Gastric Flashcards by Benjamin Schmidt

Hereditary Diffuse Gastric Cancer

What is the mutation?

CDH1
E-cadherin
(same thing)

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Hereditary Diffuse Gastric Cancer

what other cancer is associated?

lobular breast cancer

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Hereditary Diffuse Gastric Cancer

International Gastric Cancer Linkage Consortium (IGCLC) criteria for referral to genetic testing (3)

Either:
A: 2 cases of diffuse GC in 1st or 2nd degree relative with 1 case documented before age 50
B: 3 cases of diffuse GC in 1st or 2nd degree relative regardless of age.
C: 1 case of diffuse GC before age 40

25-50% will carry a mutated CDH1 allele

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Hereditary Diffuse Gastric Cancer
What is cumulative risk of GC and lobular BC?
What is average age of onset of clinically evident invasive GC?

Cumulative risk by age 80:
GC 70% in males; 56% in females
lobular BC 42% (39-52%) in females

Age of onset is 38 but varies with mutation.

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Hereditary Diffuse Gastric Cancer

endoscopic surveillance?

Annual EGD with random biopsies
Starting at age 20
or
5-10 years before earliest familial case.

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Hereditary Diffuse Gastric Cancer

when to do total gastrectomy?

Offer after childhood growth is complete and extensive QOL preoperative discussion. (early 20s)

Endoscopy fails to discover T1a cancers.

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Hereditary Diffuse Gastric Cancer

what percentage of patients will have T1a cancer on prophylactic total gastrectomy?

85% despite minimal endoscopic findings
[Strong Annals of Surg 2016]

others report up to 92%

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Hereditary Diffuse Gastric Cancer - Total Gastrectomy

What to check frozen sections for?

Confirm tumor free

2. confirm squamous mucosa and duodenal mucosa to ensure complete removal of gastric mucosa.

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Hereditary Diffuse Gastric Cancer - Total Gastrectomy

Do you need to do a D2?

No - D1 by consensus guidelines.

No evidence for D2 as lymph node mets are exceedingly rare.

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Hereditary Diffuse Gastric Cancer - Total Gastrectomy

What is the trend for Wt and QOL scores?

decrease until 6-12 months post-op and then stabilize or improve.

Final Wt loss ~10%
[Strong Annals of Surg 2016]

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Hereditary Diffuse Gastric Cancer

what percentage of all GC?

1-3%

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Hereditary Diffuse Gastric Cancer

Who to potentially offer endoscopic surveillance?

Patients refusing or deferring total gastrectomy
Patients meeting IGCLC criteria, but cannot document a mutation.
[Lim Gastrointestinal Endoscopy 2014]

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Hereditary Diffuse Gastric Cancer

What techniques might optimize endoscopic detection?

High resolution white light endoscopywith targeted and multiple random biopsies detected signet ring carcinoma in 63% of CDH1 carriers. Also used autoflourescence, but concluded it didn’t help [Lim Gastrointestinal Endoscopy 2014]

blue dye

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Hereditary Diffuse Gastric Cancer

What is the histological subtype?

Signet Ring

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Hereditary Diffuse Gastric Cancer
What percent of families have CDH1 mutation?
What are some other possible genetic causes?

40% of families have a CDH1 mutation (>100 known mutations)
alpha-catenin (CTNNA1) same pathway
other genetic syndromes (BRCA-2, Peutz-Jeghers)

[Hansford JAMA Oncology 2015]

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What percent of gastric adenocarcinoma will have positive peritoneal cytology without gross metastatic disease?

~10%

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Gastric cancer 3 year overall survival for positive peritoneal cytology and no progression after neoadjuvant therapy?

3-year OS rate of 12% versus 0% for patients who did not receive neoadjuvant therapy.

[Badgwell MDACC 18649106]

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20 year risk of a gastric remnant carcinoma?

4% (2.9% in 16 years)

[Morgagni pmid 25218580]

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Most important prognostic indicator of long term-survival for second operation for gastric cancer? (indicator of metachronous cancer)

Non-anaastomotic recurrence had 95% v 37% 5 year survival in one south korean study.

