Nasal cavity, paranasal sinuses, nasopharynx
Last revised 20 March 2008
Copyright (c) 2004-2008, PathologyOutlines.com, Inc.
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Primary references, normal anatomy, normal histology
Inflammatory/infectious lesions: allergic fungal sinusitis, allergic rhinitis, Aspergillus, chronic rhinitis, cholesterol granuloma, Churg-Strauss syndrome, eosinophilic angiocentric fibrosis, fungal ball, fungal disease, granulomatous lesions, idiopathic midline destructive disease, infectious rhinitis, Kartagener syndrome, leprosy, mucocele, Mucor, myospherulosis, nasal polyps, necrotizing sialometaplasia, pertussis, pharyngitis, rhinoscleroma, rhinosporidiosis, sinusitis, sarcoidosis, steroid injections, tuberculosis, Wegener’s granulomatosis
Nasopharyngeal carcinoma: general, keratinizing squamous cell, nonkeratinizing-differentiated, undifferentiated, papillary adenocarcinoma
Sinonasal carcinoma: general, adenocarcinoma-general, low grade adenocarcinoma, intestinal adenocarcinoma, cylindrical (transitional), small cell, squamous cell, undifferentiated (anaplastic)
Hematologic conditions: lymphoma-general, angiotropic lymphoma, diffuse large B cell lymphoma, follicular lymphoma, lymphoid hyperplasia, lymphomatoid granulomatosis, mantle cell lymphoma, NK/T cell lymphoma, peripheral T cell lymphoma, plasmacytoma
Other tumors: ameloblastoma, aneurysmal bone cyst, angiomyolipoma, angiosarcoma, astrocytoma, carcinoid, chondrosarcoma, chordoma, cocaine related, craniopharyngioma, dermoid cyst, desmoplastic small round cell tumor, fibroinflammatory proliferation, fibroma, fibromatosis, fibroosseous lesions, fibrosarcoma, follicular dendritic cell, gangliocytic paraganglioma, giant cell reparative granuloma, giant cell tumor, glial heterotopia, glomus, hemangiopericytoma-sinonasal type, leiomyoma, lobular capillary hemangioma, malignant fibrous histiocytoma, malignant peripheral nerve sheath tumor, Masson’s tumor, melanoma, meningioma, myxoma, nasal chondromesenchymal hamartoma, nasopharyngeal angiofibroma, neurofibroma, nodular fasciitis, nonsecretory cyst, olfactory neuroblastoma, osteochondromyxoma, osteoma, papilloma, paraganglioma, pituitary adenoma, pleomorphic adenoma, psammomatoid ossifying fibroma, respiratory epithelial adenomatoid hamartoma, rhabdomyoma, rhabdomyosarcoma, Rosai-Dorfman disease, salivary gland anlage tumor, salivary gland tumors-general, schwannoma, solitary fibrous tumor, teratoid carcinosarcoma, teratoma, Warthin’s
Miscellaneous: TNM staging, post-chemotherapy atypia, grossing, features to report
AJCC Cancer Staging Manual (6th Ed)
American Journal of Surgical Pathology (AJSP), Jan 1998 to Aug 2004
Archives of Pathology and Laboratory Medicine (Archives), Jan 1998 to Aug 2004
Human Pathology (Hum Path), Jan 1998 to July 2004
Modern Pathology (Mod Path), Jan 1998 to July 2004
Rosai, J: Ackerman’s Surgical Pathology (9th Ed); Mosby-Year Book, Inc., 2004
Sternberg, S: Diagnostic Surgical Pathology (4th Ed); Lippincott Williams & Wilkins, 2004
Journal search terms: nose, nasal, paranasal, sinus, nasopharynx
Please refer to these primary references for more detailed discussions and photographs
Nasal cavity, paranasal sinuses and nasopharynx form functional unit
Nasal cavity
Divided into olfactory region (superior nasal turbinates and opposed septum) and respiratory region (rest of cavity)
Nasal chambers are on either side of median plane formed by nasal septum
Bounded above by cribriform plate; bounded laterally by turbinates
Bulla ethmoidalis: elevation on lateral wall of middle meatus, site of opening of middle ethmoid meatus
Choanae: posterior opening of nasal cavity, communicates with nasopharynx
Columella: anterior extreme nasal septum
Nares: anterior openings of nasal cavity
Vestibule: slight dilation inside anterior aperture of nostril, bounded laterally by ala and lateral crus of greater alar cartilage and medially by medial crus of greater alar cartilage; lined by skin containing hair and sebaceous glands
Lateral wall: contains superior, middle and inferior nasal turbinates (conchae); below each is corresponding nasal passage or meatus
