Surgical Oncology Board Review - Gastrointestinal

Front 1

What are good treatment options for obstructing esophageal cancer in patients with metastatic or unresectable disease?

Back 1

Esophageal stent (fast relief, complications: migration esp in GE junction tumors, late bleeding)

OR

Brachytherapy (durable relief)

Chemo will give response but not as effective as others for symptom relief

Front 2

What did the CROSS trial show?

Back 2

Dutch RCT that randomized patients with resectable esophageal or GE jxn tumors to neoadjuvant chemoradiation with carboplatin and paclitaxel (5 weeks, 41 Gy) versus concurrent radiation followed by surgery versus surgery alone

75% adenocarcinoma, 23% SCC

Clinical stage T1N1 or T2-3N0-1 M0

R0 resection achieved in 92% of the patients in the chemoradiation group compared with 69% of the surgery-alone group (p<0.001)

pCR seen in 29% of patients treated with chemoradiation

Positive lymph nodes were found on final pathology in 31% of patients in the chemoradiation arm compared with 75% of the patients in the surgery-alone arm (p<0.001)

Similar rates of postoperative complications were similar between the two groups (~25% anastomotic leak, 4% mortality)

Median OS was 50 months for the chemoradiation group vs 24 months for the surgery-alone group (p=0.003)

Front 3

What are criteria for EMR approach to esophageal cancer?

Back 3

T1a lesions (restricted to muscularis mucosa)

(Risk of +LN <10% vs 20% for T1b)

Front 4

What is tylosis?

Back 4

Hyperkeratosis of the palms and soles

High risk of esophageal SCC

Chromosome 17 mutation

Begin surveillance EGD at age 20

Front 5

What is Bloom syndrome?

Back 5

AML, ALL, lymphoma

Wilms tumor

Esophageal SCC

Begin surveillance at age 20

Front 6

What is Fanconi anemia?

Back 6

FANC gene mutation

It results in congenital malformations, progressive pancytopenia, and an increased predisposition to both hematologic and solid organ malignancies. Acute myeloid leukemia is the most common malignancy in affected patients; however, squamous cell carcinomas of the head, neck, and esophagus; cervical cancer; and brain tumors may also develop. Affected individuals typically present with pancytopenia, anemia, bleeding, and easy bruising. Homozygous individuals may be identified with enhanced mitomycin C-induced chromosomal breakage analysis.

Front 7

What data is there to support definitive chemoradiation for esophageal SCC?

Back 7

FFCD 9102 - French RCT

No survival benefit for operable esophageal SCC when surgical resection was added to 5-FU and cisplatin-based chemoradiation therapy

Front 8

Describe T stages for esophageal cancer.

Back 8

Tis - In situ or high grade dysplasia

T1a—tumor does not invade submucosa and is limited to mucosa (intramucosal, can treat with EMR or photodynamic therapy or RFA or esophagectomy)
T1b—tumor invades the submucosa
T2—tumors invade the muscularis propriaT3—tumors invade the adventitia
T4a--involve adjacent structures but resectable, i.e., invade the pleura, pericardium, or diaphragm
T4b tumors--unresectable because they invade structures such as the aorta or trachea

Front 9

Describe N stages for esophageal cancer.

Back 9

N1—metastases in 1-2 regional lymph nodes

N2—metastases in 3-6 regional lymph nodes

N3— metastases in 7 or more regional lymph nodes

Front 10

Define Siewart classification.

Back 10

5-1-2-5 rule

Based on location of center of tumor with respect to anatomic EGJ

5cm to 1cm above EGJ --> Siewart I (esophageal)

1cm above to 2cm below EGJ --> Siewart II (true esophagogastric)

2cm to 5cm below EGJ --> Siewart III (gastric)

Front 11

NOTCH1 mutations are associated with which cancer?

Back 11

SCC of esophagus

Front 12

Discuss types of gastric carcinoid and management.

Back 12

Type I

- Chronic atrophic gastritis

- Most common
- EUS/surveillance reasonable if < 5 lesions all < 1cm
- Antrectomy if recurs

Type 2
- Zollinger-Ellison syndrome (ZES), MEN 1 syndrome
- EUS/surveillance reasonable if < 5 lesions all < 1cm
- Antrectomy if recurs

Type 3
- Sporadic, no association with hypergastrinemia.
- Usually larger, >2cm
- Treat like gastric cancer
- More aggressive

Front 13

What is the management of T1 ampullary cancers?

Back 13

Pancreaticoduodenectomy

Front 14

What did the Macdonald trial show?

Back 14

RCT after R0 resection for gastric cancer --> observation vs postop chemoradiation (5-FU, leucovorin, 45 Gy RT)

Median survival was 36 months in the chemoradiotherapy group vs 27 months in the surgery-only group.

Front 15

What did the MAGIC trial show?

Back 15

RCT after R0 resection for gastric/lower esophageal cancer --> Surgery alone vs Periop chemo (3 pre- and 3 post-op cycles of ECF - epirubicin, cisplatin, fluorouracil)

5-year survival rate was 36% for the perioperative group compared with 23% for the surgery-alone group.

