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Trachea

Superpage

Revised: 11 February 2013
Copyright: (c) 2001-2013, PathologyOutlines.com, Inc.

Normal anatomy


Reviewer: Adriana Handra-Luca, M.D. (see Reviewers page)
Revised: 3 February 2013, last major update October 2012
Copyright: (c) 2003-2013, PathologyOutlines.com, Inc.

General
=========================================================================

● Cartilaginous and membranous tube (also called windpipe), extending from lower larynx at C6 to upper border of T5 vertebrae, where it divides into right and left mainstem bronchi
● Flattened posteriorly
● 11 cm long, 2-2.5 cm in diameter, diameter greater in men than women, adults than children
● Anteriorly: contacts thyroid isthmus, inferior thyroid veins, neck muscles, cervical fascia, anterior jugular veins, manubrium sterni, thymus, left innominate vein, aortic arch, innominate and left common carotid arteries, deep cardiac plexus
● Posteriorly: contacts esophagus
● Right bronchus appears to be a more direct continuation of trachea, and so is the site of most foreign bodies
● Supplied by inferior thyroid arteries

Diagrams
=========================================================================



Trachea and bronchi


Tracheal bifurcation



Normal histology


Reviewer: Adriana Handra-Luca, M.D. (see Reviewers page)
Revised: 3 February 2013, last major update October 2012
Copyright: (c) 2003-2013, PathologyOutlines.com, Inc.

General
=========================================================================

● Composed of imperfect rings of hyaline cartilage, fibrous tissue, muscular fibers, mucous membranes and glands

Terminology
=========================================================================

Cartilage:
● 16-20 imperfect rings, with circular cartilaginous defect posterior, and replaced by fibrous tissue and muscular fibers
● Each cartilage is 4 mm in depth, 1 mm in thickness
● Are elastic, but may be calcified later in life

Fibrous tissue:
● Thick layer covers outer surface of cartilaginous ring, thin layer covers inner surface
● Both layers merge at upper and lower margins of cartilaginous rings

Muscular tissue:
● Longitudinal and transverse layers of smooth muscle

Mucus membrane:
● Continuous with laryngeal and bronchial membranes
● Ciliated columnar epithelium overlying areolar and lymphoid tissue with elastic fibers, blood vessels, nerves, mucous glands

Drawings
=========================================================================



Transverse section

Micro images
=========================================================================



Transverse section


Various images



Infectious disorders

Adenovirus


Reviewer: Adriana Handra-Luca, M.D. (see Reviewers page)
Revised: 8 February 2013, last major update October 2012
Copyright: (c) 2003-2013, PathologyOutlines.com, Inc.

General
=========================================================================

● Important pathogen in immunocompromised patients, who often have disseminated disease causing death (Am J Clin Pathol 2003;120:575)
● Associated with ulcerations in larynx and trachea



Infectious disorders

Cryptosporidium


Reviewer: Adriana Handra-Luca, M.D. (see Reviewers page)
Revised: 8 February 2013, last major update October 2012
Copyright: (c) 2003-2013, PathologyOutlines.com, Inc.

General
=========================================================================

● Acid fast parasite; tracheal dissemination is rare complication of intestinal infection (Clin Microbiol Rev 2002;15:145)

Case reports
=========================================================================

● 64 year old woman with lymphoma, chronic tracheitis and bronchitis (Arch Pathol Lab Med 1990;114:519)

Micro images
=========================================================================



Modified acid-fast stain (site unspecified, probably bowel)



Infectious disorders

Diphtheria


Reviewer: Adriana Handra-Luca, M.D. (see Reviewers page)
Revised: 8 February 2013, last major update October 2012
Copyright: (c) 2003-2013, PathologyOutlines.com, Inc.

