NCCN Esophageal Cancer

Question 1

Workup for esophageal cancer.

Answer 1

Upper GI and endoscopy.
EUS if no evidence of M1 disease - ER is best for accurate staging of early-stage cancer (T1a or T1b).
CT chest and abdomen w/ oral and IV contrast. Add pelvis if needed.
Consider FDG PET.
CBC and CMP.
Biospy anything that could be metastatic.
MSI testing by NGS or MMR by IHC is recommended in all newly dx patients.
PD-L1 if advanced/metastatic dz is suspected.
HER2 if metastatic adenocarcinoma is suspected.
Bronchoscopy if tumor is at or above the carina w/o evidence of M1.
Nutritional asessment and counseling.
Smoking cessation/counseling/pharmacotherapy.
Screen for family history.

Question 2

Early-stage esophageal cancers can best be diagnosed by?

Answer 2

Endoscopic resection (ER)

Question 3

What extra lab/genetic workup is needed for esophageal cancer if metastatic disease is documented?

Answer 3

MSI and HER2.

Question 4

Esophageal cancer with tumor at or above the carina w/o evidence of metastasis requires what extra workup?

Answer 4

Bronchoscopy

Question 5

What important lifestyle modification is required for esophageal cancer patients?

Answer 5

Smoking cessation. Same with lung cancer pts.

Question 6

Esophageal SCC Stage I-IVA (locoregional, except T4b or unresectable N3) assessment and next step?

Answer 6

Locoregional disease.
Requires multidisciplinary evaluation. Consider enteric feeding tube.
Assess for surgical candidacy (medical fitness and patient consent).

Question 7

Esophageal SCC pTis, pT1a, pT1b staging is defined via what workup?

Answer 7

Defined by pathology of diagnostic ER specimen. This may be therapeutic outright, but some may need additional therapy to start the surveillance.

Question 8

Esophageal SCC pTis preferred next step?

Answer 8

Endoscopic therapies: ER, ER and ablation, only ablation. Then endoscopic surveillance. *Can be done for cervical lesions.

Esophagectomy is an option, but not preferred.

Almost same for pTis, but cannot do ablation only for pT1a.

Question 9

Esophageal SCC pT1a preferred next step?

Answer 9

Endoscopic therapy: ER, or ER followed by ablation. Then endoscopic surveillance. *Can be done for cervical lesions.

Esophagectomy is an option as well, but not preferred.

Almost same for pTis, but cannot do ablation only for pT1a.

Question 10

Esophageal SCC pT1b N0 tx AND medically fit for surgery?

Answer 10

Esophagectomy.

Not the only preop dx that can go straight to surgery (also cT2, N0). These must be low risk lesions - <3 cm, well differentiated, not cervical.

Question 11

Esophageal SCC s/p esophagectomy (no previous chemoradiation) w/ R1 or R2 resection next step?

Answer 11

Chemoradiation.
Setting would be lesions Tis and T1a that opted for esophagectomy rather than endoscopy and ended up w/ microscopic or gross positive margins; or up to cT2 N0 lesions that are low risk (<3cm, well differentiated; not cervical) that got esophagectomy w/ positive margins.

Question 12

Esophageal SCC cT1b-T2 N0 (low risk: <3cm, well differentiated [ie not poorly differentiated], no LVI; not cervical SCC) and medically fit for surgery preferred primary treatment option?

Answer 12

Esophagectomy.
Gastric reconstruction is preferred. Feeding jejunostomy, though not required, is generally preferred.

Question 13

Esophageal SCC cT2 N0 but high-risk (LVI, 3cm or more, poorly differentiated),
cT3-cT4a,
or any N+,
AND NOT cervical…
Primary treatment option for medically fit patient?

Answer 13

Preop chemoradiation, response assessment, then esophagectomy (can be possible to do surveillance if NED after response assessment).
Remember: ideally the suspected lymph nodes are histologically confirmed.

Can opt for definitive chemoradiation.

Question 14

Can preclinical staging for esophageal cancer establish the number of nodes?

Answer 14

No, but you should still attempt to histologically confirm clinically suspicious nodes.

Question 15

Esophageal SCC in cervical esophagus that is either cT2 and high risk (3cm or more, LVI, or poorly differentiated), N+, or cT3 or greater primary treatment option?

Answer 15

definitive chemoradiation and follow up

Question 16

Esophageal SCC cT4b primary treatment option?

