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1. Respiratory Tract
Tissue examination
Most lesions are peripheral
Radiographic features- major diagnostic aid
Calcification
"Popcorn" type
Well defined margins
Lobulated
Growth (?)
Wedge resection
Frozen section
2. Epithelial tumors
Mesodermal tumors
Vascular tumors
Bronchial tumors
Neurogenic tumors
Developmental or unknown origin tumors
Inflammatory and other pseudo0tumors
3. Tumors of Epithelial origin
Papilloma- 5 sib-classifications
Solitary benign papilloma
Multiple benign papillomas
Combined bronchial mucous gland and surface papillary tumor
In situ papillary bronchial carcinomas
Bronchiolar papillomas
Proximal
Squamous, stalk
Distal
Clara cells
One of few lesions that can be managed by bronchoscopic resection
Recurrence is high
Rare malignant transformation
4. Polyps
Related to inflammatory polyps or URT
Squamous metaplasia; ciliated columnar epithelium and granulation tissue
Bronchoscopic removal
Tumorlet
Epithelial proliferation
Originates from Kulchitsky cells
Multifocal: < 4 mm; associated with pulmonary fibrosis
Form of peripheral carcinoid with rare metastases
5. Tumors of Mesodermal Origin
Hemangioma
Subglottic area of larynx or upper trachea of infants
Airway obstuction
Dx: Bronchoscopy
Other vascular lesions of skin, mucous membranes
Tx: Radiation therapy
Lymphangioma
Upper airway obstruction in infancy
Associated with other lesions- cystic hygroma, hemangioma in the neck
Tx: surgical excision
6. Endothelioma
More solid
Lymphatic or vascular origin
Angiomatous formation- may ne found only in lung
Most often described in newborns
Lethal course over a short period or time
Lymphangiomatosis
Rare- may be related to tuberous sclerosis
Slowly progressive- death within 10 years from pulmonary insufficiency
Young females presenting with dyspnea
Spontaneous PTX; chylothorax, hemoptysis
CXR: fine, multinodular lesions in bases- honeycombing
7. A-V Fistula
Lower lobes
Associated with hemorrhagic telangiectasia (Rendu-Osler-Weber Syndrome) in 50% of cases
R > L shunting PA-PV
Cyanosis
Clubbing
Polycythemia
DOE
Pulmonary murmur
Brain abcess or peripheral embolization
Dx: CXR,; pulmonary angiogram
Tx: Surgical resection; angiographic embolization A-V Fistula
8. Sclerosing Hemangioma
Uncommon; originates from Type II pneumocyte precursors
Coin lesion, lower lobes
Hemoptysis
Associated with hemorrhagic telangiectasia
Intravascular Bronchoalveolar Tumor
Rare; females < 30 years old
Originates from vascular endothelium
CXR: multiple pulmonary nodules- incidentally discovered
Mild dyspnea
Stable, slowly growing metastases reported
Tumor considered to be malignant
9. Hemangiopericytoma
Solitary, encapsulated, asymptomatic
Originates from pericytes associated with pulmonary capillaries
Considered malignant
Tx: Surgical resection for cure or radiation therapy for palliation
10. Fibroma
Mostly tracheobronchial in origin
Most common benign tumor of mesodermal origin in adult and pediatric age group
Collagenous/ spindle cells- myxomatous/ adipose elements
Tx: Bronchoscopic resection if stalk is present vs conservative pulmonary resection
11. Chondroma and Osteochondroma
Arise in major bronchi
May appear as true enchondrosis of bronchial cartilage
Firm, translucent; ossified/ calcified material
Second most common mesodermal tumor
Carney's triad: associated with extra-adrenal paragangliomas and gastric leiomysarcomas
12. Lipoma
Rare, intrabronchial lesion; male predominance
Slowly growing, avascular, obstructive, pedunculated
Tx: Bronchoscopic removal for small lesions; bronchotomy for larger ones
Arise in fat cells
Associated with bronchiectasis (chronic obstruction)
13. Leiomyoma