General
=========================================================================

● Tracheal involvement in pharyngeal/laryngeal diphtheria is uncommon
● Late appearance of grey-brown thick and firm false membranes of pharynx and larynx
● Death occurs rarely in non-immunized children (Vestn Otorinolaringol 1995;(2):31)

Case reports
=========================================================================

● Fatal case in a nonimmunized child (Pediatr Pathol 1994;14:391)

Gross images
=========================================================================



6 year old child's trachea and major bronchi with mucosa damaged by toxin produced by Corynebacterium diphtheriae

Micro images
=========================================================================



Complete necrosis of surface epithelium, which has been replaced by pseudomembrane of fibrin, necrotic cells and bacteria

Virtual slides
=========================================================================



Diphtheria



Non-neoplastic lesions

Amyloidosis


Reviewer: Adriana Handra-Luca, M.D. (see Reviewers page)
Revised: 4 February 2013, last major update October 2012
Copyright: (c) 2003-2013, PathologyOutlines.com, Inc.

General
=========================================================================

● Presents as either diffuse narrowing (circumferential thickening of wall) of airway or solitary / multiple nodules (pseudotumoral)
● Associated with laryngeal or nasal involvement

Clinical features
=========================================================================

● Symptoms of asthma, atelectasis, hemoptysis, obstruction
● May induce tracheomalacia in rheumatoid arthritis
● May be due to myeloma, lymphocytic interstitial pneumonia, lymphoplasmacytic lymphomas, plasma cell dyscrasias
● Does not usually evolve into systemic amyloidosis
● 15-40% die at mean 9 years after diagnosis from respiratory failure, pulmonary hemorrhage, pneumonia

Case reports
=========================================================================

● 49 year old man with chronic cough and left lung hilar mass (Arch Pathol Lab Med 2003;127:e420)
● 50 year old man with nasal obstruction (Nihon Kyobu Shikkan Gakkai Zasshi 1997;35:1378)
● 53 year old man with tracheobronchopathia osteochondroplastica and AA amyloidosis (Yonsei Med J 2009;50:721)
● 60 year old woman with pseudotumoral tracheobronchial amyloidosis mimicking asthma (J Med Case Rep 2012;6:40)
● 64 year old woman with history of myasthenia gravis and type 1 diabetes mellitus (AJNR Am J Neuroradiol 2007;28:1557)
● 69 year old woman with seropositive erosive RA and bronchopneumonia (Clin Rheumatol 2008;27:807)
● 70 year old man with tracheobronchial circumferential wall thickening and mediastinal fat infiltration (Clin Nucl Med 2011;36:723)
● Cases with involvement of upper third of the trachea (Vestn Otorinolaringol 2008;(1):67)
● Atypical case necessitating tracheotomy (Eye Ear Nose Throat Mon 1952;31:193)

Treatment
=========================================================================

● Laser therapy or bronchoscopic removal of deposits, radiation therapy, lung transplant

Gross description
=========================================================================

● Focal to diffuse nodular thickening of trachea and proximal bronchial walls with patchy mural calcification
● Also extensive bronchial stenosis, postobstructive pneumonia, atelectasis

Micro description
=========================================================================

● Extensive thickening of submucosa due to irregular nodular masses or sheets of amyloid, reduced submucosal glands, calcification or osseous metaplasia of larger airways
● Variable multinucleated, osteoclast-like giant cells and plasma cells within amyloid
● Also amyloid deposition within submucosal vessel walls

Micro images
=========================================================================



Figure 1: bronchial margin; 2: bronchi; 3: bronchi H&E; 4: Congo Red


Homogenous proteinous material with calcification under bronchus epithelium is Congo Red+


Massive amyloid deposits stain with anti-lambda light chain antibodies


69 year old woman with seropositive erosive RA

Positive stains
=========================================================================

● Congo Red (apple-green birefringence with polarized light)

Differential diagnosis
=========================================================================

● Light chain deposition disease
● Pulmonary lymphoproliferative disorders
● Pulmonary scar tissue
● Systemic amyloidosis
Tracheobronchopathia osteochondroplastica: submucosal bony and cartilaginous tissue projects into tracheobronchial lumen, no amyloid



Non-neoplastic lesions

Mucous membrane plasmacytosis


Reviewer: Adriana Handra-Luca, M.D. (see Reviewers page)
Revised: 4 February 2013, last major update October 2012
Copyright: (c) 2003-2013, PathologyOutlines.com, Inc.