Answer 16

Definitive chemoradiation. Consider endoluminal stent.

Consider chemo alone if invades into trachea, great vessels, vertebral body, or heart.

Question 17

What is the response assessment in medically fit patients with esophageal SCC s/p preoperative OR definitive chemoradiation?

Answer 17

FDG-PET/CT (or CT chest/abd w/ oral and IV contrast), upper GI and biopsy (can be omitted if planning on surgical intervention)

Question 18

What if during response assessment in medically fit patients with esophageal SCC s/p chemoradiation (preop or definitive), a clinician discovers persistent local disease? What is the additional management?

Answer 18

Esophagectomy is preferred, even after definitive chemoradiation (paclitaxel and carboplatin in CROSS trial).

Question 19

What if during response assessment in medically fit patients with esophageal SCC s/p PREOPERATIVE chemoradiation, a clinician discovers NO evidence of disease? What is the additional management?

Answer 19

Esophagectomy, but surveillance can be chosen.
If surveillance chosen, need to have upper GI and biopsy to confirm (can be omitted if surgery is planned)

Question 20

What if during response assessment in medically fit patients with esophageal SCC s/p DEFINITIVE chemoradiation, a clinician discovers NO evidence of disease? IE is there additional management?

Answer 20

None. Surveillance.
This is different than finding persistent local disease, in which esophagectomy is preferred.

Question 21

What if during response assessment in medically fit patients with esophageal SCC s/p preoperative OR definitive chemoradiation, a clinician discovers unresectable disease or metastasis? What is the additional management?

Answer 21

Palliative management

Question 22

An esophageal SCC patient has not received preoperative chemoradiation, and surgical outcome is R0, what should be done for postop management?

Answer 22

Surveillance.
ANY T. ANY N.
Don’t add anything unless R1 or R2.

Question 23

In esophageal SCC patients who have NOT received preop chemoradiation who have an R1 or R2 outcome after esophagectomy, what is the base of for the chemo (part of chemoradiation)?

Answer 23

Fluoropyrimidine

Question 24

Esophageal SCC pt s/p chemoradiation AND esophagectomy is found to have R0 resection. Tumor classification is yp T0 N0. What is the postop management?

Answer 24

Surveillance

Question 25

Esophageal SCC pt s/p chemoradiation AND esophagectomy is found to have R0 resection. Tumor classification is ANYTHING BUT yp T0 N0. What is added to postop management?

Answer 25

Nivolumab

Question 26

Esophageal SCC pt s/p chemoradiation AND esophagectomy is found to have R1 or R2 resection, what is the postop management?

Answer 26

observation until progression or palliative management

Question 27

How do you manage pTis esophageal SCC in medically UNFIT patients?

Answer 27

ER, ER followed by ablation, or ablation; then endoscopic surveillance.
Lots of endoscopic options.

Question 28

How do you manage pT1a esophageal SCC in medically UNFIT patients?

Answer 28

ER or ER followed by ablation, then endoscopic surveillance.
As opposed to pTis, cannot do ablation alone.

Question 29

How do you manage pT1b esophageal SCC in medically UNFIT patients?

Answer 29

ER or ER followed by ablation, then endoscopic surveillance.
The difference b/w pT1b and pT1a, is that with pT1b, you can add definitive chemoradiation IF the patient has poor prognostic features:
LVI, poorly differentiated, 2cm or more (not 3cm, which is used for surgical assessment), positive margins.
The difference b/w pT1b for medically unfit patients is that endoscopic therapy can be offered, as opposed to esophagectomy, which would be ideal.

Question 30

How do you manage >pT1b or N+ esophageal SCC in medically UNFIT patients?

Answer 30

Figure out what they can tolerate, and offer it:
Definitive chemoradiation, palliative RT, or palliation then follow up.

Question 31

What is follow up/surveillance for esophageal SCC?

Answer 31

H&P w/ increasing intervals and nutritional assessment and counseling.
The rest is as clinically indicated: CMP and CBC, upper GI and bx.

IE no routine EGD or CT scans.

Question 32

During follow up/surveillance for esophageal SCC, anastomotic stenosis is found w/o evidence of cancer, what can be done?

Answer 32

Dilation

Question 33

During follow up/surveillance for esophageal SCC s/p esophagectomy WITHOUT chemoradiation, locoregional recurrence is found, what is next preferred?
What are other options?

