroma whereas the pre-malignant cells are not invasive.

28
Q

How is MCN/IPMN treated and what is prognosis like?

A

MCN & IPMN are removed with the entire pancreas and duodenum (Whipple procedure) or distal pancreatectomy depending on the site of the pancreas with the problem.

With no invasion there is an excellent prognosis. With invasion it is better than de novo pancreaticductal AdenoCarcinoma (PDAC) so worth the risk.

29
Q

What are the risk factors for pancreatic duct carcinoma?

A

Age > 65

Smoking - cigarette smokers are 2 - 3 times more likely to develop pancreatic cancer

New onset diabetes

Precursor lesions

Family history

Chronic pancreatitis

30
Q

What are the types of precursor lesions for pancreatic duct carcinomas?

A

Pancreatic intraepithelial neoplasia (PanIN) dysplasia of pancreatic ducts; PanIN grade 1 - 3

Cystic neoplasms

31
Q

What are the variants of pancreatic duct carcinoma?

A

Well differentiated

Moderately differentiated

Undifferentiated

32
Q

How do pancreatic duct tumours spread?

A

Directly into adjacent organs

Perineural spaces

Early lymph node involvement

Blood stream fairly early

Prognosis is poor for this reason

33
Q

What are the molecular markers of pancreatic duct cancer?

A

K-ras, G-nas

P53

SMAD4 (unique to pancreatic cancer)

HER2

Other

34
Q

Where are pancreatic neuroendocrine tumours located?

A

Most are in the tail (means only tail needs to be resected if diagnosed correctly to be a neuroendocrine tumour)

All are malignant

Cancers of islet cells.

35
Q

Which neuroendocrine tumours have bad prognosis?

A

The high grade tumours.

36
Q

What is grading of neuroendocrine tumours based on?

A

Proliferation index of the tumours

37
Q

How are neuroendocrine tumours diagnosed pre-operatively?

A

Endoscopic US guided FNA/Brush

Team radiology/clinical, endoscopy and cytology

Rapid on site evaluation (ROSE)

Cytological assessment