Small Bowel Tumors Flashcards

1
Q

What are the typical presenting symptoms of small bowel tumors?

A

The symptoms are vague and highly variable.
Many patients may have a known mass discovered during a workup for GI bleeding or found incidentally on imaging.

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2
Q

How do some patients with small bowel tumors present due to obstruction?

A

Some patients present with colicky pain from partial or complete obstruction and are found to have a small bowel tumor during surgery.

can be intraluminal or circumferential and responsible for the obstruction, or surrounded by fibrosis causing stricture and adhesion to the mesentery or retroperitoneum.

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3
Q

What are the common presentations of GI stromal tumors (GIST)?

A

GISTs are frequently asymptomatic but can present with bleeding or obstruction

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4
Q

What paraneoplastic symptoms are associated with malignant neoplasms like adenocarcinoma and lymphoma?

A

such as weight loss and vague abdominal pain can be concerning for malignant neoplasms, particularly in the context of a known small bowel mass

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5
Q

How can neuroendocrine tumors (NETs) present symptomatically?

A

NETs producing vasoactive amines can present with symptoms such as epigastric pain, ulcerations, flushing, sweating, and diarrhea

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6
Q

How are most neuroendocrine tumors (NETs) detected?

A

Most NETs are asymptomatic and found incidentally during imaging for other conditions

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7
Q

Which genetic cancer syndromes predispose individuals to small bowel malignancy?

A

Familial adenomatous polyposis (FAP)
hereditary nonpolyposis colon cancer (HNPCC)
and Peutz-Jeghers syndrome (PJS)

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8
Q

What chronic inflammatory conditions increase the risk of small bowel malignancy?

A

Crohn’s disease and celiac sprue. The risk increases with the severity and duration of the disease.

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9
Q

How does cystic fibrosis affect the risk of small bowel malignancy?

A

Patients with cystic fibrosis have a 19-times higher incidence than the general population and an additional 2- to 5-fold higher risk after lung transplantation

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10
Q

Which lifestyle factors are associated with an increased risk of small bowel tumors?

A

Obesity
tobacco use
and high dietary intake of red meat
smoked foods, alcohol
and refined sugar are associated with a higher risk

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11
Q

What laboratory tests are included in the standard workup for small bowel tumors?

A

A complete blood count (CBC) to check for anemia or elevated white blood cell count, particularly if signs of perforation are present.

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12
Q

What do elevated liver enzymes or amylase suggest in the context of small bowel tumors?

A

They may suggest the presence of a duodenal mass or obstruction.

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13
Q

Is carcinoembryonic antigen (CEA) a reliable marker for small bowel adenocarcinoma?

A

CEA is often elevated in small bowel adenocarcinoma but is neither sensitive nor specific

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14
Q

Should serum 5-HIAA and chromogranin A be routinely tested in all cases of suspected small bowel tumors?

A

No, these tests should only be done if there is a strong clinical suspicion of a neuroendocrine tumor (NET) based on symptoms or imaging.

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15
Q

What imaging modality is commonly used upon presentation with obstructive symptoms?

A

A computed tomography (CT) scan with IV contrast is commonly used to visualize the location of obstruction and potentially the mass itself.

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16
Q

What characteristic appearance can some constricting small bowel masses demonstrate on CT?

A

An “apple core” appearance, indicating circumferential constriction of the lumen

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17
Q

Which type of small bowel masses are better visualized with oral contrast?

A

Polypoid masses that project intraluminally.

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18
Q

Why is careful evaluation of the bowel contour important in CT imaging?

A

To identify submucosal lesions and assess for associated findings such as enlarged nodes or a mesenteric-based mass

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19
Q

What might be the only sign of a nearby small tumor on imaging?

A

Enlarged mesenteric nodes.

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20
Q

Can a negative CT scan rule out a small bowel tumor?

A

No, a negative CT should not rule out a small bowel tumor, as CT is not highly sensitive for this condition

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21
Q

What is the detection rate of CT for abnormalities in patients with small bowel tumors?

A

CT can detect abnormalities in up to 80% of patients with a small bowel tumor.

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22
Q

What is the additional value of CT scans in the evaluation of small bowel tumors?

A

CT scans are valuable for staging nodes and identifying metastatic lesions.

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23
Q

Which specialized imaging modalities are helpful for NET localization and assessing multifocal/metastatic disease?

A

Octreotide scans or PET/ DOTATATE scans.

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24
Q

Are octreotide or PET/DOTATATE scans commonly used before surgical intervention?

A

Yes, in some institutions, these scans are routine before operative intervention

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25
Q

What portion of the small bowel can esophagogastroduodenoscopy (EGD) evaluate?

A

EGD can detect tumors up to the third portion of the duodenum and is an excellent initial tool for evaluating GI bleeding or masses.