General
=========================================================================

● Non-neoplastic proliferative disorder of plasma cells
● Mean 54 years, range 40-67 years, 2/3 men

Treatment
=========================================================================

● Unknown: antibiotics, steroids, surgery are unsuccessful

Gross description
=========================================================================

● Cobblestone or warty appearance of larynx, pharynx, palate, lips, mouth, tongue, trachea

Micro description
=========================================================================

● Psoriasiform epithelial hyperplasia with dyskeratosis and dense subepithelial plasmacytosis
● Diffuse but mature plasma cells are polyclonal (i.e. no light chain restriction)

Micro images
=========================================================================



Buccal mucosa: hyperplastic, parakeratinized stratified squamous epithelium with dense infiltrate of mature plasma cells in lamina propria


Buccal mucosa: plasma cells are IgG+, CD138+, both kappa+ and lambda+



Non-neoplastic lesions

Necrotizing sialometaplasia


Reviewer: Adriana Handra-Luca, M.D. (see Reviewers page)
Revised: 4 February 2013, last major update October 2012
Copyright: (c) 2003-2013, PathologyOutlines.com, Inc.

General
=========================================================================

● More commonly seen in palate
● Due to prolonged intubation

Case reports
=========================================================================

● Three cases involving submucosal glands in the trachea following prolonged translaryngeal intubation (Histopathology 1992;21:280)

Clinical images
=========================================================================



Palate: ulcers

Micro images
=========================================================================



Palate: necrotizing inflammatory process



Non-neoplastic lesions

Post-tracheostomy atypia


Reviewer: Adriana Handra-Luca, M.D. (see Reviewers page)
Revised: 4 February 2013, last major update October 2012
Copyright: (c) 2003-2013, PathologyOutlines.com, Inc.

General
=========================================================================

● Atypical cells in trachea; may also occur in bronchi
● May be due to effect of dry air on tracheal mucosa

Differential diagnosis
=========================================================================

● Squamous cell dysplasia/carcinoma in situ



Non-neoplastic lesions

Rheumatoid nodules


Reviewer: Adriana Handra-Luca, M.D. (see Reviewers page)
Revised: 4 February 2013, last major update October 2012
Copyright: (c) 2003-2013, PathologyOutlines.com, Inc.

General
=========================================================================

● Occasionally seen in patients with rheumatoid arthritis; few published articles

Case reports
=========================================================================

● 45 year old man with polyarthritis, new subcutaneous nodules and fibrosing alveolitis (Chest 1993;103:301)

Treatment
=========================================================================

● Prednisone

Micro description
=========================================================================

● Firbinoid necrosis, palisaded inflammation, vasculitis

Micro images
=========================================================================



Lung lesions (no trachea images available)



Non-neoplastic lesions

Tracheobronchitis


Reviewer: Adriana Handra-Luca, M.D. (see Reviewers page)
Revised: 4 February 2013, last major update October 2012
Copyright: (c) 2003-2013, PathologyOutlines.com, Inc.

General
=========================================================================

● Due to tracheostomy, ventilators, other causes (viral, bacterial, chemical or physical irritants)

Gross description
=========================================================================

● Necrotic ulceration at tracheostomy opening

Micro description
=========================================================================

● Erythematous mucosa

Micro images
=========================================================================



Necrotizing tracheobronchitis due to H1N1 infection (figure b)



Non-neoplastic lesions

Tracheopathia osteoplastica


Reviewer: Adriana Handra-Luca, M.D. (see Reviewers page)
Revised: 3 February 2013, last major update October 2012
Copyright: (c) 2003-2013, PathologyOutlines.com, Inc.

General
=========================================================================

● Rare disorder in which exophytic osteocartilaginous nodules arise from inner surface of tracheal ring cartilage, and are connected to one another by fibrous struts, forming an incomplete cylinder of fibro-osteocartilaginous tissue superficial to the tracheal rings
● Lesions are rarely diffuse and extensive

Case reports
=========================================================================

● 64 year old woman (Acta Otorrinolaringol Esp 1999;50:164)

Micro images
=========================================================================



Bronchial involvement: bone tissue with hematopoietic cells in bronchial mucous membrane, part of tissue has coalesced with bronchus cartilage



Tumors

Acinic cell carcinoma


Reviewer: Adriana Handra-Luca, M.D. (see Reviewers page)
Revised: 4 February 2013, last major update October 2012
Copyright: (c) 2003-2013, PathologyOutlines.com, Inc.

