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Chief Complaint: DYSPHAGIA History of Present Illness 5 months PTA 4 months & 3 weeks PTA • Experienced dysphagia and vomitting after eating solid food • Felt there was a lump in substernal area • No chest pain • Persistence of symptoms with weight loss of 10 lbs. • Consulted a family physician • Omeprazole, domperidone, disflatyl= did not provide any relief 4 months & 1 week PTA 4 months & 2 days PTA • Persistence of symptoms lead him to seek consult at UST OPD GI-MED • Advised to stop previous medication and have an esophagram • Patient was non-compliant • • • • Progressive dysphagia Unable to ingest soft meals Difficulty in drinking fluids Persistence of symptoms+ body weakness= sought consult and was admitted to our institution (medicine) Jan.22- Feb.21 EGD- circumferential, nodular, partially obstructing and friable mass from 35 cm level of esophagus down to the cardia (41cm level) Biopsy of esophageal mass- well differentiated squamous cell carcinoma Biopsy of cardia of stomach esophageal mass- revealled esophageal mucosa with severe dysplasia can’t totally rule out invasive squamous cell carcinoma Endoscopic guided insertion of NGT done CT Scan of chest and upper abdomen= soft tissue mass noted on the esophagus from the distal third up to the gastroesophageal junction causing significant narrowing of its lumen (1/26/09) 35th hospital day=started 1st cycle of radiotherapy and chemotherapy (cisplatin & 5-FU) CT Scan of whole abdomen= circumferential wall thickening in the included distal esophagus and adjacent gastric cardia with thickness ranging from 7-16mm. A solitary lymphadenopathy is seen in the perigastroesophageal region measuring 1.8x1.4 cm. (4/18/09 other hospital) CT Scan of the chest= esophageal new growth involving the middle and lower 3rd of portion with slight regression (5/6/09) 2 months PTA May 10, 2009 • Continued 2nd cycle chemotherapy & 26th session of radiotherapy • Subsequently discharged • Admitted for surgery Past Medical History + for Polio in 1958 at age 3 + for TB in 1980, 3 months treatment 2002, laceration right upper quadrant, sutured without any complications No HPN, DM, allergies, Goiter and Asthma Family History + for colon cancer, sister + asthma- siblings, mother, grandmother + for DM- mother + for PTB- father Personal & Social History 23 pack years of smoking, stopped 3 months now Alcoholic beverage drinker (brandy TID, 1 long neck for 23 years), stopped 5 months now + for substance use- tried few sessions of marijuana and shabu, but denied addiction Review of Systems General: (‐) fever/ sweats/anorexia/ weakness HEENT: (‐) visual dysfunction/redness/ itchiness/ pain/ lacrimation, (‐) deafness/ Hnnitus/ discharge, (‐) bleeding gums/ sores/ fissures/tongue abnormalities/ dental caries, (‐) sore throat/ tonsillitis, (‐) stiffness/ limitation of motion/ masses/ adenopathy/ sensation of lump in the throat Pulmonary: (‐) dyspnea/ shortness of breath/ cough/ sputum production/ hemoptysis/ wheezing/ back pain/ chest wall abnormality Cardiac: (‐) chest pain/ easy fatigability/orthopnea/ nocturnal dyspnea/ palpitations/ syncope/edema/ HPN Vascular: (‐) phlebitis/ varicosities/ claudication Gastrointestinal: (‐) nausea/ vomiting/ GI bleeding/ flatulence Genito‐urinary: (‐) urinary frequency/ urgency/ hesitancy/ dysuria/ hematuria/ nocturia/ urine stream flow abnormality/ flank pain/ stones/urethral discharge/ genital lesions/testicular mass/ perineal pain/ impotence/ vaginal discharge/ abnormal bleeding Musculoskeletal: (‐) joint stiffness/ pain/ swelling/ muscle pain/ weakness Endocrine: (‐) heat‐cold intolerance/ thyroid problems/ polyuria polydipsia polyphagia Psychiatric: (‐) anxiety/ depression/ interpersonal relationship difficulties Physical Exam BP (mm Hg): 90/60 Pulse rate / character: 80bpm, regular Respiratory rate / pattern: 18cpm, regular Temperature (°C): 36.6°C Wt. (kg.): 43.5 Ht. (cm): 158.5 BMI: 17.4 GENERAL SURVEY: conscious, coherent, ambulatory notcardio‐pulmonary distress SKIN: warm, moist dry skin, no active dermatoses HEENT: pale palpebral conjunctiva, anicteric sclera, pupils 2‐3 mm round and ERTL No alar flaring, no naso‐aural d/c. (+) NGT right Nostril. No impacted cerumen, intact tympanic bilateral membrane, nasal septum midline, (‐) tenderness, inflammation (‐) bleeding, ecchymosis (‐) anosmia, (‐) facial