Carcinoid, Gastrointestinal, And Stromal Tumor And Lymphoma

Front 1

What is produced by each cell?

G cell

EC cell

D cell

ECL

Where are the secretory granules located in these cells? Where is the hormone discharged?

What are neoplasms arising from or differentiating along this pathway called?

Back 1

G cell = gastrin producing

EC cell = serotonin producing

D cell = somatostatin producing

ECL = histamine producing

Granules located basally between nucleus and BM => discharge into lamina propria => circulation for effect on nearby cells (paracrine)

neuroendocrine tumors (carcinoids)

Front 2

What two things can lead to the hypergastremia compensatory response? What type of hyperplasia and subsequent tumor can this then lead to?

Back 2

Front 3

Less than how many centimeters is considered hyperplasia? Greater than 0.5 centimeters is considered what? Therefore, a lesion less than 0.5 cm is unlikely to do what?

What is shown in the image?

Back 3

Front 4

What type of NET? What cells are involved? What is the formation type?

Back 4

Type 1

Forms a discrete nodule

Front 5

What is an immunostain often positive in neuroendocrine tumors?

Back 5

Front 6

What are 80% of gastric carcinoid tumors associated with? Are they often malignant?

Are the other 20% often malignant? What are they associated with?

Back 6

Front 7

What is the fasting gastrin and pH level in Zollinger-Ellison syndrome?

What is considered a positive secretin stimulation test?

Back 7

Front 8

What are these all methods of treatment for?

Back 8

Zollinger-Ellison syndrome

Front 9

At what age do intestinal neuroendocrine tumors typically present? What are two common sites? Are they often at multiple sites? What is the most frequent symptom? Can obstruction occur? What is the most common neoplasm in the appendix?

Back 9

Front 10

What are these all associated with?

Back 10

Carcinoid syndrome

Front 11

Where is the tumor mass? How do you know? Is these mucosal invasion?

Back 11

Insular pattern, detail of monotonously homogenous cells, mucosal invasion

Front 12

What is happening at the arrows in this ileal neuroendocrine tumor?

Back 12

Invasion of smooth muscle

Front 13

What is the descriptive term for chromatin in neuroendocrine tumors?

Back 13

Front 14

What is the arrow pointing to? Tumor type? Malignant?

Back 14

Submucosal pattern

Front 15

In what part of the colon are neuroendocrine tumors more common? Are they large or small? Is a carcinoid tumor in the rectum common or rare?

Back 15

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Front 17

What type of tumor? Describe the gross appearance.

Back 17

Front 18

What type of cells are GISTs composed of? What protein is often expressed (gain or loss of function)?

These tumors are neoplastic transformations of pluripotent stem cells of GI tract. What results from differentiation of the following:

Smooth muscle

Nerve sheath

Interstitial cell of Cajal

Back 18

GIST are neoplasms composed of spindle, epithelioid or occasionally pleomorphic mesenchymal cells that often express the KIT (CD117) protein.
These tumors are best understood as the neoplastic transformation of pluripotent mesenchymal cells in the G.I. tract. As such they may follow different pathways of differentiation:
Smooth muscle differentiation
Leiomyoma / Leiomyosarcoma
Nerve sheath differentiation
Neurolemmoma, Schwannomas, neurofibromas
Interstitial cell of Cajal
They comprise a majority of tumors previously diagnosed as leiomyomas, leiomyoblastomas, and leiomyosarcomas and many considered as neurofibromas or schwannomas.
Older data about leiomyoma, cellular leiomyoma, epithelioid leiomyoma, leiomyoblastoma, leiomyosarcoma, and epitheliod leiomyosarcoma applies to GISTs
The expression of CD117 is due to a gain-of-function mutation

Front 19

What type of tumor? Is it in the wall? What is the brown area?

Back 19

Front 20

What is the tumor type? Should it be considered to have malignant potential?

What is the best predictor of behavior of this type of tumor?

Back 20

Submucosal spread of the tumor in the stomach.
All GISTs should consider to have some malignant potential.
The best predictor of biological behavior of GISTs is a combination of:
Tumor size
Mitotic activity
Necrosis

Front 21

Tumor type? Invasion of what? Is it malignant?

Back 21

GIST invading muscularis propria and mucosa = malignant

Front 22

What is the cell type of GIST?

Back 22

STROMAL = SPINDLE SHAPED CELLS!!

Front 23

If this is a GIST, what is this most likely a stain for? 80% have what mutation? Additional 8% have what other mutation? One more mutation that occurs in come?

Back 23

Front 24

How many GISTs occur per year? Most common in what two parts of GI tract?

What are they thought to derive from?

Back 24

Front 25

GIST:

What are the three most common symptoms?

What detects them? What can be a false negative due to location?

What technique has high sensitivity and specificity?

What is the treatment?

What drug can be given if metastatic?