General
=========================================================================

● Rare tumor similar to salivary gland tumor
● Symptoms: dyspnea, upper airway obstruction

Case reports
=========================================================================

● 27 year old woman with dyspnea (Gan No Rinsho 1984;30:1412)
● 54 year old woman with laryngotracheal junction tumor after thyroid radiation (Arch Pathol Lab Med 1981;105:266)
● 54 year old man with widely invasive tumor (Surg Today 2004;34:764)
● 56 year old woman with post-radiation tumor (Otolaryngol Head Neck Surg 1981;89:398)
● 64 year old man with predominantly mucous differentiated acinic cell adenocarcinoma of seromucous tracheal glands (Zentralbl Allg Pathol 1989;135:751)
● Case with upper airway obstruction treated with YAG laser and surgical resection (Chest 1996;110:1120)

Treatment
=========================================================================

● Wide resection, may recur



Tumors

Adenoid cystic carcinoma


Reviewer: Adriana Handra-Luca, M.D. (see Reviewers page)
Revised: 8 February 2013, last major update October 2012
Copyright: (c) 2003-2013, PathologyOutlines.com, Inc.

General
=========================================================================

● #2 most common primary malignancy of trachea after squamous cell carcinoma
● Resembles salivary gland tumor

Clinical features
=========================================================================

● Usually in upper third of trachea
● Intramural (15%) or extramural (85%)
● Symptoms: dry cough, dyspnea, hemoptysis
● May coexist with main bronchus lesion
● 20% invade locally (including thyroid gland); may metastasize to lymph nodes, nasal cavity, liver
● Lengthy clinical course, but poor long term prognosis due to late local recurrences
● Median overall surival 18 years, median disease free survival 10 years
● 75% survival rate if complete resection
● Predictors of survival: margin status, extramural disease, perineural invasion, lymph node metastases (Eur J Cardiothorac Surg 2010;37:1438, Ai Zheng 2004;23:581)

Case reports
=========================================================================

● 44 year old woman with midline neck swelling mimicking thyroid tumor (Lung India 2010;27:167)
● 52 year old woman with tracheal primary and liver metastases (Gut Liver 2009;3:127)
● 63 year old woman with metastasis to nasal cavity (Ear Nose Throat J 2009;88:E9)
● 79 year old woman treated with curettage and radiation therapy (South Med J 2011;104:68)
● Metastatic to placenta (Hum Pathol 1989;20:193)

Treatment
=========================================================================

● Chemotherapy, radiotherapy, surgery (Head Neck 2012;34:1263)

Micro description
=========================================================================

● Cribriform growth pattern of nests of tumor cells with discrete, rounded, “punched-out” gland-like spaces filled with eosinophilic or basophilic material
● Infiltrative, perineural invasion common

Micro images
=========================================================================



Uniform hyperchromatic basaloid cells surrounding acellular spaces containing mucoid and hyaline material


Tubular structures with microcysts surrounding hyalinized stroma

Cytology description
=========================================================================

● Rarely extends to thyroid and presents as thyroid nodule (Am J Clin Pathol 2004;121:551)

Cytology images
=========================================================================



Spherical globules of basement membrane material and hyperchromatic uniform rounded tumor cells with scanty cytoplasm (May Grunwald Giemsa stain)

Positive stains
=========================================================================

● Brain derived neurotrophic factor (Hum Pathol 2002;33:933)

Differential diagnosis
=========================================================================

● Thyroid tumor



Tumors

Basaloid squamous cell carcinoma


Reviewer: Adriana Handra-Luca, M.D. (see Reviewers page)
Revised: 8 February 2013, last major update October 2012
Copyright: (c) 2003-2013, PathologyOutlines.com, Inc.

General
=========================================================================

● Basaloid cells associated with dysplastic or neoplastic squamous cells (Am J Clin Pathol 1995;104:594, Head Neck Pathol 2008;2:83)
● Aggressive and rapidly fatal in upper aerodigestive tract

Micro images
=========================================================================



Head and neck tumors (site unspecified)



Tumors

Fibromyxoma


Reviewer: Adriana Handra-Luca, M.D. (see Reviewers page)
Revised: 8 February 2013, last major update October 2012
Copyright: (c) 2003-2013, PathologyOutlines.com, Inc.

