Tumor of Trachea and Esophagus Tracheal neoplasm Overview Tracheal anatomy Primary tracheal tumors Benign primary tracheal tumors Malignant primary tracheal neoplasms Secondary tracheal tumors Anatomy: Trachea 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 2-2.5 cm in diameter Extrathoracic trachea ~ 6-9 cm C shaped with posterior membranous wall connecting the arms of the “C” Mucosa : a ciliated pseudostratified columnar epithelium 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 Tumor of trachea Tracheal tumor 2 type - primary tracheal tumor - secondary tracheal tumor 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) Primary Tracheal Tumors Primary Tracheal Tumors Uncommon Incidence 2 case/million/year Men = Women Peak age : 50- 59 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 Cummings, 5 th ed. 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 BENIGN PRIMARY TRACHEAL TUMORS 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. Benign primary tracheal tumors Squamous papilloma Granular cell tumor Chondroma Leiomyoma Hemangioma 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. 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 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 1.6-4.0 % - associated HPV -11 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 Granular cell tumor 20% Multicentric tumor, more aggressive Finding : Non-encapsulated, : tend to invade locally Malignant degeneration 1-2% (never report in children) 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 ” 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 Chondroma Management : - surgery segmental tracheal resection - endoscopic resection for palliation but leads to recurrence 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 Leiomyoma Management : surgery - segmental tracheal resection - incomplete resection local recurrence Hemangioma Hemangioma of the airway occur in adults and children In adult - cavernous hemangiomas develop in the larynx - capillary hemangioma originate in subglottic area 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