Answer 33

Concurrent chemoradiation is preferred.

Chemotherapy alone is an option. (Second) Surgery is an option. Or can do palliative/best supportive care.

Response assess w/ CT chest and abdomen w/ contrast.

Recurrence again means palliative.

Question 34

During follow up/surveillance for esophageal SCC s/p chemoradiation ONLY, locoregional recurrence is found, what is next?

Answer 34

Assess medical fitness.
Can do esophagectomy if anatomically feasible and able.
Assess response w/ CT chest and abdomen w/ contrast.
If second recurrence, palliate.

Question 35

During follow up/surveillance for esophageal SCC s/p chemoradiation ONLY, locoregional recurrence is found but it is unresectable and/or patient is not medically fit, what is next?

Answer 35

palliative management

Question 36

During follow up/surveillance for esophageal SCC, regardless of treatment, metastatic disease is found, what is the management?

Answer 36

palliative

Question 37

Pt w/ esophageal SCC has unresectable locally adv disease, locally recurrent, or metastatic disease, what are the treatment options?
What factors into decision making?

Answer 37

Determine performance status:
If Karnofsky performance 60% or > or ECOG 2 or less => microsatellite and PD-L1 testing for systemic therapy.
Otherwise, palliative/best supportive care.

Question 38

For R side NSCLC, an adequate mediastinal lymphadenectomy includes what stations?

Answer 38

2R, 4R, 7, 8, 9

Question 39

For L side NSCLC, what stations constitute an adequate lymph node staging?

Answer 39

4L, 5, 6, 7, 8, 9

Question 40

Are there any differences in surgical management between SCC and adenocarcinoma for esophageal cancer for T1b?

Answer 40

For superficial pT1b ADENOCARCINOMA, ER followed by ablation can be offered vs esophagectomy.
For T1b esophageal SCC, ONLY esophagectomy is offered.

Question 41

For esophageal cancer, is there any treatment difference b/w adeno and SCC for cT2,N0 high-risk, N+, or T3-T4a?

Answer 41

For ADENO, perioperative or preoperative chemotherapy (ie WITHOUT chemoradiation) can be offered before esophagectomy.

Question 42

For esophageal adenocarcinoma or SCC, what are the options for cT4b?

Answer 42

Definitive chemoradiation.
Chemotherapy alone can be considered in settings of organs that cannot be radiated (trachea, great, vessels, vertebral body, heart).

Question 43

Are there differences in post-surgical treatment between SCC and adenocarcinoma (depending on R status of resection) for pts who HAVE NOT received preop chemo or chemoradx?

Answer 43

R0 for SCC - no matter the T or N, all move to surveillance (focus of SCC is a good resx).
R0 for adenocarcinoma - T2 and above or any N+ should be considered for chemoradiation (similar req to neoadj, except this case no neoadj was given).

R1 gets chemoradiation for either.
R2 gets chemoradiation or palliative for either.

Question 44

For esophageal adenocarcinoma s/p preop chemorad w/ R0 resection and ypT+ and/or N+ disease, what can be added to treatment for postop management?

Answer 44

Nivolumab (same as SCC).

Ie if the neoadj did not wipe out the tumor, give nivolumab for more systemic effect.

Question 45

In what situation can re-resection of an R1 resection esophageal cancer be done?

Answer 45

Can be considered only if ADENOCARCINOMA (ie NOT SCC).
Otherwise, healthy and tumor able to be resected from a technical standpoint.

Question 46

After ER for early esophageal cancer (up to select superficial pT1b [adeno]) in the setting of Barrett, what should be done with the rest of the Barrett esophagus?

Answer 46

Ablative therapy of residual Barrett.

Question 47

In relation to the cricopharyngeus (UES), what would be considered a resectable esophageal cancer?

Answer 47

> 5 cm below cricopharyngeus (UES). Usually below the sternal notch.
The 5 cm b/w the UES and the sternal notch is considered the cervical esophagus.

Question 48

How do you manage esophageal cancer with an epicenter 2.1 cm distal to the EGJ?

Answer 48

Like gastric cancer. This is Siewert Type III.

Question 49

For esophageal cancers, does N+ disease found at staging exclude from surgical candidacy?

Answer 49

No.
Only resectable w/ N+ if T1-T3.
Bulky, multi-station lymphatic involvement is a relative contraindication.