General
=========================================================================

● Very rare tumor - only a few case reports

Case reports
=========================================================================

● 53 year old man with coexisting small cell carcinoma of lung (Intern Med 1993;32:895)
● 59 year old woman with asthmatic symptoms (Arch Pathol Lab Med 1985;109:926)

Treatment
=========================================================================

● Endoscopic resection or segmental tracheal resection with end-to-end anastomosis



Tumors

Fibrous histiocytoma


Reviewer: Adriana Handra-Luca, M.D. (see Reviewers page)
Revised: 8 February 2013, last major update October 2012
Copyright: (c) 2003-2013, PathologyOutlines.com, Inc.

General
=========================================================================

● Low grade fibrohistiocytic tumor, usually children, also adults after radiation therapy for head and neck carcinoma
● Symptoms: hemoptysis, stridor, wheezing
● MFH (malignant cases) also described (Jpn J Thorac Cardiovasc Surg 2005;53:276, Changgeng Yi Xue Za Zhi 1993;16:271, Respirology 1999;4:271, Am Rev Respir Dis 1987;135:761)

Case reports
=========================================================================

● Infant tumor successfully treated by laser (Lung Cancer 2007;57:112)
● 3 year old child and 5 year old child (Jpn J Thorac Cardiovasc Surg 2002;50:495)
● 10 year old girl with dyspnea and wheezing (Eur J Cardiothorac Surg 1999;16:464)
● 15 year old girl (Am J Clin Pathol 1978;70:429)
● 17 year old girl with upper airway obstruction (Int J Pediatr Otorhinolaryngol 1990;19:295)
● Case at the level of the third tracheal ring (J Laryngol Otol 1981;95:1287)

Treatment
=========================================================================

● Complete resection; laser therapy; may recur
● Radiation or chemothrapy for malignant cases (MFH)

Micro description
=========================================================================

● Spindle cells arranged in storiform and fascicular patterns

Micro images
=========================================================================



10 year old girl with dyspnea and wheezing

Positive stains
=========================================================================

● Vimentin and CD68

Negative stains
=========================================================================

● p53



Tumors

Granular cell tumor


Reviewer: Adriana Handra-Luca, M.D. (see Reviewers page)
Revised: 8 February 2013, last major update October 2012
Copyright: (c) 2003-2013, PathologyOutlines.com, Inc.

General
=========================================================================

● Benign tumor of possible neurogenic origin, rare in trachea, with solid sheets of large ovoid cells with ample, eosinophilic cytoplasm, eccentric nuclei, and prominent nucleoli (Ann Otol Rhinol Laryngol 1984;93:457)
● Luminal or secondarily involved; usually upper third of trachea
● Adults, children, may be associated with pregnancy
● May be incidental findings associated with lung and nodal tuberculosis, bronchial carcinoma (Orv Hetil 2002;143:239)
● Clinically appears as yellowish patch
● May rarely be malignant and invade retrotracheal space

Case reports
=========================================================================

● 14 year old girl with benign granular cell tumor in ventral carina (Ann Thorac Surg 2005;79:e15)
● 20 year old man with hemoptysis, cough, pleuritic chest pain (Korean J Intern Med 2007;22:101)
● 21 year old pregnant woman with tumor at retrotracheal space (Yonsei Med J 1999;40:76)
● 45 year old woman with pulmonary tuberculosis and tracheal tumor (Pneumologie 2008;62:158)
● Woman with recurrent disease in subsequent pregnancies (Laryngoscope 2004;114:143)

Treatment
=========================================================================

● Resection (Laryngoscope 1992;102:807)

Micro images
=========================================================================



20 year old man with hemoptysis, cough and pleuritic chest pain

Positive stains
=========================================================================

● S100, neuron-specific enolase, CD68

Electron microscopy description
=========================================================================

● Progressive cytoplasmic granulation of cells, with transition from "early granular cells" to fully developed mature granular cells (Arch Pathol Lab Med 1987;111:1065)

Differential diagnosis
=========================================================================

● Hurthle cell neoplasm: may appear similar with fine needle aspiration (Diagn Cytopathol 2000;22:379)



Tumors

Invasive fibrous tumor


Reviewer: Adriana Handra-Luca, M.D. (see Reviewers page)
Revised: 8 February 2013, last major update October 2012
Copyright: (c) 2003-2013, PathologyOutlines.com, Inc.