Front 16

What did the ARTIST I trial show?

Back 16

RCT that compared adjuvant chemotherapy alone (capecitabine and cisplatin) vs combined treatment with capecitabine and radiotherapy

Unlike in Macdonald and MAGIC trials, all patients underwent D2 lymphadenectomy

Similar 3-year disease-free survival and overall survival rates (so overall was a negative trial), but in patients with positive lymph nodes, combined treatment demonstrated improved overall survival.

Front 17

What did the 15 year follow up of the Dutch trial comparing D1 vs D2 lymphadectomy for gastric cancer show?

Back 17

- Gastric cancer-specific death rates were lower in the D2 lymphadenectomy group compared with the D1 group (48% vs. 37%)

- Lower loco-regional recurrence rate was reported for the D2 group

- D2 lymphadenectomy associated with higher postoperative morbidity, mortality, and reoperation rates

- No survival advantage for the group undergoing D2 lymphadenectomy inclusive of splenectomy and pancreatectomy

- BUT subgroup analysis of patients undergoing D2 lymphadenectomy without pancreatectomy and splenectomy demonstrated improved survival

Front 18

How many lymph nodes should be obtained during gastric cancer resection?

Back 18

15

Front 19

What is the optimal adjuvant treatment after Whipple for duodenal adenocarcinoma?

Back 19

Postop FOLFOX

Front 20

What is the management of appendiceal goblet cell tumor found on lap appy specimen with no metastatic disease?

Back 20

GCTs are aggressive tumors. 20% or more metastatic at presentation

Right hemicolectomy is recommended for all GCTs of the appendix regardless of size, grade, and margin status.

Adjuvant FOLFOX is recommended for lymph node-positive tumors.

In cases of metastatic disease, treat with systemic chemotherapy (FOLFOX) or cytoreductive surgery with or without intraperitoneal chemotherapy

Front 21

What are the indications for right hemicolectomy for appendiceal carcinoid?

Back 21

- Tumor > 2 cm or size cannot be determined

- Invasion of the base of the appendix

- Incompletely resected tumor or positive margin

- Lymphovascular invasion

- Invasion of the mesoappendix

- Intermediate- or high-grade tumor, tumors with mixed histology (goblet-cell carcinoid, adenocarcinoid)

- Lymph node involvement

Front 22

Describe salient features of Peutz Jehger syndrome.

Back 22

- Autosomal dominant

- Mutation in STK11 on chromosome 19

- Mucocutaneous pigmentations on buccal mucosa and lips

- GI hamartomatous polyps

- Increased risk for gastrointestinal and other malignancies.

Front 23

What lab tests should you order in a patient with suspected carcinoid syndrome from GI neuroendocrine tumor?

Back 23

Serum chromogranin A (for tumor burden baseline and monitoring)

Urinary 5-hydroxyindoleacetic acid (5-HIAA) (for confirming suspected carcinoid syndrome)

Front 24

What is the standard management of low grade appendiceal mucinous neoplasm with pseudomyxoma peritoneii? Is adjuvant therapy indicated?

Back 24

Cytoreductive surgery and HIPEC

No adjuvant therapy for these low grade neoplasms (contrast with peritoneal disease from metastatic colorectal cancer)

Front 25

What is the staging workup for a patient with gastric cancer that is T2 or greater and/or N1 or greater and no obvious metastatic disease?

Back 25

Chest/abd/pelv CT

PET-CT

Staging laparoscopy with peritoneal washings
(mostly for T3 or N1, but consider for T2 if other high risk features or high risk patient)

Front 26

What molecular marker should be evaluated in tumors of patients with metastatic gastric cancer?

Back 26

HER-2

Front 27

Describe T staging for gastric cancer.

Back 27

Tis Intraepithelial tumor without invasion of lamina propria

T1a Tumor invades lamina propria or muscularis mucosa without invading submucosa

T1b Tumor invades submucosa

T2 Tumor invades muscularis propria

T3 Tumor penetrates subserosal connective tissue without invasion of visceral peritoneum or adjacent structures

T4a Tumor invades serosa (visceral peritoneum)

T4b Tumor invades adjacent structures

Front 28

Describe N staging for gastric cancer

Back 28

N0 none

N1 1-2 nodes involved

N2 3-6 nodes involved

N3a 7-15 nodes involved

N3b 16 or more nodes involved

Front 29

Describe Japanese nodal staging based on stations.

Back 29

N1: lesser curvature (1, 3, 5) and greater curvature (2, 4, 6)

N2: left gastric (7), common hepatic artery (8), celiac artery (9) and splenic artery (10, 11)

N3: hepatoduodenal, retropancreatic, root of mesentery

N4: paraaortic

Front 30

What did the ToGA trial show?

Back 30

Improved OS with addition of trastuzumab in patients with HER2 + advanced gastric cancer compared to chemo alone

Front 31

Discuss hereditary diffuse gastric cancer.