Back 25

Front 26

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Front 27

Post-Transplant Lymphoproliferative Disorder

Immunosuppression can allow _____ infected with _____ virus to proliferate in an uncontrolled manner.

Blood test for _____ DNA can detect it.

If the immunosuppression can be safely decreased, sometimes the _____ will come under control, sometimes not.

Back 27

Post-Transplant Lymphoproliferative Disorder

Immunosuppression can allow B cells infected with Epstein-Barr virus to proliferate in an uncontrolled manner.

Blood test for EBV DNA can detect it.

If the immunosuppression can be safely decreased, sometimes the proliferation will come under control, sometimes not.

Front 28

GI Lymphoma:

Common or rare? What ages?

What percent in the stomach? Where is the rest?

Associated with what three things?

About 50% are what type of tumor?

Back 28

Front 29

What can produce nodules visible in the mucosa? What can cause it to regress?

What are the three translocations its associated with?

Back 29

Front 30

Lymphomas in chronically immunosuppressed patients tend to be what type? Positive for what? Consistently over express what?

Patients with refractory gluten disease may develop what type of lymphoma? Location?

Back 30

A variety of lymphomas have been described in the GI tract; they tend to have the same morphology and behavior as their counterparts in other organs. Two forms are peculiar to the GI tract.
Lymphomas in chronically immunosuppressed patients (e.g. transplant recipients, inflammatory bowel disease). They tend to be large-cell lymphomas, EBV positive and consistently overexpress p53. They tend to respond well to treatment.
Patients with refractory gluten disease may develop T-cell lymphomas (Enteropathy-Associated T-cell Lymphoma) most often in the small bowel

Front 31

What type of neoplasm? What is it infiltrating and expanding into?

What is the challenge for the pathologist?

Back 31

Front 32

Most of the GI tract has an abundance of lymphoid tissue (often with germinal centers) in the _____ regions. This lymphoid tissue is a major line of defense against pathogens; being closely associated with the GI mucosa it has been called _____. Tumors believed to be derived from these tissues are often referred by the ugly name of “_____”.
Gastric lymphomas are a perfect example of how reality does not fit in our neat, little, elegant classifications.
Gastric lymphomas are more frequent in patients with chronic _____ infection. These lymphomas are locally invasive and may produce distant _____; therefore, fit our classification as malignant tumors. But, these “malignant tumors” often regress and disappear if _____ is eradicated. Therefore they are not “_____” and do not fit the concept of malignant, but being invasive and metastasizing cannot be called hyperplasias.
Philosophic considerations aside, most of these lymphomas (“maltomas”) are _____-cell tumors, and constitute about _____% of all gastric lymphomas. They often have abundant _____ cell-like features. They tend to be indolent tumors and their degree of malignancy (biologic behavior) is difficult to predict from microscopic features alone.

Back 32

Most of the GI tract has an abundance of lymphoid tissue (often with germinal centers) in the submucosal regions. This lymphoid tissue is a major line of defense against pathogens; being closely associated with the GI mucosa it has been called Mucosa Associated Lymphoid Tissue (MALT). Tumors believed to be derived from these tissues are often referred by the ugly name of “Maltomas”.
Gastric lymphomas are a perfect example of how reality does not fit in our neat, little, elegant classifications.
Gastric lymphomas are more frequent in patients with chronic H. pilori infection. These lymphomas are locally invasive and may produce distant metastasis; therefore, fit our classification as malignant tumors. But, these “malignant tumors” often regress and disappear if H. pilori is eradicated. Therefore they are not “autonomous” and do not fit the concept of malignant, but being invasive and metastasizing cannot be called hyperplasias.
Philosophic considerations aside, most of these lymphomas (“maltomas”) are B-cell tumors, and constitute about 50% of all gastric lymphomas. They often have abundant plasma cell-like features. They tend to be indolent tumors and their degree of malignancy (biologic behavior) is difficult to predict from microscopic features alone.

Front 33

Germinal centers can be a feature of what tumor type? However they can also be present in H. pylori gastritis.

Back 33

Front 34

The feature visible on routine H&E stain most diagnostic of MALToma is lymphocytes surrounding, infiltrating, and overrunning gastric glands. What is this lesion called?

Back 34

Many neoplastic cells also have plasmacytoid features (B-cell tumors)

Front 35

MALTomas are composed of mature __-cells.

Back 35

Front 36

Almost every GI lymphoma patient presents with what? What are some other symptoms?

How do you diagnose?

What is the treatment (MALToma and for others)?

Is the prognosis good for MALToma?

Back 36

Front 37

Can gastric lymphomas infiltrate the wall?

What makes gastric MALTomas unresponsive to eradication of H. pylori? Low grade can transform into high grade with additional acquisition of what?

Back 37

Front 38

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??

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Front 40

What is the diagnosis?

Back 40

Zollinger-Ellison Syndrome

Front 41

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??

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??

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??

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