General
=========================================================================

● Invasive tracheal neoplasm of proliferating fibroblasts with moderate nuclear pleomorphism and low mitotic activity; some similarities to inflammatory pseudotumor of lung, fibrous histiocytoma, fibromatosis (Hum Pathol 1989;20:180)
● Unclear if distinct from solitary fibrous tumor, which may invade trachea from mediastinum (Thorac Cardiovasc Surg 2004;52:110)
● Tumors invade to or between plates of cartilage
● May recur, but do not metastasize



Tumors

Lymphoepithelioma-like carcinoma


Reviewer: Adriana Handra-Luca, M.D. (see Reviewers page)
Revised: 8 February 2013, last major update October 2012
Copyright: (c) 2003-2013, PathologyOutlines.com, Inc.

General
=========================================================================

● EBV related, nonkeratinized tumor with syncytial sheets of undifferentiated cells with minimal cytoplasm, prominent nucleoli, numerous mitoses and lymphocytes
● Similar to nasopharyngeal tumor, but very rare in trachea

Case reports
=========================================================================

● 22 year old man with dyspnea on exertion (Surg Today 2007;37:584)
● 27 year old woman with EBV+ tumor (Ann Thorac Cardiovasc Surg 2007;13:191)
● Young woman treated with surgery, radiotherapy and chemotherapy (Indian J Cancer 2002;39:112)

Treatment
=========================================================================

● Resection, radiotherapy



Tumors

MALT lymphoma


Reviewer: Adriana Handra-Luca, M.D. (see Reviewers page)
Revised: 8 February 2013, last major update October 2012
Copyright: (c) 2003-2013, PathologyOutlines.com, Inc.

General
=========================================================================

● Low grade lymphoma similar to tumors elsewhere (see Lymphoma and plasma cell neoplasms chapter)
● May represent recurrence of parotid tumor, be associated with bronchial tumors
● Polypoid or multiple tumors
● Symptoms: cough, dyspnea, hoarseness, weight loss

Case reports
=========================================================================

● 44 year old woman treated with long term survival (Thorax 2005;60:82)
● 44 year old woman (Intern Med 1992;31:655)
● 62 year old woman with Sjogren syndrome (Nihon Kokyuki Gakkai Zasshi 2011;49:543)
● 71 year old woman (Sao Paulo Med J 2012;130:126 )
● 74 year old woman with laser photoresection (Intern Med 1999;38:276)
● 83 year old woman (Yonsei Med J 2008;49:860)
● Early case with severe upper airway obstruction (Am J Surg Pathol 1992;16:71)

Treatment
=========================================================================

● Bronchoscopic resection, R-COP

Micro description
=========================================================================

● Diffuse infiltrate of small lymphocytes with centrocyte-like features surrounding reactive follicles
● Plasmacytoid cells and lymphoepithelial lesions present

Micro images
=========================================================================



71 year old woman with a history of dry coughing and dyspnea


83 year old woman with a dry cough and dyspnea

Positive stains
=========================================================================

● Light chain restriction

Negative stains
=========================================================================

● CD5, CD10



Tumors

Mucoepidermoid carcinoma


Reviewer: Adriana Handra-Luca, M.D. (see Reviewers page)
Revised: 8 February 2013, last major update October 2012
Copyright: (c) 2003-2013, PathologyOutlines.com, Inc.

General
=========================================================================

● Usually low grade lesion with squamous cells without atypia in desmoplastic stroma

Clinical features
=========================================================================

● Usually children with a luminal polyp, possibly with bronchus extension
● May occur in patients with tracheobronchopathia osteochondroplastica
● Symptoms: dyspnea, stridor

Case reports
=========================================================================

● 9 year old boy with dyspnea and diaphoresis (Interact Cardiovasc Thorac Surg 2012;15:311)
● 11 year old girl with dedifferentiated tumor (Histopathology 2009;55:472)
● 22 year old woman with exertional dyspnea and stridor (Pneumologia 2011;60:164)
● 51 year old man with tracheobronchopathia osteochondroplastica (Chirurg 1995;66:231)
● 78 year old woman with oncocytic tumor (Arch Pathol Lab Med 1999;123:635)

