Pancreas Flashcards

1
Q

Chronic pancreatitis: Associations with pancreatic cancer.

A

Either one can cause the other; either one can mimic the other.

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2
Q

Chronic pancreatitis: Gross-pathology clues to alcoholic origin (2).

A

Pseudocyst.

Intraductal calculi.

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3
Q

Chronic pancreatitis: Possible confounding effects on islets of Langerhans (2).

A

Aggregations of islets may mimic neoplasm.

Islets may abut nerves.

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4
Q

Chronic pancreatitis: Helpful signs of benignity (2).

A

Preservation of lobular architecture.

No glands adjacent to muscular blood vessels.

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5
Q

Genes that predispose to chronic pancreatitis:

A. Names (2).
B. Other implications (2).

A

A. PRSS1, SPINK1.

B. Acute pancreatitis in childhood; pancreatic cancer.

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8
Q

Autoimmune pancreatitis: Histology (3).

A

Duct-centered lymphoplasmacytic infiltrate.

Interstitial fibrosis.

Venulitis.

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9
Q

Autoimmune pancreatitis: Treatment.

A

May respond to steroids.

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10
Q

Pancreatic pseudocyst: Outcomes (3).

A

Spontaneous resolution.

Infection.

Erosion into adjacent organs.

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11
Q

Pancreatic pseudocyst: Cytology (2).

A

Necrotic and hemorrhagic debris.

Hemosiderin-laden macrophages.

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12
Q

Pancreatic pseudocyst: Laboratory findings in cyst fluid.

A

Elevated amylase and CA19-9.

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13
Q

Lymphoepithelial cyst: Epidemiology.

A

M : F = 4 : 1.

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14
Q

Lymphoepithelial cyst: Gross pathology.

A

Thin wall; keratinous contents.

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15
Q

Lymphoepithelial cyst: Histopathology (2).

A

Lining composed of keratinized squamous epithelium.

Underlying lymphoid tissue.

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16
Q

Lymphoepithelial cyst: Main differential diagnosis.

A

Mature cystic teratoma also contains adnexal structures and sometimes cartilage.

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17
Q

Lymphoepithelial cyst: Clinical associations.

A

None demonstrated.

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18
Q

Serous cystic neoplasm: Epidemiology.

A

More common in women.

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19
Q

Serous cystic neoplasm: Association.

A

Von Hippel-Lindau syndrome.

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20
Q

Serous cystic neoplasm: Variants in gross pathology (4).

A

Microcystic.

Macrocystic.

Solid.

Association with well-differentiation neuroendocrine tumor.

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21
Q

Serous cystic neoplasm: Histopathology (3).

A

Lined by bland cells analogous to normal pancreatic serous cells.

Solid variant consists of the same type of cell.

Central scar (may be seen radiologically).

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22
Q

Serous cystic neoplasm: Special stain.

A

Positive for PAS, sensitive to diastase (contain glycogen).

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23
Q

Serous cystic neoplasm: Mutation.

A

Some tumors have a mutation involving VHL on chromosome 3p.

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24
Q

Serous cystic neoplasm: How to distinguish benign from malignant.

A

Serous cystadenocarcinoma: Extremely rare; diagnosed by its spread to other organs.

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25
Q

Mucinous cystic neoplasms: Epidemiology.

A

F : M = 20 : 1.

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26
Q

Mucinous cystic neoplasms: How many are malignant?

A

About one third.

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27
Q

Mucinous cystic neoplasms: Anatomic location (2).

A

Body or tail of pancreas.

No connection with the ductal system.

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28
Q

Mucinous cystic neoplasms: Histopathologic classification (4).

A

Noninvasive:
− Low-grade dysplasia.
− Intermediate-grade dysplasia.
− High-grade dysplasia.

Invasive: Cystadenocarcinoma.

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29
Q

Mucinous cystic neoplasm:

A. Stroma.
B. Cytokeratins (2).

A

A. Ovarian-like.

B. CK7 in most, CK20 in about ⅔.

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30
Q

Mucinous cystic neoplasms: Important gene.

A

Smad4/DPC4:
− Unmutated in most noninvasive tumors.
− Lost in about half of invasive tumors.

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31
Q

Mucinous cystic neoplasm: Other possibly mutated genes (3).

A

RNF43, KRAS2, TP53.

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32
Q

Intraductal papillary mucinous neoplasm: Epidemiology.