Fourth in order of frequency
Originates from smooth muscle cells
Bronchiolar smooth muscle cells
Pulmonary vessels
Sx: cough, hemoptysis, pneumonia, bronciectasis
50% intrabronchial- 50% peripheral
Can be associated with similar cutaneous lesions
Tx: Conservative surgical approach
Malignant variant: Leiomyosarcoma
14. Order of Frequency of Mesodermal Tumors
Fibroma
Chondroma
Lipoma
Leiomyoma
15. Granular Cell Tumors (Myoblastoma)
Previously thought to originate from myoblasts
Originates from Schwann cells or histiocytes
Arises from the tongue or skin
6% originate endobronchially
Tx: Surgical removal with wide margins
Bronchoscopic removal associated with recurrence < 8 mm
16. Developmental or Unknown Origin
Hamartomas
Most common benign tumor of the lung
8% of coin-shaped lesions
0.25% of general population
CXR: "Popcorn" lesion
Malignant transformation- rare
True neoplasm
Malignant transformation of the epithelial component
Chondromatous hamartoma
Adenomatous hamartoma of infancy
Pulmonary blastoma
17. Teratoma
Rare: 50% are benign
Characteristics of all germ layers
Must be differentiated from metastatic lesion
Testicle
Direct invasion from mediastinum
18. Chemodectoma
One reported case
Arise from chemorecptor cells in the lung
19. Clear Cell (sugar) Tumor
Resembles hypernephroma
Abundant glycogen content
Characteristics of Type II glycogen storage disease
20. Thymoma
Ectopic thymus tissue (rare)
Intrapulmonary thymoma may be associated with Myasthenia Gravis
21. Inflammatory and Other Pseudotumors
Plasma Cell Granulomas (Histiocytoma)
Solitary pulmonary nodule
May be associated with: systemic disease, plasma protein imbalance, NS local inflammatory reaction
Micro: Plasma cells and lymphocytes
Local exclusion/ lobotomy- cure
May be associated with multiple myeloma or other malignancies
Do not confuse with plasmacytoma- a malignant lesion
22. Pseudolymphoma
Discrete localization
Unilateral
Resembles lymphoid interstitial pneumonitis
Rare conversion to malignant lymphoma
Tx: Lobectomy/ segmental resection with follow-up
23. Xanthoma
Post-inflammatory lesion- encapsulated
Micro: foam cells
Conservative resection
No reports of recurrence
24. Amyloid
Deposition may be diffuse of localized
Occasionally may be obstructive
Tx: Bronchoscopic removal/ resection
25. Tracheobronchopathia Osteoplastica
Multiple cartilaginous/ osseous projections into lower tracheal lumen
Usually found at autopsy
Rarely obstructive
Tx: Bronchoscopic removal/ tracheal resection
Bronchial Adenomas
26. Most are not benign adenomas but are malignant neoplasms
Long natural histories
5% of all primary pulmonary neoplasms
Two groups
Carcinoid tumors
Bronchial mucous gland tumors
27. Classification
Carcinoid tumors
Typical
Atypical
Bronchial gland tumors
Adenoid cystic carcinoma (Clindroma)
Mucoepidermoid carcinoma
Bronchial gland adenoma
28. Carcinoid Tumors
85-90% or bronchial adenomas
Neuroendocrine tumor from Kulchitsky's cells (argentaffin)
APUD tumor
Mostly in lobar bronchi (70%); others quote higher incidence in periphery or lung
More frequent on right and in lower lobes
"Mulberry" lesion
Small cell uniform tumor cells in a vascular stroma
Atypical carcinoid 10-15%
Pleomorphism
Nuclear irregularity
Increased mitotic activity
29. Atypical carcinoid
More active biologic behavior
50-60% lymph node metastases (5-10% with typical carcinoids)
10% distant metastases
Poorer prognosis
Most patients- 5th to 6th decade; slight female predominance
25-30% asymptomatic- present with abnormal CXR
Sx: Cough, hemoptysis, recurrent pneumonitis
Carcinoid syndrome- 1-2%; usually associated with metastases
Cushing's syndrome; hyperinsulism
60-80% seen bronchoscopically: Biopsy (?)