Sphenoethmoidal recess: above superior turbinate, site of opening of sphenoidal sinus
Turbinates (concha): comprise lateral walls of each nasal cavity
Superior meatus: along upper border of middle turbinate, site of opening of posterior ethmoid meatus
Middle meatus: below and lateral to middle turbinate
Drawings: lateral wall of nasal cavity #1, #2 after removal of conchae, cartilages of nose-side view, from below, bone and cartilage of septum
Nasopharynx
Respiratory passage above and behind the soft palate
Part of pharynx, which also includes oropharynx and hypopharynx
Begins anteriorly at posterior turbinates and extends along plane of airway to the level of the free border of the soft palate
Anterior wall is perforated by posterior nares (choanae)
Posterior wall is also its roof, as well as the posterior base of skull; extends inferiorly to level of free border of soft palate where oropharynx begins
Lateral wall contains ostium of eustachian tube, surrounded by mucosa covered cartilaginous prominence; ostium is anterior to pharyngeal recess (fossa of Rosenmuller)
Gross image: regions of pharynx
Paranasal sinuses
Diverticula of nasal cavity that extend into neighboring bones
Ethmoid sinuses: between the orbits; well developed at birth
Frontal sinuses: most anterior, above the orbits; small/rudimentary at birth; develop through puberty
Maxillary sinuses: under the cheeks; small/rudimentary at birth; develop rapidly during childhood until permanent teeth develop
Sphenoid sinuses: most posterior at base of brain; small/rudimentary at birth; develop rapidly during childhood until permanent teeth develop
Ohngren’s line: connects medial canthus of eye to angle of mandible; used to divide maxillary sinus into anteroinferior portion (infrastructure), associated with good prognosis for carcinomas, and superoposterior portion (suprastructure), with a poor prognosis for carcinomas
Nasal cavity
Lined by stratified squamous and respiratory type pseudostratified columnar epithelium, separated by transitional epithelium in some places
Respiratory mucosa (also called Schneiderian membrane) may contain goblet cells; may undergo squamous metaplasia
Superior third of nasal septum, superior turbinate and cribriform plate are covered with thinner olfactory mucosa, usually patchy in adults, which has neuroendocrine features
Seromucinous glands (resembling salivary glands) are present in submucosa, numerous near eustachian tube opening of nasopharynx, may undergo oncocytic metaplasia with increasing age
Normally no lymphoid tissue
Nasopharynx
Lined by stratified squamous epithelium (inferior anterior and posterior walls and anterior lateral walls) and respiratory type epithelium (around nasal choanae and roof of posterior wall); remaining areas have mixtures of squamous and respiratory or intermediate epithelium (also called transitional although it does not resemble urothelium ultrastructurally)
Intermediate epithelium is usually concentrated as a wavy ring at junction of nasopharynx and oropharynx
Seromucinous glands may undergo oncocytic metaplasia, and rarely form a mass or obstruct eustachian tube
Abundant lymphoid tissue present, particularly at rim of eustachian tube opening (Gerlach’s tonsil); functionally equivalent to that of GI tract or mucosal-associated lymphoid tissue (MALT)
Paranasal sinuses
Mucosa is continuous with nasal cavity and identical (respiratory type epithelium), but thinner and with fewer goblet cells and seromucinous glands
Normally no lymphoid tissue
Inflammatory/infectious lesions
Due to Aspergillus, Curvularia lunata or Fontana-Masson positive dematiaceous fungi (Drechslera, Bipolaris, Exoserohilium)
Produces “allergic mucin” (lamellated collection of inspissated inflammatory debris with numerous eosinophils and Charcot-Leyden crystals) and fungal hyphae
Mucin may require curettage to remove, may recur
May cause bony erosion due to pressure remodeling, but usually not invasive
Affects immunocompetent patients ages 20-49 years with chronic allergy, asthma, nasal polyps or sinusitis
May be due to tenacious mucin that traps normally nonpathogenic, low-virulence fungal organisms
Diagnosis initially missed in half of cases