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26
Q

What is the role of endoscopic ultrasound in evaluating small bowel tumors?

A

Endoscopic ultrasound is useful as an adjunct to standard EGD but is limited to the evaluation of duodenal lesions

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27
Q

What techniques can be used for deeper small bowel evaluation beyond the duodenum?

A

Balloon-assisted deep enteroscopy or push endoscopy techniques can be used, typically by expert gastroenterologists.

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28
Q

What is the advantage of video capsule endoscopy (VCE) in evaluating small bowel tumors?

A

VCE can evaluate the mucosal surface of the entire small bowel and captures an average of 30,000 images during an examination

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29
Q

What is a limitation of video capsule endoscopy (VCE)?

A

VCE does not allow for tissue diagnosis and is contraindicated in patients with obstructive symptoms.

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30
Q

What are the three types of adenomas in the small bowel?

A

Villous, tubular, and Brunner’s gland associated.

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31
Q

Do adenomas in the small bowel have malignant potential?

A

Yes, some adenomas have malignant potential similar to colorectal cancer.

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32
Q

What is the conventional pathway followed for managing small bowel adenomas?

A

The colorectal cancer pathway, with endoscopic resection if possible

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33
Q

What increases the malignant potential of adenomas in the small bowel?

A

Association with Familial Adenomatous Polyposis (FAP).

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34
Q

What classification system is used to guide the management of small bowel polyps?

A

The Spigelman classification.

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35
Q

What is Spigelman Classification

A

see

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36
Q

When is surgical resection indicated for small bowel adenomas?

A

For large adenomas not amenable to endoscopic resection, especially those with villous features on biopsy.

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37
Q

What screening should be performed for patients with small bowel adenomas?

A

Colonoscopy to check for synchronous colorectal lesions.

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38
Q

What are leiomyomas and where are they typically located?

A

Leiomyomas are small tumors found in the submucosa of the small bowel.

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39
Q

Why might leiomyomas require resection?

A

They may cause obstruction, but their presence alone is not an indication for surgery.

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40
Q

What is a challenge in diagnosing leiomyomas?

A

Differentiating them from leiomyosarcomas, often leading to surgical resection to rule out malignancy.

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41
Q

How are lipomas in the small bowel typically diagnosed?

A

Through CT imaging due to their characteristic fatty density.

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42
Q

When should hamartomas associated with Peutz-Jeghers Syndrome (PJS) be resected?

A

Only if they cause bleeding or obstructive symptoms.

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43
Q

What are fibromyxomas and ganglioneuromas, and do they require resection?

A

These are types of benign tumors that do not require resection, though biopsy may be needed for diagnosis.

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44
Q

What imaging techniques are useful for identifying hemangiomas in the small bowel?

A

CT with IV contrast or MR enterography.

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45
Q

Why do hemangiomas in the small bowel typically not require biopsy?

A

Because they are easily differentiated on imaging studies.

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46
Q

What type of cells are small bowel NETs derived from?

A

Enterochromaffin cells, also known as Kulchitsky cells

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47
Q

Where are NETs found in the small bowel?

A

Throughout the crypts of Lieberkühn.

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48
Q

What is the histological hallmark of NETs?

A

Cytoplasmic core granules containing chromogranin A, synaptophysin, and neuron-specific enolase.

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49
Q

What percentage of malignant small bowel tumors do NETs account for?

A

More than 20%.

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50
Q

What proportion of individuals present with multifocal tumors > 2 cm?

A

40%.

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51
Q

What percentage of patients with small bowel NETs have invasion into the muscularis propria and regional lymph nodes at presentation?

A

70%.

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52
Q

What is the rate of liver metastasis in patients at the presentation of small bowel NETs?

A

50%.

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53
Q

What is the five-year cancer-specific survival for localized small bowel NETs?

A

95%.

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54
Q

How does five-year survival change for small bowel NETs with nodal and distant disease?

A

84% for nodal disease and 51% for distant disease.

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55
Q

What is the overall survival rate for individuals with distant metastatic small bowel NETs?

A

32%.

56
Q

How are well-differentiated NETs graded?

A

Based on the Ki-67 index (%) and mitotic index per 10 high-powered fields (HPF)

57
Q

Where do most gastrointestinal NETs occur?

A

35% to 45% in the small intestine, with most found within 60 cm of the ileocecal valve.

58
Q

How do carcinoid lesions appear on a CT scan compared to adenocarcinomas?

A

Carcinoid lesions are hyperenhancing, while adenocarcinomas are only moderately enhancing

59
Q

What can carcinoid tumors cause in relation to the mesentery?

A

Foreshortening of the mesentery secondary to a desmoplastic reaction.

60
Q

What is a common finding on imaging when the primary small bowel NET is not visualized?

A

Bulky mesenteric adenopathy.