30. Treatment
Resection: lobectomy, pneumonectomy
Resect recurrent and metastatic disease
Bronchoscopic resection- high rate of recurrence
Ten year disease-free interval
Typical carcinoids- 85-90%
Atypical carcinoids or typical carcinoids with lymph node metastases- 50%
31. Bronchial Gland Tumors
Adenoid Cystic Carcinoma (Cylindroma)
Occurs in trachea
30% lymph node metastases
Submucosal and perineural lymphatic spread
Sx: cough, hemoptysis, wheezing, obstructive pneumonitis in 6th decade
Tx: wide surgical excision with lymph node dissection- RT palliative
50% long-term survival with wide surgical resection
32. Mucoepidermoid Carcinoma
Epidermoid and mucous producing elements
Behavioral correlates with microscopic appearance
Tx: Complete resection
Prognosis
Low-grade: good with adequate resection
High-grade: poor despite therapy
Rare; mainly in lobar/ mainstream bronchi
33. Mixed Tumors
Rare; similar to salivary gland tumors
Require tracheal reconstruction
Bronchial Mucous Gland Adenomas
Rare; benign; conservative resection
Extended Outline
Benign Tumors of the Lung
1. 5% of all tumors of the tracheobronchial tree
2. most are within lung parenchyma
3. usually asymptomatic unless associated with bronchial obstruction
Classification of benign tumors of the lung and tracheobronchial tree (see chart)
Clinical features
1. mode of presentation depends on location and size
2. most are peripheral and found on routine CXR
3. chronic cough and chest pain are most frequent symptoms (40%)
4. central lesions are more likely to be symptomatic
5. diagnosis
a. CXR, CT scan
b. bronchoscopy for central lesions
c. peripheral lesions- needle biopsy, thoracoscopy, open biopsy
d. conservative resection is indicated unless there is a surrounding destructive process
Tumors of epithelial origin
Papillomas of the tracheobronchial tree
1. most common laryngeal tumor in children
2. rare in adults
3. single or multiple lesions
4. subclasses
a. solitary benign
b. multiple benign
c. benign combined with bronchial mucous gland and surface papillary tumors
d. papillary bronchial CIS
e. bronchial papillomas
5. Approximately 50% of solitary bronchial papillomas are associated w/ lung carcinoma
Tumors of Mesodermal Origin
Hemangioma
1. infrequent in lung, more common in trachea and mainstem bronchi
2. multiple one third of cases
3. 60% of cases are associated with generalized hereditary telangiectasia (Oler-Rendu-Weber disease
4. surgical treatment is conservative resection, YAG laser for endobronchial lesions
Lymphangioma
Hemangioendothelioma
Hemangiopericytoma
1. may arise anywhere in the body
2. lesions are large and 50 % malignant
3. originate from capillary endothelium
4. centrally located, highly vascular
5. treatment is conservative surgical resection unless malignant
Pulmonary Lymphangiomyomatosis
1. extensive hamartomas involving smooth muscle of the lungs, lyphatics, hilar,abdominal and lower cervical lymph nodes
2. progressive disorder of childbearing age females with diffuse interstial proliferation of smooth muscle in the lungs, lymph nodes and thoracic duct
3. lesions can produce SOB, emphysema, pneumothoraces, pulmonary hemorrhage with hemoptysis and chylothorax
4. treatment is conservative surgical resection
Bronchial Tumors
Fibroma
1. extremely rare
2. may arise from the peripheral parenchyma of the lung of from the walls of the trachea and bronchi
3. treatment is bronchoscopic removal or YAG laser therapy
Chondroma and Osteochondroma
Lipoma
1. least common benign tumors of the lung
2. may occur endobronchially or in parenchyma
3. 90% are in middle aged males
4. Typically present as solitary nodules
Granular Cell Myoblastoma
1. arise in tongue, skin, subcutaneous breast tissue and occasionally in the lungs
2. occurs in larger bornchi producing obstructive symptoms
3. high recurrence rate if not completely excised
Leiomyoma
1. primary neoplasm of muscular origin
2. sheets of smooth muscle cells predominate
3. rare
Tumors of Developmental or Unknown Origin
Hamartoma
1. most common benign tumor of the lung- 75% of all benign tumors
2. constitute 80 % of all coin lesions
3. derived form embryological remnants
4. 90 % are located in the periphery
5. most commonly in subpleural position
6. conservative resection treatment of choice
Pulmonary Teratoma
Chemodectoma
Inflammatory and other Pseudotumors of the Lung
Plasma cell Granuloma (Histiocytoma)
1. usually in younger patients
2. composed of plasma cells, lymphocytes and fibrous tissue
3. conservative surgical resection
Pseudolymphomas of the Lung
1. Presents as a solitary nodule that can mimic bronchogenic carcinoma
2. composed of lymphoid cells including mature lymphocytes and plasma cells
3. diagnostic feature is the presence of germinal centers
4. may lead to malignant lymphoma
5. long term follow up required
Amyloid Tumors
1. may be localized in bronchus or multiple or difuse bronchial deposits
2. can be a diffuse or localized parenchymal process
3. disease may eventually prove fatal
4. tumors are shelled out readily from surrounding parenchyma
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