A

Slightly more common in men.

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33
Q

Intraductal papillary mucinous neoplasm: How many are invasive?

A

About one third.

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34
Q

Autoimmune pancreatitis: Helpful stains (2).

A

IHC for IgG4.

Elastin stain or Movat’s stain to demonstrate venulitis.

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35
Q

Intraductal papillary mucinous neoplasm: Histopathologic classification.

A

Same as that of mucinous cystic neoplasm.

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36
Q

Intraductal papillary mucinous neoplasm: Stroma.

A

Paucicellular.

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37
Q

Intraductal papillary mucinous neoplasm: Invasive types (2).

A

Tubular (ductal).

Colloid (mucinous non-cystic): At least 80% of the tumor must have mucinous histology.

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38
Q

Intraductal papillary mucinous neoplasm: Immunohistochemstry.

A

Intestinal type: MUC2.

Pancreaticobiliary type: MUC1.

39
Q

Intraductal papillary mucinous neoplasm: Possibly mutated genes (5).

A

Smad4/DPC4.

RNF43, KRAS2, TP53, GNAS.

40
Q

Intraductal papillary mucinous neoplasm: Favorable prognostic factors (2).

A

Overall: Absence of invasion.

Invasive tumors: Colloid histology.

41
Q

Pancreatic intraepithelial neoplasia: Size.

A

Usually less than 0.5 cm.

42
Q

Pancreatic intraepithelial neoplasia: Histopathology (2).

A

Occurs within smaller pancreatic ducts.

If there are papillae, these are shorter than those of a mucinous papillary neoplasm.

43
Q

Pancreatic intraepithelial neoplasia: Associated histopathologic finding.

A

Lobular acinar units may show atrophy and scarring.

44
Q

Pancreatic intraepithelial neoplasia: Immunohistochemistry.

A

Positive for MUC1.

45
Q

Pancreatic intraepithelial neoplasia: Mutated genes.

A

PanIN-1: Activation of KRAS2.

PanIN-2: Inactivation of p16/CDKN2A.

PanIN-3: Loss of Smad4/DPC4 and of TP53.

46
Q

Pancreatic intraepithelial neoplasia: Surgical significance.

A

The presence of PanIN at a margin usually does not matter.

47
Q

Invasive ductal adenocarcinoma: Gross-pathology clue to the diagnosis.

A

“Double-duct” sign: Dilatation of main pancreatic duct and bile duct due to obstruction.

48
Q

Histology of invasive ductal adenocarcinoma: Architectural clues to the diagnosis (5).

A

Loss of lobular architecture.

Glands immediately adjacent to muscular blood vessels.

Incomplete lumens.

Necrotic lumens.

Perineural or vascular invasion.

49
Q

Histology of invasive ductal adenocarcinoma: Cytologic clue to the diagnosis.

A

Within a single gland, there is more than a fourfold variation in size of the nuclei.

50
Q

Adenosquamous carcinoma: Definition.

A

At least 30% of the tumor is squamous.

51
Q

Colloid carcinoma:

A. Definition.
B. Association.

A

A. At least 80% of the tumor has colloid histology.

B. Intraductal papillary mucinous neoplasm (almost always).

52
Q

Medullary carcinoma: Histopathology (3).

A

Syncytial growth.

Pushing borders.

Lymphocytic infiltrate.

53
Q

Medullary carcinoma: Mutation.

A

Microsatellite instability.

54
Q

Signet-ring carcinoma:

A. Stain.
B. Differential diagnosis.

A

A. Loss of E-cadherin.

B. Signet-ring carcinomas of stomach and breast must be excluded.

55
Q

Undifferentiated carcinoma, NOS: Histologic variants (3).

A

Anaplastic, sarcomatoid, carcinosarcoma.

56
Q

Undifferentiated carcinoma with osteoclast-like giant cells: Histopathology.

A

Osteoclast-like giant cells accompanied by pleomorphic mononuclear cells.

57
Q

Pancreatic cancer: Associated cancer-syndrome genes (4).

A

BRCA2 and PALB2.

STK11.

ATM.

58
Q

Intraductal papillary mucinous neoplasm: Anatomic location (2).

A

Most tumors arise in the head of the pancreas.

Connection with the ductal system.

60
Q

Autoimmune pancreatitis: Laboratory finding.

A

Elevated serum IgG4.

61
Q

Autoimmune pancreatitis: Associated autoimmune diseases (5).

