Cancer: Other GI Tumors Flashcards

0
Q

Where are leiomyomas typically found in the esophagus?

A

The distal 1/3.

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1
Q

What is the most common mesenchymal tumor of the esophagus?

A

Leiomyoma.

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2
Q

How do leiomyomas typically present?

A

Dysphagia.

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3
Q

Where in GI tract are you unlikely to find leiomyomas?

A

The stomach and small bowel. They are usually in the esophagus and colon.

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4
Q

What markers are expressed by leiomyomas?

A

Smooth muscle actin and desmin.

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5
Q

What is the most common mesenchymal tumor of the GI tract?

A

A gastrointestinal stromal tumor (GIST).

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6
Q

What markers are typically expressed by GISTs?

A

c-Kit, a tyrosine kinase.

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7
Q

What is the cell of origin for GISTs?

A

The interstitial cells of Cajal, which are pacemakers for GI motility.

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8
Q

In which layer of the GI tract can the interstitial cells of Cajal typically be found?

A

In the muscularis propia, near the myenteric plexuses. (They can similarly be found in the muscularis mucosa near submucosal plexuses.)

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9
Q

Why is it thought the GISTs derive from interstitial cells of Cajal?

A

They both express c-Kit.

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10
Q

What characteristics do GISTs that are c-Kit negative usually have?

A

(1) epithelioid morphology

(2) PDGFRA mutations

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11
Q

How are GISTs treated?

A

(1) surgical resection
(2) imatinib, a tyrosine kinase inhibitor (especially effective for c-Kit positives)
(3) sunitinib malate, for the imatinib-resistant

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12
Q

Where in the GI tract are GISTs most common?

A

The stomach, followed by the small bowel.

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13
Q

What is an important initial indicator of malignancy for GISTs?

A

Location. 20% of GISTs in the stomach are malignant, compared to half in the small bowel and a majority in the colon and esophagus (though they are rare in these latter locations).

Size and mitotic count are also important.

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14
Q

What genetic disorders are associated with GISTs?

A

(1) neurofibromatosis type 1

(2) Carney’s triad

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15
Q

What is Carney’s triad?

A

A generic disorder in young females characterized by:

(1) GISTs
(2) pulmonary chondromas
(3) paragangliomas

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16
Q

What are the 3 general categories of neuroendocrine tumors?

A

(1) well-differentiated NET
(2) poorly-differentiated neuroendocrine carcinoma, small cell type
(3) poorly-differentiated neuroendocrine carcinoma, large cell type

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17
Q

Where in the wall of the GI tract can neuroendocrine cells typically be found?

A

They are concentrated at the base of crypts and glands, thus NETs typically develop at the junction of the mucosa and submucosa.

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18
Q

How do NETs appear, grossly?

A

Yellow-tinged sessile nodules, usually with an intact or slightly eroded surface.

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19
Q

What is a characteristic cytological feature of NETs?

A

Salt and pepper chromatin.

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20
Q

What are markers of NETs on immunohistochemistry?

A

(1) synaptophysin
(2) chromogranin
(3) CD56

21
Q

Which GI tract NETs are more aggressive: functional or non-functional?

A

Functional.

22
Q

What causes carcinoid syndrome?

A

A serotonin-secreting NET.

23
Q

What is the clinical presentation of carcinoid syndrome?

A

(1) flushing
(2) diarrhea and abdominal cramps
(3) right-sided heart disease (fibrosis of tricuspid valve)

24
Q

Why is carcinoid syndrome arising from a GI NET usually a sign of malignancy?

A

Serotonin released from the GI tract is typically inactivated by first-pass metabolism by the liver. Thus if the patient presents with symptoms, the tumor has likely metastasized.

25
Q

What genetic disorder is associated with gastrinomas?

A

MEN 1.

26
Q

Why are gastrinomas more often symptomatic?

A

Gastrin is metabolized by the kidney, thus it is subject to first pass metabolism in the liver.

27
Q

What are the gross changes in ZES?

A

(1) thickened rugal folds in the stomach

(2) peptic ulcers

28
Q

What is the most common site for extranodal lymphomas?

A

The GI tract, with stomach, small bowel and colon in decreasing order.

29
Q

What are predisposing factors for GI lymphomas?

A

(1) infection
(2) celiac disease
(3) IBD
(4) immunodeficiency

30
Q

What path do B cells take in their maturation once leaving the bone marrow?

A

In the lymph node:

(1) mantle zone
(2) germinal center
(3) marginal zone or paracortex

31
Q

What is the cell of origin in MALT lymphoma?

A

Marginal zone B cells.

32
Q

What is the cell of origin in follicular lymphoma?

A

Germinal center B cells.

33
Q

Where are MALT lymphomas most common?

A

The stomach.

34
Q

What condition are MALT lymphomas most associated with?

A

H. pylori infection.

35
Q

What genetic anomaly is most associated with MALT lymphoma?

A

A t(11:18) translocation, particularly in cases that do to respond to treatment of H. pylori infection.

36
Q

What are complications of more advanced MALT lymphomas?

A

(1) transformation to diffuse large B cell lymphoma

(2) metastasis

37
Q

How are MALT lymphomas treated?

A

(1) treat H. pylori infection

(2) surgery, radiation, chemo

38
Q

What is a concerning histological finding in MALT lymphoma?

A

Lymphoepithelial lesion.

39
Q

Where is the most common location for follicular lymphoma in the GI tract?

A

The small bowel.

40
Q

J: This refers to a presentation of follicular lymphoma in which there are multiple polyps.

A

What is lymphomatous polyposis?

41
Q

What is in the ddx for lymphomatous polyposis?

A

(1) reactive lymphoid hyperplasia (HIV)
(2) mantle zone lymphoma
(3) follicular lymphoma

42
Q

What cytogenetic anomaly is associated to follicular lymphoma?

A

A t(14:18) translocation resulting in a rearrangement of BCL2.

43
Q

In what location in the GI tract is mantle zone lymphoma most common?

A

The colon.

44
Q

What cytogenetic anomaly is most associated with mantle zone lymphoma?

A

A t(11:14) translocation resulting in an over expression of cyclin D1.

45
Q

J: This cancer is characterized by a starry sky pattern with tingible body macrophages on histology.

A

What is Burkitt lymphoma?

46
Q

What is the most common cytogenetic anomaly in Burkitt lymphoma?

A

A t(8:14) translocation involving c-myc.

47
Q

What types of Burkitt lymphoma typically present in the abdomen?

A

(1) non-endemic

(2) immunodeficiency-associated

48
Q

What lymphoma has a high association with celiac disease?

A

Enteropathy-type T cell lymphoma.

49
Q

How can you differentiate celiac disease from enteropathy-type T cell lymphoma?

A

(1) cellular atypia
(2) aberrant T cell antigen expression
(3) T cell clonality