Treatment: surgical removal, low-dose corticosteroids, possibly immunotherapy for underlying allergy
Gross: green, brown or black mucin with consistency of clay; gray-brown, laminated, gelatinous or translucent cut surface
Micro: mucosal edema, marked eosinophilic infiltrate, allergic mucin in 92%, eosinophils may have degenerative changes of smudged, elongated or basophilic nuclei; also plasma cells and lymphocytes; rare noninvasive fungal hyphae (often found only with GMS stain), rare neutrophils
Micro images: 1-allergic mucin (arrows) with fungus; 2-allergic mucin (arrows) without fungus; 3-fungi with sparse and fractured hyphae
References: Hum Path 2004;35:474
Also called hay fever
Due to exposure to plant pollens, fungi, dust mites, animal allergens
Affects 20% of US population
IgE mediated
Endoscopic images: swollen pale turbinates with thick secretions
Micro: mucous secretions have neutrophils and prominent eosinophils
Causes fungus balls (see below) in nasal antrum of immunocompetent patients with minimal inflammatory response, microabscesses or multinucleated giant cells
Also causes invasive aspergillosis, regardless of immune status, with extension into retroorbital region, cranium or parapharyngeal space; often fatal
Also causes allergic fungal sinusitis (see above)
Micro: septate hyphae that branch at 45 degrees
DD of invasive fungal infections: Zygomycetes, Pseudallescheria boydii, Paecilomyces, Alternaria, Cladosporium trichoides, Fusarium
Sequel to acute rhinitis (symptoms lasting 6 weeks or less), with development of secondary bacterial infection
Associated with deviated septum or nasal polyps; also ulceration and infection extending into sinuses
Endoscopic images: pale turbinates and dry secretions in smoker
Rare in nasal cavity or sinuses; usually associated with chronic middle ear disease
May be a reaction to hemorrhage
Treatment: excision
Case reports: maxillary sinus lesion of 38 year old man (Archives 2002;126:217)
Micro: foreign body giant cells surrounding empty, needle shaped spaces (cholesterol clefts representing cholesterol crystals that have dissolved due to alcohol in staining process), chronic inflammatory infiltrate, hemosiderin laden macrophages, dilated lymphatics
2/3 have nasal polyps or mucosal crusting
Micro: discrete necrotizing granulomas with numerous eosinophils, often forming microabscesses
DD: Wegener’s granulomatosis
Eosinophilic angiocentric fibrosis
Very rare; unknown cause
May be mucosal counterpart of granuloma faciale
Involves any portion of upper respiratory tract, often nasal septum and sinus mucosa
Usually women, mean age 44 years
Case reports: 45 year old man with no history of allergic disease (Archives 2004;128:90)
Treatment: corticosteroids, resection
Micro: thick collagen bundles, perivascular onion-skin fibrosis with eosinophil-rich inflammatory infiltrate; no granulomas, no necrosis, no vasculitis
DD: granuloma faciale (inflammatory vascular reaction with facial papules, neutrophilic and eosinophilic infiltrate, vasculitis with fibrinoid material in vessel walls)
Also called mycetoma, chronic noninvasive fungal sinusitis
Usually immunocompetent patients, often prior history of sinus disease, trauma or foreign body
Gross: grumous, friable, gray-brown-black material, often with clotted blood
Micro: tightly packed extramucosal hyphae, usually in maxillary antrum; no/minimal host response, no allergic mucin or tissue invasion; usually due to Aspergillus (hyphae with septation and branching at 45 degree angles, no spores)
Includes sinusitis (acute fulminant, chronic invasive), mycetoma, saprophytic infestation (fungal spores on mucous crusts of respiratory passages) and allergic fungal sinusitis
May develop post-steroid injection with amorphous foreign material
Cholesterol granulomas in paranasal sinuses are rare
Also due to fungal infections, myospherulosis, tuberculosis, Wegener’s granulomatosis
Idiopathic midline destructive disease
Diagnosis of exclusion
Gross: usually perforated nasal septum, perforated palate, erosion of antral bone or ulceration of skin overlying nose or antrum