Remeber that during clinical staging, the N stage is not able to be determined, only N+ or N-.

Question 50

What esophageal T4a tumors are resectable?

Answer 50

Pericardium, pleura, diaphragm

Question 51

An esophageal cancer patient has a positive supraclavicular node that is positive. Can you resect?

Answer 51

No.
Pt’s w/ distant (including nonregional lymph nodes) or metastasis (stage IV) are unresectable.

*NCCN: Patients with EGJ and supraclavicular lymph node involvement should be considered unresectable.

Question 52

What are acceptable conduits for esophageal resection? What is preferred?

Answer 52

Gastric (preferred), colon, jejunum.

Question 53

An esoph ca pt undergoes definitive chemoradiation, and on follow-up/surveillance is found to have localized resectable cancer (and is medically operable). Can they be considered for esophagectomy?

Answer 53

Yes. SCC and adenocarcinoma.

Question 54

In esoph ca pts requiring esophagectomy w/o induction chemo, how many nodes should be removed to provide adequate nodal staging?

Answer 54

15. These would be T1b pts.

Question 55

For systemic therapy for esophageal cancer, what can be added for HER2 overexpression?

Answer 55

Trastuzumab.
HER2, MSI, and PD-L1 testing should be tested if metastatic disease is documented or suspected.

Question 56

What characteristics should be identified on EGD for the evaluation and management of possible esophageal cancer?

Answer 56

The location of the tumor relative to the teeth and EGJ, the length of the tumor, the extent of circumferential involvement, and the degree
of obstruction should be carefully recorded to assist with treatment planning. If present, the location, length, and circumferential extent of
Barrett esophagus should be characterized in accordance with the Prague criteria, and mucosal nodules should be carefully documented.

Question 57

Endoscopic Therapy for Early-Stage Esophageal Squamous Neoplasia: no high-risk features. What next?

Answer 57

Lesion size <15 mm: Endoscopic resection
(with either endoscopic submucosal dissection [ESD] or endoscopic mucosal resection [EMR]) ± ablation

Lesion size ≥15 mm: ESD ± ablation

EMR can only be offered if <15 mm

Question 58

Endoscopic Therapy for Early-Stage Esophageal Squamous Neoplasia:
Endoscopic (ulcerated) or pathologic (poorly differentiated or LVI) high-risk features

What next?

Answer 58

Esophagectomy for patients who are medically fit (ESOPH-4) (preferred) or
Preoperative or definitive chemoradiation

Endoscopic hi-risk features in SCC can’t get endoscopic therapy

Question 59

Endoscopic Therapy for Early-Stage Esophageal High-Grade Dysplasia (HGD)/Adenocarcinoma: no nodule or mass.
How do you manage based on the pathology?

Answer 59

High-grade dysplasia: Endoscopic resection (with either ESD or EMR) ± ablation or Ablation alone

Early carcinoma: Endoscopic resection
(with either ESD or EMR) ± ablation

Early carcx (cells past basement membrane) can’t have ablation alone

Question 60

Endoscopic Therapy for Early-Stage Esophageal High-Grade Dysplasia (HGD)/Adenocarcinoma:
How does a nodule affect the management?
How do high-risk features affect the management?

Answer 60

No high-risk biopsy features:
- Nodule/mass size <2 cm: Endoscopic resection (with either ESDg or EMR) ± ablation
- Nodule/mass size ≥2 cm: ESD ± ablation or Esophagectomy (for patients who are medically fit)

Presence of high-risk biopsy features (poor differentiation or LVI): Consider endoscopic resection if technically feasible to accurately stage

Question 61

EUS for esophageal cancer: A dark expansion
of layers 1–3 corresponds with?

Answer 61

infiltration of the superficial and deep mucosa plus the submucosal, T1 disease

Question 62

EUS for esophageal cancer: A dark expansion of layers 1–4 correlates with?

Answer 62

penetration into the muscularis propria, T2 disease

Question 63

EUS for esophageal cancer: expansion beyond the smooth outer border of the muscularis propria correlates with?

Answer 63

invasion
of the adventitia, T3 disease

Question 64

EUS for esophageal cancer: Loss of a bright tissue plane between the area of tumor and surrounding structures such as the pleura,
diaphragm, and pericardium correlates with?

Answer 64

T4a disease

Question 65

EUS: invasion of surrounding structures such as the trachea, aorta, lungs, heart, liver, or pancreas correlates with?