PMID 18080844

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Endoscopic Mucosal Resection (EMR) indications

GE cancer limited to the muscularis mucosa (T1a) with <10% risk of lymph node involvement

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Reccurent Esophageal Cancer

Re-resection if surgically feasible;
Consider brachytherapy for non-surgical candidate
[Wong Hee Kam, Rivera Brachytherapy 2015]

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Tylosis

autosomal dominant
hyperkeratosis of palms
Esophageal SSC at age 45

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Bloom Syndrome

Autosomal recessive chromosomal breakage
Leukemias
Wilm’s Tumors
Early esophageal SCC

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Fanconi’s Anemia

autosomal recessive DNA repair mutation
congenital malformation
AML
SCC of numerous locations
Pancytopenia

Risk of cancer with a biopsy of high grade dysplasia with Barretts Esophagus?

10% for T1b or higher

Barretts Esophagus Surveillance

no dysplasia - every 3-5 years low grade dysplasia - every 6-12 months high grade dysplasia - every 3 months (or eradicate)

Treatment for esophageal cancer invading the pericardium?

Still resectable

Siewert classification

I is within 5 cm above the GE junction II is 1 cm above or 2 cm below the GE junction III is 5 cm below the GE junction but invades up to it (stage and treat III like gastric cancer)

Common mutation in SCC

inactivation of Notch1

What are optimal staging studies before and after neoadjuvant chemoRT for esophageal cancer?

before: EUS (better for nodes) and PET (complimentary) after: just PET

Three classes of gastric carcinoids

Type I (>60%) - associated with chronic atrophic gastritis Type II - associated with MEN-I Type III - sporadic

Type I gastric carcinoids are

well differentiated and limited to the mucosa without invasion.

Work-up for type I and type II gastric carcinoids

Endoscopy and CT scan are standard | Nuclear med test exists (Somatostatin Scintigraphy)

Treatment of type I and Type II gastric carcinoids

Endoscopic removal and 6 month surveilance Antrectomy and local resection for recurrence (<5 tumors, all <1cm, no evidence of metastatic disease on imaging)

Type III gastric carcinoids

usually solitary arise in normal stomach >2cm

Treatment of Type II gastric carcinoids

Same surgical approach as gastric adeno | 15% have lymph node involvement at presentation.

ACOSOG Z9001

DeMatteo trial for adjuvant imatinib for GISTs. Stopped early since results were so positive. 20% recurrence with placebo v 8% with gleevec

RTOG 0132

Good outcomes with neoadjuvant imatinib. Has been accepted without any direct comparrison

Scandinavian Sarcoma Group XVIII

1 v 3 years of imatinib trial. three years is better, patients tend to recur once it is stopped.

Inclusion criteria for Scandinavian Sarcoma Group XVIII

``` At least one of the following: tumor >10cm mitotic count > 10/50hpf tumor >5 cm and mitosis > 5 tumor rupture before or during surgery ```

Surgery for ampullary adenocarcinoma

Whipple! All other are investigational with inferior results. 58% v 78% OS at 2 years [Roggin KK, Yeh JJ, Ferrone CR 2005]

What patients saw the benefit of 3 years of gleevec in SSG 18

only c-kit exon 11 mutations.

Another phrase for goblet cell tumors

mixed adeno-neuroendocrine carcinomas

Management of goblet cell tumor of the appendix

agressive; right hemicolectomy for all, unless there is metastatic disease.

systemic therapy for goblet cell tumor

FOLFOX

Management of carcinoid tumor of the appendix

``` Right hemicolectomy for any of the following: tumor >2cm indeterminate size involvement of the base lymphovascular invasion invasion of the mesoappendix intermediate and high grade tumors tumors with mixed histology (adeno-, goblet-) ```

STK11

gene for Peutz Jehgers Syndrome

Peutz Jeghers Syndrome

pigmented oral spots GI hamartomas numerous malignancies

Medical causes of increases Chromogranin A

``` PPIs H2 blockers chronic atrophic gastritis renal failure liver failure ```

Non-neuroendocrine tumors that can secrete Chromogranin A

pancreatic cancer hepatocellular carcinoma prostate cancer

What additional organ should you take out for all carcinoid tumors?

Gallbladder; | Somatostatin can cause gallstones

Median survival for mucinous appendiceal tumors

16 years

What adjuvant therapies are there after HIPEC for mucinous appendiceal tumors?

NONE; | Can consider imaging surveilance and tumor markers.

EPIC (intraperitoneal chemotherapy)

No heat No OR No level I data

Most common complication of HIPEC

prolonged ileus

Side effect of cisplatin HIPEC

renal failure - Give thiosulfate

survival after HIPEC for low grade mucinous appendiceal tumors

80% at 20 years

survival after HIPEC for high grade mucinous appendiceal tumors

45% at 20 years