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Tumor of Trachea and Esophagus
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Tracheal neoplasm
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Overview Tracheal anatomy Primary tracheal tumors
Benign primary tracheal tumors Malignant primary tracheal neoplasms Secondary tracheal tumors
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Anatomy: Trachea Trachea เป็นอวัยวะที่อยู่แนวกลางตัว ยกเว้นส่วนปลายที่จะเอียงไปทางด้านขวา จะมีรอยเว้า 2 แห่ง เนื่องจากมีรอยกดของ aortic arch ทางด้านซ้าย และ arch of azygos veinทางด้านขวา Trachea wall ประกอบด้วย horseshoe-shaped cartilages อัน เชื่อมติดกันด้านหลังด้วย thick fibromuscular membrane เส้นผ่าศูนย์กลาง 19.5 ม.ม ในผู้ชาย และ 17.5 ม.ม ในผู้หญิง
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Anatomy: Trachea Fibromuscular tube Support by cartilagenous rings
Lower border of cricoid cartilage – top of the carina spur Average length 11 cm (10-13 cm) Adult trachea cm in diameter Extrathoracic trachea ~ 6-9 cm Trachea, Anatomy 1 ประกอบด้วย fibromuscular และ cartilaginous tube มีความยาว เซนติเมตร เริ่มต้นจาก cricoid cartilage ในคอจนถึง bifurcation ที่เรียก tracheal carina Trachea เป็นอวัยวะที่อยู่แนวกลางตัว ยกเว้นส่วนปลายที่จะเอียงไปทางด้านขวา จะมีรอยเว้า 2 แห่ง เนื่องจากมีรอยกดของ aortic arch ทางด้านซ้าย และ arch of azygos veinทางด้านขวา Trachea เมื่อเข้ามาในส่วนของทรวงอกได้ประมาณ 6-9 ซ.ม จะแบ่งออกเป็น Bronchi 2 อัน Right main bronchus จะยาวประมาณ 2.2 ซ.ม และ Left main bronchus จะยาวประมาณ 5 ซ.ม Right main bronchus จะมีเส้น ผ่าศูนย์กลาง 15.3 ม.ม และ Left main bronchus จะยาวมีเส้นผ่าศูนย์กลาง 13 ม.ม
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C shaped with posterior membranous wall connecting the arms of the “C”
Mucosa : a ciliated pseudostratified columnar epithelium
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Anatomy: Blood supply to trachea
Branches of inferior thyroid artery supply the upper trachea Branches of bronchial artery supply the lower trachea Branches arrive the trachea via lateral pedicles
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Tumor of trachea Tracheal tumor 2 type - primary tracheal tumor
- secondary tracheal tumor
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Tracheal resection with end to end anastomosis
Tracheal mobilization maneuvers Extreme flexion of the neck (1-6 cm) Incising the annular ligament (1-2 cm) Suprahyoid or infrahyoid release of the upper laryngotracheal unit (2.5-5 cm) Blunt dissection and mobilization of the lower tracheal segment (0.5-1 cm) All combinations yields 4-6 cm (~ patient age and range of neck motion)
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Primary Tracheal Tumors
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Primary Tracheal Tumors
Uncommon Incidence 2 case/million/year Men = Women Peak age : yr Risk factor : Smoking In adults : Malignant >80% In children : Benign > 90 % Most frequent : proximal and distal 1/3 of trachea Originate from any layer in tracheal wall Classified : - Epithelial tumors - Mesenchymal tumors Primary tumor can originate from any layer in the tracheal wall Cummings, 5 th ed.
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Classification of Tracheal Tumors
Epithelial Neoplasms Benign Squamous cell papilloma Papillomatosis Pleomorphic adenoma Malignant Squamous cell carcinoma Adenoid cystic carcinoma Carcinoid Mucoepidermoid carcinoma Adenocarcinoma Small-cell undifferentiated carcinoma Secondary malignancy Invasion by adjacent malignancy Metastases Nonneoplastic tumors Tracheobronchopathia osteochondroplastica Amyloidosis Inflammatory pseudotumor Mesenchymal Neoplasms Benign Fibroma Hemangioma Granular cell tumor Schwannoma Neurofibroma Fibrous histiocytoma Pseudosarcoma Hemangioendothelioma Leiomyoma Chondroma Chondroblastoma Lipoma Malignant Leiomyosarcoma Chondrosarcoma Paraganglioma Spindle-cell sarcoma Lymphoma Malignant fibrous histiocytoma Rhabdomyosarcoma
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BENIGN PRIMARY TRACHEAL TUMORS
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Benign primary tracheal tumors
Uncommon in adult Usually smooth, well circumscribe, round, soft, and small < 2 cm Chest CT Not extend through the tracheal wall May presence of calcium within the lesion benign histology Cummings, 5 th ed.
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Benign primary tracheal tumors
Squamous papilloma Granular cell tumor Chondroma Leiomyoma Hemangioma
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Squamous Papilloma Superficial, sessile or papillary masses consisting of a connective tissue core covered by squamous epithelium Adult : rare : associate with heavy smoking Children : most common tracheal neoplasm Cause : HPV 6, 11 Cummings, 5 th ed.
