RTC Dec 11 2009 Felicitas Koller and Eric Grogan Cancer Statistics, United States

New Cases 200000 Deaths

150000

100000

50000 Number of patients of Number 0 L B P C E un re ro olo so g ast st r p at ec hage e tal al

Histology http://www.cancer.org/statistics Squamous Cell Adenocarcinoma Worldwide >>> < Men vs. 3:1 (fifth decade) 15:1 (fifth decade) Women

Age Rarely <30; mortality greatest in men Rarely < 40, incidence increases with age 60‐70

Disease Exposure to environmental factors; Barrett’s 40 fold increase Association smoking, ETOH, pickled/smoked; deficiencies; tylosis, Plummer‐Vinson syndrome; end‐stage achalasia

Location Squamous in upper and middle Submucosal glands of ; third heterotopic islands of columnar epithelium; malignant degeneration of metaplastic columnar epithelium

Survival 70% with polypoid; 15% advanced Nature Sensitive to chemotherapy Embedded in Barrett's esophagus, less sensitive to chemotherapy

ƒ Commences at C6 joins at T11, usually 25‐30 cm long. 2.5 cm in diameter

ƒ Structures: distance from incisors ƒ 13 cm – cricopharyngeus ƒ 22 cm azygous vein ƒ 22‐27cm carina ƒ 40 cm EG junction The Layers at??

The esophagus lacks a serosa: mucosa, submucosa and muscularis propria

Two interconnecting lymphatic plexuses arising from the submucosa and muscularis layers. GE junction at?? Endoscopically: The squamocolumnar epithelial junction (Z‐ line)

The transition from the smooth esophageal lining to the rugal folds of the stomach

Externally: The collar of Helvetius (or loop of Willis) The gastroesophageal fat pad are consistent identifiers of the GEJ

Case Presentation 3/24/09 CC: 73 yo man with a 2 month history of progressive difficulty with swallowing. Solid foods pass the upper esophagus with some difficulty. Has no lodging, but has to drink more water No hx of or stricture HTN, HLD, BPH, hx. of arrhythmia PSH: Anterior neck fusion WHAT ADDITIONAL HISTORY? Smoking, drinking, anemia, GERD or GERD sx, foul breath, regurgitation of food, hx of chemical or radiation exposure, medications

DIFFERENTIAL DX? Obstructive lesions: tumors, inflammatory masses; Zenker's , esophageal webs mediastinal masses ,cervical spondylosis, radiation, chemical or medication induced; Schatzki's Ring, vascular compression, enlarged aorta or left atrium, aberrant vessels, lymphadenopathy, substernal thyroid

Neuromuscular disorders: achalasia; DES, Hypertensive LES, nutcracker esophagus, scleroderma

INITIAL TEST? Barium Swallow‐Why? both functional and anatomic. A

B

C D 1. A=nutcracker esophagus, B=cancer C=achalasia, D= cancer 2. A=Schatzki’s ring, B=cancer, C=normal, D=Barrett’s esophagus 3. A=reflux, B=compression from mediastinal mass, C= normal, D=achalasia 4. A=diffuse , B=Zenker’s diverticulum, c=normal, d=cancer

ƒ EGD with biopsy—tissue 1. Location (w/ respect to incisors) 2. Nature of the lesion 3. Proximal and distal extent of the lesion 4. Relationship of the lesion to the cricop., GEJ, cardia 5. Distensibility of the stomach ƒ CT scan thorax—length of the tumor, thickness of the esophagus and stomach, lymph node status and distant disease to the liver and lungs ƒ PET scan—primary mass, regional lymph nodes and distant disease ƒ Esophageal ultrasound—depth of the tumor, the length of the tumor, the degree of luminal compromise, the status of regional lymph nodes, and involvement of adjacent structures ƒ EGD: ‐6‐7 mm, 3cm long, 28 cm from the incisors. Patchy antral erythema, normal

ƒ Pathology: invasive moderately differentiated squamous cell carcinoma, vascular invasion

ƒ CT: focal mid‐esophageal wall thickening, no adenopathy

ƒ PET: intense focal FDG activity in the mid‐thoracic esophagus immediately posterior to the left mainstem bronchus. 3 FDG avid nodules: r of midline at the same level, subcarinal and right paratracheal 1ST hyperechoic (white), represents the superficial mucosa (epithelium and ).

2nd hypoechoic (black), represents the deep mucosa (muscularis mucosa).

3rd hyperechoic (white), represents the submucosa.

4thhypoechoic (black), represents the muscularis propria.

5th hyperechoic (white) is the periesophageal tissue.