Answer 65

T4b disease

Question 66

Mediastinal and perigastric lymph nodes are readily seen by EUS, and the identification of enlarged, hypoechoic (dark), homogeneous, well
circumscribed, rounded structures in these areas correlates with?

Answer 66

presence of malignant or inflammatory lymph nodes; FNA biopsy them

Question 67

Should EGD/EUS/FNA be done before CT/PET?

Answer 67

The pre-procedure review of CT and FDG-PET scans is recommended, when available, prior to esophagogastroduodenoscopy (EGD)/EUS, to become fully familiar with the nodal distribution for possible FNA.

Question 68

For esophageal cancer, patients with advanced tumors, clinical T3, or N+ disease should be considered for?

Answer 68

Laparoscopic staging with peritoneal washings.

Positive peritoneal cytology (performed in the absence of visible peritoneal implants) is associated with poor prognosis and is defined as M1 disease.

Question 69

Does N+ disease make an esophageal cancer unresectable?

Answer 69

T1–T3 tumors are resectable even with regional nodal metastases (N+), although bulky; multi-station lymphatic involvement is a relative
contraindication to surgery, to be considered in conjunction with age and performance status.

Question 70

What presentations of esophageal cancer are considered unresectable (not including patient fitness)?
4 major scenarios.

Answer 70

• cT4b tumors with involvement of the heart, great vessels, trachea, or adjacent organs including liver, pancreas, lung, and spleen are
  unresectable.
• Most patients with multi-station, bulky lymphadenopathy should be considered unresectable, although lymph node involvement should be considered in conjunction with other factors, including age, performance status, and response to therapy.
• Patients with EGJ and supraclavicular lymph node involvement should be considered unresectable.
• Patients with distant (including nonregional lymph nodes) metastases (stage IV) are unresectable.

Question 71

What should be added to first-line chemotherapy for advanced HER2 overexpression positive esophageal adenocarcinoma?

Answer 71

Trastuzamab

Question 72

Should stents be used to manage dysphagia in patients who are set to undergo neoadjuvant chemoradiation and curative surgery?

Answer 72

Stent placement is generally not advised in patients who may undergo curative surgery or during chemoradiation therapy, due to concerns that stent-related adverse events may preclude curative surgery or increase acute toxicity during chemoradiation therapy.

Question 73

What are palliative options for managing complete esophageal obstruction 2/2 cancer?

Answer 73

• Endoscopic lumen restoration, generally performed via simultaneous retrograde (via a gastrostomy tract) and antegrade endoscopy.
• Establish enteral access for purposes of hydration and nutrition if endoscopic lumen restoration is not undertaken or is unsuccessful.
• Surgical or radiologic placement of J-tube or gastrostomy tube.
• External beam radiation therapy (EBRT).
• Brachytherapy may be considered in place of EBRT if a lumen can be restored that allows for the use of appropriate applicators.
• Brachytherapy should only be performed by practitioners experienced with the delivery of esophageal brachytherapy.
• PDT can effectively treat esophageal obstruction, but is less commonly performed due to associated photosensitivity and costs.
• Chemotherapy.
• Surgery may on occasion be useful in carefully selected patients.

Question 74

What are palliative options for managing severe (but not complete) esophageal obstruction 2/2 cancer?

Answer 74

• Severe esophageal obstruction (able to swallow liquids only).
• Wire-guided dilation or balloon dilation (caution should be exercised when dilating malignant strictures as this may be associated with an increased risk of perforation).
• Endoscopy or fluoroscopy-guided placement of partially or fully covered expandable metal stents.
• There are data suggesting a lower migration and stent occlusion rates with the larger diameter covered expandable metal stents, but an increased risk of other complications such as bleeding and esophago-respiratory fistula.
• If possible, the distal end of the stent should remain above the EGJ to reduce symptoms of reflux and risk of aspiration.
• EBRT and brachytherapy both effectively treat malignant dysphagia.
• The onset of symptom relief for EBRT or brachytherapy is slower compared to endoscopic palliation but is also likely to be more durable.

Question 75

Preferred postoperative systemic therapy for esophageal cancer if preop chemoradiation followed by R0 resection, but residual disease found in resected specimen (tumor or nodal disease)?

Answer 75

Nivolumab.

Question 76

When may neadjuvant immunotherapy be used for esophageal cancer?

Answer 76

MSI-H/dMMR tumors