Micro: acute and chronic inflammatory cells with variable necrosis; may have multinucleated giant cells or granulomas; no atypical lymphocytes, no fibrinoid necrosis of vessels, no prominent eosinophils
DD: Wegener’s granulomatosis (different clinical findings)
Also called “common cold”
Due to adenovirus, echovirus and rhinoviruses
Symptoms: catarrhal discharge, thick, edematous, erythematous nasal mucosa; enlarged turbinates
May cause pharyngotonsillitis; may have secondary bacterial infection
Situs inversus, bronchiectasis and sinusitis, due to defective ciliary action
Affects nasal mucosa in 95% of patients; may be initial manifestation of disease
Usually affects nasal septum and inferior turbinate
Micro: early - plasma cells with lesser numbers of histiocytes and lymphocytes; later - broad sheets of histiocytes with foam cells; well formed granulomas of tuberculous type are uncommon
Positive stains: acid fast stains
Also called pseudocyst
Complication of chronic sinusitis due to outflow obstruction
May destroy contiguous bones and resemble a neoplasm
2/3 in frontal sinus; also anterior ethmoid sinus
Micro: inflammatory cells and mucin lift epithelium of sinus and periosteum away from underlying bone; epithelium may undergo squamous metaplasia; extravasation of mucin into lamina propria with muciphages
Relatively common life-threatening fungal infection, associated with diabetic ketoacidosis, poor glycemic control or immunosuppression
Spreads rapidly across nerves and tissue planes to blood vessels of orbit and brain, causes thrombosis, hemorrhage and infarction
Member of phylum Zygomycota, class Zygomycetes, order Mucorales; found in high-organic matter and soil
Mortality rate of 48%
Case reports: 29 year old man post bone marrow transplant with psychosis and gingival lesion (Archives 2000;124:883)
Micro: broad nonseptate hyphae branching at 90 degrees, accompanied by numerous neutrophils and histiocytes within granulation tissue
Micro images: fungal hyphae in vessel lumen that infiltrate vessel wall; asexual structures (very rare in tissue)
Iatrogenic lipogranuloma after hemostatic packing of nasal cavity or paranasal sinuses with petrolatum based ointment and gauze
Resembles subcutaneous disease of East Africa
Micro: large tissue spaces with saclike structures containing brown spherules resembling Prototheca but actually clumped red blood
cells
Negative stains: GMS
Common; not neoplastic, but may fill entire nasal cavity or extend into cranial cavity or orbit
In children, must rule out cystic fibrosis
Often recur due to persistence of causative factors
Endoscopic images: gelatinous polyp #1; #2; #3; large polyp extending into oropharynx
Micro: edematous lamina propria with variable inflammatory infiltrate including eosinophils; subtypes include angiectatic (angiomatous), cystic, edematous, fibrous, glandular
Angiectatic polyps
Extensive vascular proliferation and ectasia with pseudoamyloid deposition that simulates malignancy
Due to marked reactive and reparative changes
May cause life threatening epistaxis, facial deformity or bone erosion and remodeling
Gross: gelatinous, semitranslucent masses with smooth, blue-gray glistening surface
Micro: dilated thin walled vessels in pools of eosinophilic material, associated with patchy necrosis and atypical spindle cells; associated with choanal polyps arising in paranasal sinuses and protruding into nasopharynx
Micro images: (1) proliferation of thin walled vascular channels; (2) thin walled vessels embedded in amorphous material (asterisks) or surrounded by amorphous material (arrows); (3) atypical stromal cells (arrows) and fibrin thrombi (right side-asterisk); (4) vascular channels lined by large hyperchromatic endothelial cells; also atypical stromal cells
DD: angiofibroma, hemangioma
References: Archives 2000;124:406
Antrochoanal polyp
4-6% of nasal polyps
Frequently occur in childhood
90% solitary
Arise from wall of maxillary antrum, extending through large primary or secondary maxillary ostium into nasal cavity
May pass into choanae or nasopharynx
Gross: long narrow stalk with firm, fibrous body
Gross images: long polyp extending from maxillary sinus into nasopharynx