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Squamous Papilloma Cause : transmitted from mother to fetus during childbirth Frequent recurs and difficult to completely eradicate Usually regresses spontaneously after puberty Major lesion occur isolated to the larynx (90-95%) - 11% occur in the trachea in addition to the larynx - 1.2 % isolated to the trachea
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Squamous Papilloma Treatment : similar laryngeal papilloma
- recent : CO2 laser : Adjuvant treatment with α-interferon Malignant transformation to SCCA or Verrucous CA - incidence of malignant degeneration % - associated HPV -11
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Granular cell tumor Neurogenic in origin , from schwann cell
No sex predilection Children : rare 50% of granular cell occur in the head and neck region - 10 % in the larynx - rare : in the cervical trachea
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Granular cell tumor 20% Multicentric tumor, more aggressive
Finding : Non-encapsulated, : tend to invade locally Malignant degeneration 1-2% (never report in children)
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Granular cell tumor Management : surgery - Tumor size < 8 mm :
“ Bronchoscopic resection ” - Tumor size > 8 mm high likelihood of full-thickness wall involvement recurrent after bronchoscopic removal “ Segmental tracheal resection ”
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Chondroma Most common benign mesenchymal tracheal tumor
Cartilaginous origin Most common site - internal aspect of the posterior cricoid lamina Hard , smooth , broad-based and covered by intact mucosa Radiography : 75% found calcification, not distinguish from chondrosarcoma
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Chondroma Management : - surgery segmental tracheal resection
- endoscopic resection for palliation but leads to recurrence
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Leiomyoma Origin : smooth muscle of tracheal wall , typically from the membranous portion of the lower third of the trachea Finding : Smooth contoured , polypoid mass and usually have a broad base
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Leiomyoma Management : surgery - segmental tracheal resection
- incomplete resection local recurrence
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Hemangioma Hemangioma of the airway occur in adults and children
- cavernous hemangiomas develop in the larynx - capillary hemangioma originate in subglottic area
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Hemangioma Tracheal Hemangioma occur more often in young children and most common obstructive subglottic mass Asymptomatic at birth, but most will cause stridor within the first 6 months of life Cutaneous hemangioma 50% of patient
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Hemangioma Finding : capillary hemangioma
- Submucosal mass with covered by normal respiratory epithelium - Site : posterolateral aspect of the subglottic trachea - smooth , bluish in color , and project into the airway from sessile base
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Hemangioma Management - Spontaneous regress by 2-3 yr of age
Close observation is usually advocated - In symptomatic patient : radiation , steriod , laser ablation
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Miscellaneous benign primary tumor
Adenomas Myoepithelial cell tumors Lipomas Fibromas Schwannomas Neurilemomas Hemangiomas Paragangliomas Fibrous histiocytomas Neurofibromas Chrondoblastomas Benign mucoepidermoid tumors Angiofibromas Xanthomas Myoblastomas Hamartomas Glomus tumors Intratracheal goiters Chemodectomas All combined, these tumors represent fewer than 10% of all primary tracheal tumors.
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Miscellaneous benign primary tumor
Treatment - surgical excision - may be successfully managed with endoscopic resection
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Malignant primary tracheal neoplasms
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Malignant Primary Tracheal Neoplasms
In adults The majority of primary tracheal tumors are malignant Typically, malignant lesions shorter duration of symptoms than benign lesions larger in size irregular surface 75% of all primary malignant tracheal neoplasms are squamous cell carcinoma or adenoid cystic carcinoma
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Malignant Primary Tracheal Neoplasms
Historically, more common in men than women squamous cell histology was most prevalent Recently, incidence of squamous cell carcinomas has declined incidence of adenoid cystic carcinomas has increased male = female
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Malignant Primary Tracheal Neoplasms
The most frequent presenting symptoms are cough (72%) dyspnea (66%) hemoptysis (39%) stridor (39%) hoarseness (31%)
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Squamous Cell Carcinoma
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Squamous Cell Carcinoma
After 1980, incidence is decreased significantly Men : Women = 2:1 Mean age is 60 years old Hx of cigarette smoking : 90% Synchronous or metachronous respiratory tract malignancies occur in 30% Sputum cytology : 50 % of pts are positive for cancer When SCCA involve the membranous tracheal wall Invasion from a primary esophageal tumor should always be considered
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Squamous Cell Carcinoma
Exophytic growth with ulceration Aggressive and tend to growth rapidly Extend longitudinally & circumferentially Localized or multiple scattered Often involved at the lateral tracheal wall
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Squamous Cell Carcinoma
Occur at any level of trachea or carina Penetrate extraluminal : Adjacent structure may be invade directly
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Squamous Cell Carcinoma
Metastasize early Adjacent peritracheal lymph node metastasis Less common hematogenous metastases Bone (most often) Lung Liver Adrenal
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Squamous Cell Carcinoma
Pathology Nest and sheet of squamous cell with various degree of differentiation Scant cytoplasm High nuclear to cytoplasm ratio
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Squamous Cell Carcinoma
Treatment : Complete resection Radiation therapy -> palliate patients with unresectable SCCA Resectable : median survival 38 months , 5-year survival rates 39.1% Unresectable : median survival 8.8 months , 5-year survival rates 7.3% Mediastinal LN involvement -> prognostic implication following Sx 5-year survival : negative mediastinal LN 48.4% and positive LN 12.5%
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Adenoid Cystic Carcinoma
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Adenoid Cystic Carcinoma
“Cylindromas” Seen in all age groups Peak age incidence years old Usually arise in the trachea or main bronchi No associated with sex, race, or cigarette smoking
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Adenoid Cystic Carcinoma
Nonencapsulated, slow-growing, low-grade malignant tumors Originate from the epithelium of the glands lining the mucosa of the respiratory tract Perineural, submucosal, and distant metastatic spread Lymphatic metastases uncommon (13% to 19%) Hematogenous metastases 44% Metas occurs most often to lung and bone
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Adenoid Cystic Carcinoma
Push mediastinal structures aside rather than invade them
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