asymmetry. Moist buccal mucosa, non‐hyperemic NECK: supple neck, lymph nodes non‐palpable cervical LN thyroid gland not enlarged, no other masses THORAX / LUNGS: symmetrical chest expansion, no retractions, no lagging, equal tactile fremiti, resonant lung fields, breath sounds with expiratory wheeze on both upper lung fields more prominent right CARDIOVASCULAR: adynamic precordium, AB 5th LICS MCL, S1>S2 apex, S2 > S1 at the base, no murmurs All pulses normal ABDOMEN: scaphoid abdomen, (+) 6 cm diagonal scar at RUQ, normoactive bowel sounds, tympanitic on percussion, Traube’s space not obliterated, no direct or rebound tenderness, spleen not palpable (‐) fluid wave, (‐) CVA tenderness MUSCULOSKELETAL: Asymmetric lower extremiHes (leI longer and thinner than the right), (‐) tenderness, (‐) swelling NEUROLOGIC EXAM: normal Salient Features 53 years old Male BMI: 17.4 (N: 18.5-23) Progressive dysphagia to solids and liquids vomiting Body weakness (+) family history of colon CA 23 smoking pack years, stopped 5 months ago Alcoholic Brandy TID 1 long neck for 23 years, stopped 5 months ago (+) substance abuse: marijuana,shabu (-) lymphadenopathies (-)anorexia Salient Features EGD: circumferential, nodular, partially obstructing and friable mass from 35cm level of esophagus down to the cardia (41cm level) Biopsy of esophageal mass Squamous cell carcinoma well differentiated. Biopsy of cardia of stomach esophageal mass Revealed esophageal mucosa with severe dysplasia cannot totally rule out invasive squamous cell cacinoma (well differentiated) Endoscopic guided insertion of NGT CT Scan of chest & upper abdomen soft tissue mass noted in the esophagus from the distal third up to the gastroesophageal junction causing significant narrowing of its lumen Salient Features CT scan of whole abdomen circumferential wall thickening in the included distal esophagus and adjacent gastric cardia, with thickness ranging from 7‐16 mm. A solitary lymphadenopathy is seen in the perigastroesophageal region measuring 1.8 x 1.4 cm. CT scan of chest esophageal new growth involving the middle and lower third of portion with slight regression Esophageal Cancer (Squamous Cell Ca) Dysphagia • Difficulty in swallowing, the primary symptom of esophageal disorders. • Sensation of sticking or obstruction of the passage of food through the mouth, pharynx, or esophagus Harrison’s Principles of Internal Medicine 17th ed. pp 237-239 Dysphagia Mechanical due to large bolus or narrow lumen Motor due to weakness of peristaltic contractions or impaired deglutitive inhibition causing nonperistaltic contractions and impaired sphincter relaxation Harrison’s Principles of Internal Medicine 17th ed. pp 237-239 Dysphagia Mechanical Oropharyngeal Esophageal Motor Oropharyngeal Harrison’s Principles of Internal Medicine 17th ed. pp 237-239 Esophageal Dysphagia Mechanical Oropharyngeal Esophageal Motor Oropharyngeal Harrison’s Principles of Internal Medicine 17th ed. pp 237-239 Esophageal Esophageal Dysphagia Normally can be distended up to 4cm in diameter Dysphagia to solid food <2.5cm Dysphagia to fluids <1.3cm . Harrison’s Principles of Internal Medicine, 17th ed Squamous Cell Carcinoma of the Esophagus Most common type of carcinoma of the esophagus – 90% Age > 50 Most symptomatic tumors are quite large by the time they are diagnosed and have already invaded the wall or beyond 20% -upper third, 50% - middle third, and 30% - lower third of the esophagus Robbins and Cotran Pathologic Basis of Disease, 7th ed. Squamous Cell Carcinoma of the Esophagus Most squamous cell carcinomas are moderately to well differentiated Rich lymphatic network in the sub mucosa promotes extensive circumferential and longitudinal spread Areas of metastasis upper third - cervical lymph nodes middle third - mediastinal, paratracheal, and tracheobronchial nodes lower third - gastric and celiac groups of nodes Robbins and Cotran Pathologic Basis of Disease, 7th ed. Pathogenesis of Esophageal Carcinoma Injury Stratified squamous Epithelium Ulcer Hyperplasia Cell Death Inflammation Gastric Metaplasia Dysplasia Glandular Dysplasia Adenocarcinom a Carcinoma Pathogenesis of Squamous Cell Carcinoma Injury Stratified squamous Epithelium Ulcer Hyperplasia p53 gene mutation Squamous Cell Carcinoma Cell Death Inflammation Dysplasia Carcinoma Clinical Features Insidious in onset Produces dysphagia and obstruction