Micro: thin surface mucosa with no thickened basement membrane; stroma with stellate cells, less edema and fewer glands than inflammatory polyp; may have prominent dilated vessels with thrombosis or infarct; prominent eosinophils in only 20%
Micro images: thin surface epithelium, edematous stroma with prominent dilated vessels and minimal inflammation #1; #2; #3
Atypical stromal cells
Common; have prominent cytoplasm, enlarged hyperchromatic nuclei resembling “radiation fibroblasts” or sarcoma
More common in younger patients or those with prominent fibrous stroma
No increased cellularity, no prominent vasculature, no mitotic figures
Resemble polyp on low power
Cystic fibrosis polyp
Must rule out cystic fibrosis in any child with nasal polyps
Present in 20% with cystic fibrosis
May arise up to 12 years before clinical diagnosis; rarely is initial manifestation of disease in adults
Micro: similar to inflammatory polyps, but no basement membrane thickening, no submucosal hyalinization, no/rare stromal eosinophils; may have large cystic glands with inspissated mucin in lumina; mucous glands, cysts and mucous contain predominantly acid mucin
Positive stains: purple-blue mucin with combined Alcian blue PAS stain (vs. red for inflammatory polyp)
Inflammatory polyp
Most common type of nasal polyp
Due to recurrent attacks of rhinitis (allergic, inflammatory)
Most people with polyps are NOT atopic; only 0.5% with atopy develop polyps
Usually ages 30 years or older (rarely < age 20 years)
Often recurs after surgery
Often associated with asthma, chronic rhinitis, aspirin intolerance (14%)
Gross: usually multiple and bilateral and involve nasal cavity and paranasal sinuses; have translucent, moist or edematous cut surface (opaque areas may represent papilloma); broad base of attachment is present; usually not destructive
Gross images: inflammatory polyps
Micro: respiratory epithelium, often with squamous metaplasia, edematous and loose stroma with hyperplastic mucous glands, inflammatory infiltrate (lymphocytes, plasma cells, eosinophils, neutrophils, mast cells); mucosa may be ulcerated or infected; basal membrane may be thickened; may have “bizarre” stromal cells (large and pleomorphic) due to reactive changes; may have prominent glandular component
Micro images: respiratory mucosa with edematous stroma with numerous eosinophils and plasma cells
Virtual slides: respiratory epithelium overlying edematous stroma with eosinophils and plasma cells
DD: papilloma
Benign process that resembles squamous cell carcinoma or mucoepidermoid carcinoma
Occurs after surgery on nose or sinuses
Micro: necrosis of seromucinous glands with secondary squamous metaplasia and reactive atypia
Micro images: various images
Possibly life-threatening nasopharyngeal infection caused by Bordetella pertussis
Causes progressive, repetitive paroxysmal coughing, mild systemic complaints and lymphocytosis
Worldwide causes 60 million severe cases and 1 million deaths annually
In US, 5000-7000 annual cases, deaths usually only in infants younger than 1 year
Outbreak in Wisconsin (USA) in 1993 with attack rates of 23% (ages 30-49 years) and 33% (ages 50+)
Vaccination widely given, but efficacy may wane 4 years after last dose
Diagnosis: culture requires 5-7 days, also PCR
Treatment: erythromycin (but some cases are resistant)
References: Archives 2002;126:173
Often secondary bacterial infection by Streptococcus pneumonia or Staphylococcus aureus
Severe infections occur in infants or adults with neutropenia, HIV, diabetes, antibiotics
Streptococcus pneumonia pharyngitis: treat with antibiotics to prevent subsequent glomerulonephritis or rheumatic fever
Viruses: rhinovirus, echovirus, adenovirus, respiratory syncytial virus, influenza
Also called scleroma
Rare; chronic granulomatous disease affecting nasal cavity (95-100%), nasopharynx (18-43%), larynx (15-40%), trachea (12%) or bronchi (2-7%) caused by Klebsiella rhinoscleromatis
Usually low socioeconomic environments of central/South America, Africa, Middle East, Philippines, India; rare in US (usually immigrants)
Most common in young adults