A

Chronic sclerosis sialadenitis.

PSC, inflammatory bowel disease.

Retroperitoneal fibrosis.

Riedel’s thyroiditis.

62
Q

Acinar-cell carcinoma: Epidemiology.

A

More common in males.

63
Q

Clinical triad of acinar-cell carcinoma:

A. Frequency.
B. Components.
C. Cause.

A

A. Occurs at presentation in 15% of cases.

B. Metastatic fat necrosis, arthralgias, peripheral eosinophilia.

C. Lipase.

64
Q

Acinar-cell carcinoma: Anatomic location.

A

Head or tail or pancreas.

65
Q

Acinar-cell carcinoma: Histologic patterns (2).

A

Acinar or solid.

66
Q

Acinar-cell carcinoma: Cytology.

A

Granular cytoplasm; basally oriented nucleus with a single large nucleolus.

67
Q

Acinar-cell carcinoma: Variant.

A

Partial neuroendocrine differentiation.

68
Q

Acinar-cell carcinoma: Mutation.

A

KRAS2 is rarely implicated.

69
Q

Pancreatoblastoma: Associated syndromes (2).

A

Beckwith-Wiedemann.

FAP.

70
Q

Pancreatoblastoma: Laboratory abnormality.

A

Elevated AFP in some cases.

71
Q

Pancreatoblastoma: Anatomic location.

A

Head or tail of pancreas.

72
Q

Pancreatoblastoma: Components (2,3).

A

Required: Acinar and squamoid.

Optional: Neuroendocrine, ductal, mesenchymal.

73
Q

Pancreatoblastoma: Immunohistochemistry (4).

A

Acinar cells: Trypsin, antichymotrypsin, lipase.

Squamoid nests: Nonspecific labeling with biotin.

74
Q

Pancreatoblastoma: Mutation.

A

Loss of heterozygosity at chromosome 11p near WT-2.

75
Q

Well-differentiated pancreatic neuroendocrine tumors: Possibly associated gene and its location.

A

MEN1 on chromosome 11q13.

76
Q

Well-differentiated pancreatic neuroendocrine tumors: Definition of microadenoma.

A

Less than 0.5 cm.

77
Q

Well-differentiated pancreatic neuroendocrine tumors: Important histologic prognostic factor.

A

Mitotic rate.

78
Q

Glucagonoma: Presentation (3).

A

Diabetes.

Necrotizing migratory erythema.

Stomatitis.

79
Q

Gastrinoma: Presentation.

A

Duodenal ulcer.

80
Q

VIPoma: Presentation (3).

A

Watery diarrhea.

Achlorhydria.

Hypokalemia.

81
Q

Well-differentiated pancreatic neuroendocrine tumors: Histological clues to type of hormone.

A

Presence of amyloid may indicate an insulinoma.

Presence of psammoma bodies may indicate a somatostatinoma.

82
Q

Well-differentiated pancreatic neuroendocrine tumors: Special stains (2).

A

Grimelius.

Fontana-Masson.

83
Q

Well-differentiated pancreatic neuroendocrine tumors: Cytokeratin.

A

Usually negative for CK7, unlike pancreatic ductal adenocarcinoma.

84
Q

Well-differentiated pancreatic neuroendocrine tumors: Relation of size to malignancy.

A

All tumors larger than 0.5 cm should be considered malignant.

85
Q

High-grade neuroendocrine carcinoma:

A. Synonym.
B. Possible presentation.

A

A. Small-cell carcinoma.

B. Paraneoplastic syndrome such as Cushing’s syndrome.

86
Q

High-grade neuroendocrine carcinoma: Mitotic rate.

A

More than 20 per 10 high-power fields (by definition).

87
Q

High-grade neuroendocrine carcinoma: Mutated genes (2).

A

RB, TP53.

88
Q

Solid-pseudopapillary neoplasm: Anatomic location.

A

Can arise from any region of the pancreas.

89
Q

Solid-pseudopapillary neoplasm: Cytology.

A

Nuclear grooves.

90
Q

Solid-pseudopapillary neoplasm: Extracellular features (2).

A

Hyaline globules.

Cholesterol clefts.

91
Q

Solid-pseudopapillary neoplasm: Immunohistochemistry (2).

A

Positive for CD10, (nuclear) β-catenin.

92
Q

Solid-pseudopapillary neoplasm: Mutation.

A

β-Catenin.