Back 31

- Autosomal dominant

- CDH1 mutation - defect in adhesion protein E cadherin - found in 30-50% of families

- Avg age at diagnosis is 37

- Lifetime risk of gastric cancer 70-80%

- Assoc with lobular carcinoma in women

Front 32

What are the guidelines for risk reducing surgery in patients with hereditary diffuse gastric cancer?

Back 32

- Total gastrectomy should be offered starting at age 18 and should be done no later than age 40

- Offer earlier if there is a family member who had gastric cancer before age 25

- Conduct baseline endoscopy prior to surgery

- Do not need to do D2 dissection if no cancer detected beforehand

- Women should consider risk reduction for breast cancer (tamoxifen, bilateral prophylatic mastectomy, etc)

Front 33

What life threatening condition should always be considered in a patient with esophageal cancer who presents with acute onset upper GI bleeding?

Back 33

Aortoesophageal fistula

Front 34

Other than TNM classification, what factors do the staging systems for esophageal adenocarcinoma and SCC take into account?

Back 34

Esophageal and EGJ adenocarcinoma: T, N, M, Grade

Esophageal and EGJ SCC: T, N, M, Grade, Tumor location (upper/proximal is worse)

Front 35

What are the causes of appendiceal mucocele?

Back 35

"Mucocele" itself is not a pathologic diagnosis but the end result of luminal obstruction and distention of the appendix with fluid

Causes include:

Retention cysts

Hyperplastic polyps

Mucinous cystadenoma

Mucinous cystadenocarcinoma

Front 36

Does a ruptured mucocele always lead to pseudomyxoma peritoneii?

Back 36

No. Mucus dispersed from ruptured appendix due to retention cyst or hyperplastic polyp will not cause PMP

But ruptured mucoceles secondary to cystadenoma or cystadenocarinoma can lead to PMP

Front 37

What is DPAM?

Back 37

Disseminated peritoneal adenomucinosis

- Proper name for PMP

- Associated with benign but ruptured appendiceal cystadenoma

- Indolent course

Compare to peritoneal mucinous carcinomatosis

Front 38

Name 2 radiographic criteria that predict poor response to HIPEC for peritoneal surface malignancy

Back 38

- Segmental obstruction of the small bowel

- Tumor nodules >5 cm in diameter on small bowel surfaces or directly adjacent to the small bowel mesentery

Front 39

Define Stage IA and IB gastric cancer.

Back 39

IA: T1 N0

IB: T2 N0 M0

T1 N1 M0

Front 40

Define Stage IIA and IIB gastric cancer

Back 40

IIA: T3 N0 M0

T2 N1 M0

T1 N2 M0

IIB: T4a N0 M0

T3 N1 M0

T2 N2 M0

T1 N3 M0

Front 41

Define Stage IIIA, IIIB, and IIIC gastric cancer.

Back 41

IIIA: T4a N1 M0

T3 N2 M0

T2 N3 M0

IIIB: T4b N0 M0

T4b N1 M0

T4a N2 M0

T3 N3 M0

IIIC: T4b N2 M0

T4b N2 M0

T4a N3 M0

**Yes, it's crazy that you need to memorize this.

Front 42

You are consulted to place a venting gastrotomy tube in a patient with obstructing, metastatic gastric cancer. You notice ascites on the CT scan. What should you do prior to procedure?

Back 42

Drain the ascites

Front 43

What are the 4 clinical assessments described by Sugarbaker to select patients who respond best to cytoreductive surgery and HIPEC?

Back 43

PREOP

1) Histopathologic assessment - Noninvasive malignancies (PMP, peritoneal mesothelioma) more likely to respond

2) CT C/A/P – Exclude segmental small bowel obstruction & nodules >5 cm in diameter on small bowel surfaces or directly adjacent to the small bowel mesentery in the jejunum or upper ileum.

INTRAOP

3) Peritoneal cancer index (PCI) - Quantitative indicator of prognosis derived from the size and distribution of nodules on the peritoneal surface

4) Completeness of cytoreduction score - size of persisting tumor nodules after maximal cytoreduction), are derived intraoperatively.

Front 44

Describe PCI and how to calculate score.

Back 44

Abdomen divided into 9 sections + 4 divisions of midgut (upper/lower jejunum, upper/lower ileum)

Score each section 0-3

0 = no tumor

1 = tumor < 0.5cm

2 = tumor 0.5cm-5cm

3 = tumor > 5cm

Max score 39

Front 45

Describe CRS & HIPEC technique

Back 45

Modified lithotomy

NGT, Foley, lines

Subxiphoid to pubis incision

Assess to make sure complete CR can be achieved (<1mm)

Proceed with peritonectomy, greater and less omentectomy, cholecystectomy, and organ specific resection including bowel resection

Set up inflow, outflow, temperature probes, close skin

Mitomycin C (15mg/m2), 42 degrees C, 90 minutes, shake

Keep urine output high (>400cc/h) using dopamine if needed

Monitor body temp, turn off Bair hugger, use ice if needed

Open, wash out

Anastomoses

Drains

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