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Surgical Oncology > Gastric > Flashcards

Gastric Flashcards

1
Q

Hereditary Diffuse Gastric Cancer

What is the mutation?

A

CDH1
E-cadherin
(same thing)

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2
Q

Hereditary Diffuse Gastric Cancer

what other cancer is associated?

A

lobular breast cancer

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3
Q

Hereditary Diffuse Gastric Cancer

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

A

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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4
Q

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

A

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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5
Q

Hereditary Diffuse Gastric Cancer

endoscopic surveillance?

A

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

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6
Q

Hereditary Diffuse Gastric Cancer

when to do total gastrectomy?

A

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

Endoscopy fails to discover T1a cancers.

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7
Q

Hereditary Diffuse Gastric Cancer

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

A

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

others report up to 92%

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8
Q

Hereditary Diffuse Gastric Cancer - Total Gastrectomy

What to check frozen sections for?

A
  1. Confirm tumor free

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

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9
Q

Hereditary Diffuse Gastric Cancer - Total Gastrectomy

Do you need to do a D2?

A

No - D1 by consensus guidelines.

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

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10
Q

Hereditary Diffuse Gastric Cancer - Total Gastrectomy

What is the trend for Wt and QOL scores?

A

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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11
Q

Hereditary Diffuse Gastric Cancer

what percentage of all GC?

A

1-3%

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12
Q

Hereditary Diffuse Gastric Cancer

Who to potentially offer endoscopic surveillance?

A

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

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13
Q

Hereditary Diffuse Gastric Cancer

What techniques might optimize endoscopic detection?

A

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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14
Q

Hereditary Diffuse Gastric Cancer

What is the histological subtype?

A

Signet Ring

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15
Q

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

A

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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16
Q

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

A

~10%

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17
Q

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

A

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

[Badgwell MDACC 18649106]

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18
Q

20 year risk of a gastric remnant carcinoma?

A

4% (2.9% in 16 years)

[Morgagni pmid 25218580]

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19
Q

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

A

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

PMID 18080844

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20
Q

Endoscopic Mucosal Resection (EMR) indications

A

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

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21
Q

Reccurent Esophageal Cancer

A

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

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22
Q

Tylosis

A

autosomal dominant
hyperkeratosis of palms
Esophageal SSC at age 45

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23
Q

Bloom Syndrome

A

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

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24
Q

Fanconi’s Anemia

A 
autosomal recessive DNA repair mutation
congenital malformation
AML
SCC of numerous locations
Pancytopenia
25
Q

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

A

10% for T1b or higher

26
Q

Barretts Esophagus Surveillance

A

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

27
Q

Treatment for esophageal cancer invading the pericardium?

A

Still resectable

28
Q

Siewert classification

A

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)

29
Q

Common mutation in SCC

A

inactivation of Notch1

30
Q

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

A

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

31
Q

Three classes of gastric carcinoids

A

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

32
Q

Type I gastric carcinoids are

A

well differentiated and limited to the mucosa without invasion.

33
Q

Work-up for type I and type II gastric carcinoids

A

Endoscopy and CT scan are standard

Nuclear med test exists (Somatostatin Scintigraphy)

34
Q

Treatment of type I and Type II gastric carcinoids

A

Endoscopic removal and 6 month surveilance
Antrectomy and local resection for recurrence

(<5 tumors, all <1cm, no evidence of metastatic disease on imaging)

35
Q

Type III gastric carcinoids

A

usually solitary
arise in normal stomach
>2cm

36
Q

Treatment of Type II gastric carcinoids

A

Same surgical approach as gastric adeno

15% have lymph node involvement at presentation.

37
Q

ACOSOG Z9001

A

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

38
Q

RTOG 0132

A

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

39
Q

Scandinavian Sarcoma Group XVIII

A

1 v 3 years of imatinib trial.

three years is better, patients tend to recur once it is stopped.

40
Q

Inclusion criteria for Scandinavian Sarcoma Group XVIII

A 
At least one of the following:
tumor >10cm
mitotic count > 10/50hpf
tumor >5 cm and mitosis > 5
tumor rupture before or during surgery
41
Q

Surgery for ampullary adenocarcinoma

A

Whipple!
All other are investigational with inferior results. 58% v 78% OS at 2 years

[Roggin KK, Yeh JJ, Ferrone CR 2005]

42
Q

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

A

only c-kit exon 11 mutations.

43
Q

Another phrase for goblet cell tumors

A

mixed adeno-neuroendocrine carcinomas

44
Q

Management of goblet cell tumor of the appendix

A

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

45
Q

systemic therapy for goblet cell tumor

A

FOLFOX

46
Q

Management of carcinoid tumor of the appendix

A 
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-)
47
Q

STK11

A

gene for Peutz Jehgers Syndrome

48
Q

Peutz Jeghers Syndrome

A

pigmented oral spots
GI hamartomas
numerous malignancies

49
Q

Medical causes of increases Chromogranin A

A 
PPIs
H2 blockers
chronic atrophic gastritis
renal failure
liver failure
50
Q

Non-neuroendocrine tumors that can secrete Chromogranin A

A

pancreatic cancer
hepatocellular carcinoma
prostate cancer

51
Q

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

A

Gallbladder;

Somatostatin can cause gallstones

52
Q

Median survival for mucinous appendiceal tumors

A

16 years

53
Q

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

A

NONE;

Can consider imaging surveilance and tumor markers.

54
Q

EPIC (intraperitoneal chemotherapy)

A

No heat
No OR
No level I data

55
Q

Most common complication of HIPEC

A

prolonged ileus

56
Q

Side effect of cisplatin HIPEC

A

renal failure - Give thiosulfate

57
Q

survival after HIPEC for low grade mucinous appendiceal tumors

A

80% at 20 years

58
Q

survival after HIPEC for high grade mucinous appendiceal tumors

A

45% at 20 years

Surgical Oncology (23 decks)

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