Treatment
=========================================================================

● Resection

Micro description
=========================================================================

● Low grade lesion with foci of squamous differentiation without atypia in desmoplastic stroma
● Prominent vascular component
● No marked atypia, no necrosis, no mitotic activity, no angiolymphatic invasion, may be dedifferentiated

Micro images
=========================================================================



9 year old boy


Oncocytic tumor: simple cysts, oncocytes and anti-mitochondrial antibody

Positive stains
=========================================================================

● Keratin (entire tumor)
● Oncocytic tumor: PTAH and antimitochondrial antibody in oncocytes

Negative stains
=========================================================================

● Neuroendocrine markers, Congo red

Differential diagnosis
=========================================================================

● Carcinoid tumor



Tumors

Papilloma / Papillomatosis


Reviewer: Adriana Handra-Luca, M.D. (see Reviewers page)
Revised: 8 February 2013, last major update October 2012
Copyright: (c) 2003-2013, PathologyOutlines.com, Inc.

General
=========================================================================

● Benign squamous lesion similar to laryngeal lesions, due to infection with HPV6, HPV11 (Chron Respir Dis 2011;8:233, J Med Virol 2004;72:473)

Clinical features
=========================================================================

● Juvenile and adult onset
● Symptoms: cough, hemophtysis
● May disappear spontaneously
● May occur in patient with primary biliary cirrhosis, Cowden disease, tuberculosis
● Cases associated with laryngeal lesions have low incidence of malignant transformation
● Cases limited to trachea and bronchi usually begin in adults, have higher incidence of malignant transformation
● Tracheotomy is associated with progression of laryngeal papilloma into trachea (Zhonghua Er Bi Yan Hou Ke Za Zhi 2000;35:384)

Case reports
=========================================================================

● 27 year old man with large papillary mass and invasive component (Am J Surg Pathol 1980;4:79)
● 42 year old woman with multiple papillomatous-like tumors involving the trachea and bronchi (Chest 1983;83:817)
● 52 year old woman with breathlessness on exertion (Ann Thorac Surg 2004;77:2201)
● 57 year old man with malignant transformation of solitary papilloma (Can Respir J 2008;15:375)
● 58 year old man with Cowden's disease (Intern Med 2006;45:987)
● 66 year old man with recurrent papilloma (Asian J Surg 2007;30:88)
● 72 year old woman with multiple squamous papillomas (Intern Med 2008;47:1535)

Treatment
=========================================================================

● HPV vaccine (to prevent); medical therapy, multiple surgery if recurrence

Micro description
=========================================================================

● Usually benign squamous, intermediate and ciliated cells overlying fibrovascular stroma
● Invasive tumor may have intracystic papillary structures, atypical invasive epithelium

Micro images
=========================================================================



Laryngeal papilloma

Differential diagnosis
=========================================================================

● Oat cell carcinoma: looks similar at bronchoscopy



Tumors

Rhabdomyosarcoma


Reviewer: Adriana Handra-Luca, M.D. (see Reviewers page)
Revised: 4 February 2013, last major update October 2012
Copyright: (c) 2003-2013, PathologyOutlines.com, Inc.

General
=========================================================================

● Very rare; usually affects children
● Symptoms: dyspnea, hemoptysis, stridor
● No distant metastases

Case reports
=========================================================================

● 14 month old girl with biphasic stridor (J Laryngol Otol 2012;126:966)
● 12 year old girl with severe dyspnea (J Laryngol Otol 1988;102:735)
● 65 year old man with cough, wheezing and hemoptysis (Hum Pathol 1980;11:572)

Treatment
=========================================================================

● Surgery, chemotherapy, radiotherapy



Tumors

Small cell carcinoma


Reviewer: Adriana Handra-Luca, M.D. (see Reviewers page)
Revised: 3 February 2013, last major update October 2012
Copyright: (c) 2003-2013, PathologyOutlines.com, Inc.