61
Q

Gastrointestinal Neuroendocrine Neoplasm Classification System

A

see

62
Q

What surgical procedure is recommended for regional spread of carcinoid tumors?

A

En bloc resection of the mass and mesentery, including an extensive lymphadenectomy

63
Q

Which type of exploration is typically preferred for carcinoid tumor resection?

A

Open exploration.

64
Q

What should be inspected during surgery for carcinoid tumors?

A

Multicentric disease and liver metastases.

65
Q

Why is resecting the primary lesion important even if metastases are present?

A

For local control and to improve longevity.

66
Q

What percentage of disease burden should be resected for optimal symptomatic improvement?

A

90%.

67
Q

What is sometimes implemented for patients with liver metastasis from carcinoid tumors?

A

A staged approach to resection.

68
Q

What is a carcinoid crisis and what triggers it?

A

A hemodynamically significant surge in vasoactive peptides triggered by operative manipulation of carcinoid tumors.

69
Q

How is a carcinoid crisis typically managed?

A

With high-dose somatostatin analogues and hemodynamic stabilization.

70
Q

What is the evidence status on using prophylactic somatostatin analogues for carcinoid crisis prevention?

A

The evidence is mixed, and practices vary among surgeons and institutions

71
Q

What surgical option exists for small duodenal carcinoid tumors?

A

Endoscopic resection.

72
Q

How can anterior wall duodenal carcinoid lesions be excised?

A

With laparoscopic or open surgery.

73
Q

What approach is recommended for select posterior duodenal lesions?

A

Local excision through an anterior duodenotomy to expose the posterior wall.

74
Q

What procedure is recommended when both anterior and posterior duodenotomies are performed?

A

Gastrojejunostomy to prevent obstruction from duodenal narrowing.

75
Q

What tool can be used for guidance during resection of tumors in the second portion of the duodenum?

A

A Fogarty balloon introduced through the gallbladder or cystic duct and passed proximally through the ampulla.

76
Q

What procedure is performed after the use of a Fogarty balloon in duodenal tumor surgery?

A

Cholecystectomy, which avoids opening the common bile duct.

77
Q

What surgical procedure may be necessary if a tumor is too close to the ampulla to be safely resected?

A

Pancreaticoduodenectomy.

78
Q

How much small bowel is typically removed during a small bowel carcinoid resection?

A

1 to 2 feet of small bowel with wide lymphatic mesenteric clearance.

79
Q

Why might more bowel resection be required than originally anticipated in small bowel carcinoid cases

A

Mesenteric involvement is often underestimated and can be misleading.

80
Q

What must patients be counseled about preoperatively when extensive resection is considered?

A

The risks associated with extensive bowel resection

81
Q

What is a necessary consideration in cases of extensive small bowel mesenteric involvement?

A

Leaving mesenteric disease behind may be required to avoid short-gut syndrome.

82
Q

What is the minimal length of small bowel that should remain to avoid significant complications post-resection?

A

At least 120 cm.

83
Q

When is it recommended to avoid surgical intervention for extensive but relatively asymptomatic mesenteric disease

A

When systemic medical treatment with a somatostatin analogue is more appropriate

84
Q

Which conditions predispose patients to small bowel NETs?

A

Multiple endocrine neoplasia type I (MEN1) and neurofibromatosis

85
Q

Where do small bowel NETs associated with MEN1 and neurofibromatosis primarily occur?

A

In the foregut, presenting as gastrinomas or somatostatinomas.

86
Q

What type of cancer accounts for more than a third of small bowel cancers?

A

Adenocarcinoma

87
Q

Where do small bowel adenocarcinomas most commonly occur?

A

Primarily in the duodenum.

88
Q

Why are small bowel adenocarcinomas often diagnosed at advanced stages?

A

They are relatively asymptomatic until they cause obstruction or clinically evident bleeding

89
Q

What proportion of small bowel adenocarcinoma cases present with nodal spread

A

One third.

90
Q

What percentage of small bowel adenocarcinoma cases present with distant metastases?

A

A quarter.

91
Q

What is the 5-year survival rate for most cases of small bowel adenocarcinoma?

A

20% to 40%.

92
Q

What is the mainstay of therapy for small bowel adenocarcinoma?

A

Surgical resection.

93
Q

What surgical procedure should be performed for small bowel adenocarcinoma at the terminal ileum?

A

Ileocolectomy

94
Q

What is the recommended surgical approach for duodenal adenocarcinomas located at the ampulla?

A

Pancreaticoduodenectomy.

95
Q

What is a reasonable surgical option for unresectable primary small bowel adenocarcinoma?

A

Palliative surgical bypass of the obstructive lesion

96
Q

Is palliative radiation generally beneficial for unresectable small bowel adenocarcinoma?

A

No, it is usually not beneficial.

97
Q

What percentage of GISTs are found in the stomach?

A

More than 50%.