gradually and late Patient progressively alters their diet from solid to liquid foods Extreme weight loss Debilitation Risk Factors Alcohol consumption increases the risk of squamous cell cancer 10 to 25 times Combined cigarette use and alcohol consumption can increase the risk of squamous cell cancer up to 100-fold Ingestion of nitrosamines Contamination of food by specific fungi Temperature of ingested fluids Presence of mechanical irritants to the esophagus Silica Crushed seeds Cancer of the Upper Gastrointestinal Tract by Posner et. al., November 2001 Chronic injury to the esophagus due to: Caustic ingestion Stasis of foodstuffs in patients with achalasia Gastroesophageal acid reflux disease Familial abnormality that is associated with squamous cancer of the esophagus Tylosis A, which carries a 25 percent lifetime risk. Cancer of the Upper Gastrointestinal Tract by Posner et. al., November 2001 Enzinger et al NEJM 2003 DIAGNOSTIC TOOLS OBJECTIVE: To identify and locate the problem, as well as determine the extent of the diseasE 1.) CBC, PT/APTT, Electrolytes, TPAG 2.)12 lead ECG 3.)Spirometry 4.)Chest Xray 5.)CT scan CBC - may show anemia secondary to iron deficiency or chronic disease. PT and aPTT - may demonstrate hepatic insufficiency or nutritional deficiencies; also detects abnormalities in blood clotting Electrolytes – should be obtained to determine imbalances, changes in fluid volume occur preop, intra op and post op Spirometry - measures lung function, specifically the measurement of the amount (volume) and/or speed (flow) of air that can be inhaled and exhaled. 12 lead ECG – a non invasive device that records electrical activity of the heart as well as detects possible abnormalities Chest X-ray – to determine the condition of the heart and other adjacent structures. CT scans - best tool for staging; to exclude the presence of metastases (M staging) to the lungs and liver; determines if adjacent structures have been invaded. Primary tumor Regional Lymph nodes Distal Metastasis • • • • • Tis in situ T1 Lamina propria,submucosa T2 Muscularis propria T3 Adventitia T4 Adjacent strictures • N0 none • N1 Involved • • • • M0 none M1 Distant M1a Cervical/Celiac LN M1b Other distant metastasis (Enzinger et al NEJM 2003) Esophageal Cancer 6th most frequent tumor disease worldwide Characterized by rapid development and fatal prognosis in most cases Occurrence increases with age with the highest incidence in the age group 50–70 years The disease is diagnosed more frequently in males than in females (3:5) Most frequent histological type is squamous cell carcinoma EPIDEMIOLOGY OF ESOPHAGEAL CANCER – AN OVERVIEW ARTICLE Department of Preventive Medicine, Faculty of Medicine, Palacky University Olomouc, Hnevotinska 3, 775 15 Olomouc, Czech Republic Helena Kollarova et al; March 29, 2007; Incidence Esophageal cancer incidence worldwide 462 117 in the year 2002 315 394 cases were diagnosed in males 146 723 cases in females In males, the incidence is approximately three times higher than in females. EPIDEMIOLOGY OF ESOPHAGEAL CANCER – AN OVERVIEW ARTICLE Department of Preventive Medicine, Faculty of Medicine, Palacky University Olomouc, Hnevotinska 3, 775 15 Olomouc, Czech Republic Helena Kollarova et al; March 29, 2007; Cancer of the Upper Gastrointestinal Tract by Posner et. al., November 2001 Cancer of the Upper Gastrointestinal Tract by Posner et. al., November 2001 Cancer of the Upper Gastrointestinal Tract by Posner et. al., November 2001 Mortality Mortality rates represent roughly 90 % of the incidence rates of the disease. EPIDEMIOLOGY OF ESOPHAGEAL CANCER – AN OVERVIEW ARTICLE Department of Preventive Medicine, Faculty of Medicine, Palacky University Olomouc, Hnevotinska 3, 775 15 Olomouc, Czech Republic Helena Kollarova et al; March 29, 2007; Philippines: Mortality 1998 Male 252 per 100 000 (0.7%) Females 139 per 100 000 (0.4%) from WHO www.who.int; 1998 Squamous Cell Carcinoma Squamous cell cancers represent the single most common malignancy of the esophagus worldwide. Endemic areas for squamous cell cancer of the esophagus Northern littoral in Iran Linxian, China Regions of South Africa, where the incidences are as high as 150 cases per 100,000 population. Cancer of the Upper Gastrointestinal Tract by Posner et. al., November 2001 In the United States Incidence rate of squamous cell cancers is about 3 per 100,000 