A, A T1 lesion is observed as a hypoechoic thickening of the mucosal layer adjacent to the normal‐appearing wall pattern; B, a T2 lesion is seen as a hypoechoic mass invading into but not through the muscularis propria

ƒ T2 N1 by EUS ƒ What’s his stage ƒ What are his treatment options Tumor Nodes Metastasis TX: Primary tumor cannot be NX: Regional lymph MX: Distant metastasis cannot be assessed assessed nodes cannot be assessed

T0: No evidence of primary tumor N0: No regional lymph M0: No distant metastasis node metastasis

Tis: Carcinoma in situ N1: Regional lymph M1: Distant metastasis Tumors of the lower thoracic esophagus: node metastasis M1a: Metastasis in celiac lymph nodes M1b: Other distant metastasis

Tumors of the midthoracic esophagus: M1a: Not applicable M1b: Nonregional lymph nodes and/or other distant metastasis Tumors of the upper thoracic esophagus: M1a: Metastasis in cervical nodes

M1b: Other distant metastasis

T1: Tumor invades lamina propria or submucosa

T2: Tumor invades muscularis propria

T3: Tumor invades

T4: Tumor invades adjacent structures

Stage 0 Stage 1 Stage 2 Stage 3 Stage 4 Tis, N0, M0 T1, N0, M0 Stage IIA T3, N1, M0 Any T, any N, M1 T2, N0, M0 T4, any N, T3, N0, M0

Stage IIB Stage IVA T1, N1, M0 Any T, any N, T2, N1, M0 M1a

Stage IVB Any T, any N, M1b

1. alone 2. Surgery adjuvant chemo 3. Chemoradiation alone 4. Neoadjuvant chemoradiation and then surgery Case Presentation 4/2009‐6/2009 Patient receives neoadjuvant chemotherapy (cisplatin and capecitibine) as well as radiotherapy.

Repeat PET imaging shows : 1.Decreased LN size with loss of FDG activity. 2.In the middle esophagus right below the carina, moderate FDG activity is seen associated with residual esophageal wall thickness. On previous scan, this was primary site of the cancer with intense FDG uptake and significant esophageal wall thickness.

EGD: 1.UES was located at 19‐cm from insertion, and the GE junction was located at 41‐cm from insertion.

2.31‐cm from insertion there was a partially‐circumferential superficial ulcer with tissue. There were proximal esophageal plaques consistent with candidal esophagitis.

There was no Barrett's. ‐ Stomach: normal ‐ Duodenum: normal

ƒ Transhiatal

ƒ Transthoracic (Ivor –Lewis)

ƒ Three field (McKeown)

ƒ Minimally invasive ƒ Approach / Incisions ƒ Anastamosis ƒ Transhiatal ƒ Neck ƒ Transthoracic ƒ Chest ƒ Minimally invasive

ƒ Conduit ƒ Route ƒ Stomach ƒ Posterior mediastinum ƒ Colon ƒ , ƒ Retrosternal supercharged ƒ Subcutaneous ƒ Other Case Presentation JI underwent operation 7/28/09: 1. Bronchoscopy. 2. Esophagogastroduodenoscopy. 3. Laparotomy with transthoracic esophagectomy. 4. Jejunostomy feeding tube. 5. Ligation of thoracic . 6. Pyloroplasty

Pathology: 1) ESOPHAGUS AND STOMACH, DISTAL ESOPHAGECTOMY: LIMITED RESIDUAL SQUAMOUS CELL CARCINOMA, DISTAL ESOPHAGUS, IN SUBMUCOSA AND MUSCULARIS PROPRIA, 1.8 MM IN GREATEST EXTENT; PRESENT IN A BACKGROUND OF SEVERE MUCOSAL AND SUBMUCOSAL ULCERATION; ALL SURGICAL MARGINS WIDELY FREE OF CARCINOMA; TWO LYMPH NODES NEGATIVE FOR MALIGNANCY (0/2), (SEE COMMENT).

2) LYMPH NODES, LEVEL 7, EXCISION: 3 LYMPH NODES NEGATIVE FOR MALIGNANCY (0/3).

COMMENT: These findings correspond to AJCC pathologic stage yIIA (ypT2N0M n/a).

ƒNo difference in operative time, blood loss, morbidity or mortality

ƒSurvival similar

ƒAnastomotic Leak rate ƒ Cervical 11% ƒ Thoracic 6%

Putnam et al., Ann Thor Surg, 1994

ƒ Identified endoscopically

ƒ Resection, mediastinal debridement

ƒ Diversion, delayed reconstruction

ƒ 50% mortality ƒ Smoking cessation ƒ Enteral nutrition in pts undergoing induction tx ƒ Epidural anesthesia ƒ Bronchopulmonary hygiene ƒ Early extubation ƒ No difference between TTE and THE • Incidence 1‐3% (TTE=THE)

• Suspected with high continued CT output

• Pleural triglycerides, lymphocytes, chylomicrons

• Most will not close with conservative management ƒ Non‐operative stratagies ƒ NPO, TPN ±octreotide ƒ MCT diet ± octreotide

ƒ IR embolization of thoracic duct

ƒ Operative ligation ƒ VATS vs. open