98
Q

What proportion of GISTs occur in the jejunum?

A

25%.

99
Q

From which cells are GISTs derived?

A

Interstitial cells of Cajal.

100
Q

What percentage of small bowel tumors do GISTs comprise?

A

Approximately 10%.

101
Q

How often are GISTs malignant at presentation, especially in the small bowel?

A

About 20% to 30%.

102
Q

How do GISTs typically appear on CT scans?

A

As smooth, well-defined masses with exophytic growth and internal heterogeneity.

103
Q

What distinctive characteristics might GISTs have on imaging?

A

Areas of central hemorrhage or necrosis.

104
Q

Do GISTs frequently metastasize to lymph nodes?

A

No, they rarely metastasize to nodes or spread outside the abdominal cavity.

105
Q

What organ is commonly affected by metastasis from GISTs?

A

The liver, presenting with multiple serosal-based nodules.

106
Q

What is the significance of c-KIT immunostaining in diagnosing GISTs?

A

It helps differentiate GISTs from other smooth muscle sarcomas.

107
Q

What percentage of GISTs have activating mutations of the KIT oncogene

A

Over 80%.

108
Q

Is a CT-guided biopsy usually necessary for diagnosing GISTs?

A

No, the radiographic appearance is distinctive, and resection is indicated

109
Q

What surgical approach is often ideal for resecting GISTs?

A

A laparoscopic approach.

110
Q

How much margin is recommended during a segmental enterectomy for GIST?

A

Only 2 cm.

111
Q

What preoperative treatment can help shrink bulky GISTs or those involving adjacent organs?

A

Imatinib.

112
Q

What is the recurrence rate of GISTs within 5 years?

A

More than 50%.

113
Q

How long should high-risk GIST patients be treated with imatinib postoperatively?

A

A minimum of 12 to 24 months.

114
Q

What are high-risk features for GIST recurrence?

A

Tumor size > 2 cm
high mitotic index
poorly differentiated cell type
presence of metastasis, and positive margin.

115
Q

What type of sarcoma is most commonly found in the small bowel?

A

Leiomyosarcoma.

116
Q

In which part of the small bowel do sarcomas most often occur?

A

The ileum.

117
Q

What is the approximate 5-year survival rate for small bowel sarcomas?

A

Approximately 50%.

118
Q

What is the recommended treatment for resectable primary small bowel sarcoma?

A

Radical surgical excision.

119
Q

What should be considered if a small bowel sarcoma is deemed unresectable?

A

Surgical bypass.

120
Q

How does the role of palliative radiation in managing small bowel sarcoma compare to other small bowel tumors?

A

Palliative radiation plays a greater role in sarcoma management.

121
Q

What is the most frequently encountered extranodal site for lymphoma?

A

The small bowel.

122
Q

What type of lymphoma most commonly involves the small bowel?

A

Non-Hodgkin-type lymphoma.

123
Q

Where in the small bowel is lymphoma most commonly found?

A

The ileum, which is the most lymphoid-rich region.

124
Q

Which patients have an increased risk of developing small bowel lymphoma?

A

Patients with Celiac disease (20-fold higher risk) and those with chronic immunosuppression, including transplant recipients and individuals with HIV.

125
Q

How does small bowel lymphoma typically appear on a CT scan with IV contrast?

A

As a well-circumscribed, homogeneous mass.

126
Q

What is essential for diagnosing small bowel lymphoma?

A

A tissue diagnosis with enough tissue for cytopathology and flow cytometry.

127
Q

What is the primary treatment for most small bowel lymphomas?

A

Multidrug chemotherapy.

128
Q

What is the 5-year survival rate for small bowel lymphoma?

A

Approximately 50%.

129
Q

Which groups have a poorer prognosis for small bowel lymphoma?

A

Males and the elderly.

130
Q

When might surgery be necessary for small bowel lymphoma?

A

For obstruction or if the diagnosis is made postoperatively after surgery for small bowel obstruction.

131
Q

What are appropriate diagnostic tools for small bowel lymphoma in patients without signs of SBO?

A

CT-guided or endoscopic biopsy.

132
Q

What is the main role of biopsy in diagnosing small bowel tumors?

A

To distinguish lymphoma, as most other small bowel tumors require resection as the primary management

133
Q

How does secondary involvement of the small bowel from metastatic lesions typically present?

A

As multifocal advanced cancer, not as an isolated small bowel lesion.

134
Q

Which cancers can spread to the small bowel?

A

Lung, melanoma, breast, colon, and cervical cancers

135
Q

How can sarcomas and adenocarcinomas affect the small bowel?

A

Through direct erosion or carcinomatosis of the overlying peritoneum

136
Q

What surgical intervention might be needed for the small bowel if impacted by secondary metastatic lesions?

A

Resection or bypass, depending on the type and stage of the primary tumor.