population, Mortality: 12,000 deaths from squamous cell esophageal cancer in 1998. Men are more commonly affected than are women Highest incidence occurs during the sixth through eighth decades of life Cancer of the Upper Gastrointestinal Tract by Posner et. al., November 2001 Cancer of the Upper Gastrointestinal Tract by Posner et. al., November 2001 Complications Weight Loss Nutritional Deficiencies Dysphagia Solid food impaction Severe stenosis Requires endoscopic intervention for disimpaction. Regurgitation of food or oral secretions Significant luminal obstruction Halitosis Food stasis Regurgitation American Medical Network: Esophageal Cancer; James C. Chou et.al Pulmonary complications from aspiration Pneumonia Pulmonary abscess The tumor mass Compression Obstruction of the tracheobronchial tree Leading to dyspnea, chronic cough, and at times postobstructive pneumonia. Esophagoairway fistula may develop with tumor invasion of the trachea or bronchus. Airway fistulas are severely debilitating and are associated with significant mortality owing to the high risk of pulmonary complications such as pneumonia and abscess. TREATMENT 1. Surgical Management (curative) Treatment of Choice for early cancer primary goal is complete resection of tumor and involved lymph nodes anyone with disease up to T3 N1 must be used with other management to improve survival esophagectomy: approaches include transthoracic, transhiatal, transoral route Radical Resection - Surgical resection that takes the blood supply and lymph system supplying the organ along with the organ. thorascopic tools, laparoscopic tools gastric/colonic mobilization Radiation Therapy The medical use of ionizing radiation as part of cancer treatment to control malignant cells Radiotherapy may be used for curative or adjuvant cancer treatment May be used as the primary therapy. Radiation therapy works by damaging the DNA of cells. The damage is caused by a photon, electron, proton, neutron, or ion beam directly or indirectly ionizing the atoms which make up the DNA chain Chemotherapy Treatment of cancer through Chemicals Refers to antineoplastic drugs used to treat cancer or the combination of these drugs into a cytotoxic standardized treatment regimen. Chemotherapy acts by killing cells that divide rapidly, one of the main properties of cancer cells. Most chemotherapeutic drugs work by impairing mitosis It also harms cells that divide rapidly under normal circumstances which results in the most common side-effects of chemotherapy. Some drugs cause cells to undergo apoptosis or programmed cell death 2. RADIATION & CHEMOTHERAPY CURABLE DISEASE Combined is superior to radiation alone Achieved overall survival rates that equal or exceed those of historical surgical cohorts (though no trials comparing them) Cisplatin and fluorouracil Radiation with chemotherapy 75% local control rate : improve swallowing 30% actuarial disease free survival rate 18% overall survival rate High Morbidity from adverse effects 3. Neoadjuvant therapy Preoperative radiation and chemotherapy then resection PALLIATIVE THERAPY Most patient with esophageal cancer have advanced stage at time of initial medical consultation <20% survive in 1st year Goal of Palliation: improvement of dysphagia Pain Management PALLIATIVE THERAPY DISPLACEMENT THERAPY ABLATIVE THERAPY Dilation therapy Contact thermal Stenting Noncontact Thermal Cytotoxic injection Photodynamic therapies DILATION STENT PALLIATIVE THERAPY Bleeding and esophageal fistula are the most common adverse effects No improvement of pain and anorexia Esophageal stent placement can well manage fistulas from primary malignancy Enteral Nutrition Enteral feeding when feasible Attempt to improve functional status before and after surgery, during chemoradiation Oral route: precluded by anorexia, gastric dysmotility, and generalized debilitation Surgical jejunostomy Prognosis The prognosis of esophageal cancer is generally unfavorable. Long-term survival is only approximately 5 % of patients. Of patients who undergo radical esophagectomies,10–20 % survive 5 years. In patients with inoperable cancer, the median survival is 13–29 months. EPIDEMIOLOGY OF ESOPHAGEAL CANCER – AN OVERVIEW ARTICLE Department of Preventive Medicine, Faculty of Medicine, Palacky University Olomouc, Hnevotinska 3, 775 15 Olomouc, Czech Republic Helena Kollarova et al; March 29, 2007;