General
=========================================================================

● Histology similar to bronchial tumor
● 6% of primary tracheal tumors in 1993 study (Thorax 1993;48:688)

Clinical features
=========================================================================

● Endotracheal nodule or eccentric wall thickening, papillomatous at endoscopy
● May be tracheobronchic
● May occur with hypophosphatemia osteomalacia and Cushing syndrome
● May occur secondary to breast ductal carcinoma with radiation, or be metastatic from lung primary
● May metastasize to CNS

Case reports
=========================================================================

● 25 year old woman with central airway obstruction and dyspnea (Br J Radiol 2008;81:e120)
● 27 year old man (Rev Pneumol Clin 1994;50:172)
● 42 year old woman with multiple papillomatous-like tumors involving trachea and bronchi. (Chest 1983;83:817)
● 58 year old woman with ankle swelling, dysphagia, widespread bone and joint pains and hypophosphataemia (J Clin Pathol 1994;47:80)
● 60 year old man with dyspnea on exertion and faint stridor (Thorax 1980;35:72)
● Ultrastructural evidence of endocrine differentiation (Arch Pathol Lab Med 1984;108:149)

Treatment
=========================================================================

● Radio-chemotherapy (Presse Med 1992;21:1905)

Micro description
=========================================================================

● Classic features of large neuroendocrine cells with high N/C ratio, hyperchromatic nuclei, crush artifact, nuclear molding
● May show combined morphology with squamous cell carcinoma and giant cell carcinoma

Micro images
=========================================================================



Small clusters and rosettes of small tumour cells


Adjacent islands of oat cell and squamous cell carcinoma surrounded by stroma containing bizarre giant cells (Haematoxylin and eosin X500)


Esophagus: small cell carcinoma, oat cell type

Positive stains
=========================================================================

● Keratin; also bombesin and CEA

Differential diagnosis
=========================================================================

● Extension from bronchial tumor



Tumors

Squamous cell carcinoma


Reviewer: Adriana Handra-Luca, M.D. (see Reviewers page)
Revised: 3 February 2013, last major update October 2012
Copyright: (c) 2003-2013, PathologyOutlines.com, Inc.

General
=========================================================================

● Exophytic, obstructive tumor usually in smokers (J Am Coll Surg 2006;202:237), but also children
● Most common primary malignancy of trachea (40%-75%, Cancer 1990;66:894)

General
=========================================================================

● Usually arises in lower third of trachea (Eur Arch Otorhinolaryngol 1993;250:383, Acta Otolaryngol 1991;111:1162)
● May be extension of other head and neck primary
● May be associated with pneumoconiosis or post-tracheotomy scar.(Respiration 1993;60:250)
● Rapid clinical course, poor prognosis (Am J Clin Oncol 2011;34:32, Virchows Arch 2009;455:423, Jpn J Clin Oncol 1997;27:305)

Case reports
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● 11 month old boy with severe dyspnea and stridor (Int J Pediatr Otorhinolaryngol 1998;43:163)
● 37 year old woman with hypohidrotic ectodermal dysplasia (J Laryngol Otol 2002;116:742)
● 50 year old man with unresectable basaloid squamous cell carcinoma (J Cancer Res Ther 2010;6:321)
● 52 year old woman with HPV and squamous cell carcinoma in a solitary tracheal papilloma (Ann Thorac Surg 2004;77:2201)
● 54 year old man with dyspnea, hemoptysis, cough and weight loss (Acta Otorhinolaryngol Ital 2010;30:209)
● 70 year old woman with double-primary cancer of the lung and trachea (Nihon Kyobu Shikkan Gakkai Zasshi 1996;34:216)
● 78 year old man with spindle cell sarcomatoid carcinoma (Tuberk Toraks 2009;57:337)

Treatment
=========================================================================

● Surgical excision with end-to-end anastomosis, radiation therapy (Med Princ Pract 2004;13:69)

Micro description
=========================================================================

● Variants include spindle cell, sarcomatoid
● May arise in papilloma
● May be combined with small cell and giant cell carcinoma

Micro images
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Basaloid squamous cell carcinoma


Various images

Differential diagnosis
=========================================================================

Adenoid cystic carcinoma
● Extension of esophageal tumor: see Arch Pathol Lab Med 1984;108:983
● Metastasis from colon carcinoma (Can Assoc Radiol J 1989;40:198)

End of Trachea > Superpage


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