Initially nonspecific rhinitis, then purulent, fetid rhinorrhea with crusting; then granulomatous stage with blue-red granular nasal mucosa with intranasal rubbery nodules or polyps, epistaxis, anosmia, enlargement of uvula, anesthesia of soft palate, variable airway obstruction; finally sclerosis
Treatment: tetracycline or trim-sulfa, possibly steroids, surgery to correct scars or stenosis
Microbiology: MacConkey agar cultures are 50-60% sensitive; bacteria is gram negative, encapsulated, nonmotile diplobacillus, member of Enterobacteriaceae, not normal flora, infective via drops or contamination of material that is inhaled
Case reports: polypoid intranasal mass in 32 year old woman (Archives 2001;125:159)
Micro: initially inflamed granulation tissue; later hyperplastic mucosa with pseudoepitheliomatous squamous hyperplasia, granulomatous inflammation, foamy macrophages (Mikulicz cells containing bacteria) and plasma cells with Russell bodies; variable vasculitis and ulceration; late-fibrosis, lymphocytes and plasma cells but no Mikulicz cells
Positive stains: PAS, Giemsa, Steiner or Hotchkiss-McManus stains for gram negative bacteria
EM: large phagosomes containing bacilli and finely granular material (antibodies on bacterial surface and aggregates of bacterial mucopolysaccharides)
DD: Rosai-Dorfman disease, leprosy
Caused by Rhinosporidium seeberi, traditionally thought to be a fungus, but actually an aquatic protistan parasite (J Clin Microbiol 1999;37:2750, Emerg Infect Dis 2000;6:273, Indian J Med Microbiol 2002;20:119)
Endemic in southern India, Sri Lanka and emigrants from this region (Diagn Pathol 2006;1:25, Singapore Med J 2004;45:224); rare indigenous cases in US (South Med J 1996;89:65)
Natural aquatic habitat, transmitted through traumatized epithelium, most commonly in nose and eye, also skin, ear, genitals and rectum
Risk factors: bathing or working in stagnant water
Rarely presents with disseminated skin disease (Indian J Dermatol Venereol Leprol 2007;73:185, Indian J Dermatol Venereol Leprol 2001;67:332)
Not transmitted person to person.
Case reports: Case of the Week #97
Treatment: surgical excision, may recur; no effective medical treatment
Gross: hyperplastic, polypoid, red, granular masses of nasal cavity; yellow pinhead spots represent mature sporangia; superficial mucus is common
Micro: large (100 to 450 microns), thick walled sporangia with 1000+ endospores, each 6-10 microns, accompanied by a mixed inflammatory infiltrate; may not be present in all sections and may need additional sampling for diagnosis
Micro images: image #1; #2; #3
Positive stains: GMS, PAS
DD: myospherulosis (large tissue spaces with saclike structures containing brown spherules that resemble Prototheca but are actually clumped red blood cells, GMS negative), Coccidiodes immitis (smaller spherules: 30-60 microns, smaller endospores: 2-5 microns), often arthroconidia and hyphae
Acute sinusitis
Often preceded by acute or chronic rhinitis
Usually self limited or well controlled with supportive treatment
Acute invasive fungal sinusitis (fulminant invasive fungal sinusitis)
Often seen in immunocompromised or diabetic patients, often ocular involvement or intracranial extension, associated with progressive disease and death
Treatment: vigorous systemic antifungal therapy
Micro: vascular invasion by fungi is present and may cause thrombi; also neutrophils, eosinophils, tissue necrosis and hemorrhage
Chronic sinusitis
Mixed bacterial flora, develops after acute sinusitis
May produce osteomyelitis of orbit or thrombophlebitis of dural venous sinus
Most commonly in maxillary sinus, and preceded by periapical infection from floor of sinus
May need biopsy for accurate diagnosis
Noninvasive fungal disease is often Aspergillus
Treatment: surgery to control disease may be necessary
Gross: edematous mucosa which blocks drainage and may cause empyema of sinus
Micro: glandular hyperplasia, squamous metaplasia, basement membrane thickening, inflammatory cells including eosinophils, edema; bone may show thickening and remodeling with osteoblastic rimming and fibrosis of bone marrow spaces; presence of allergic mucin (lamellated collection of inspissated inflammatory debris) suggests fungal organisms
Chronic noninvasive fungal sinusitis
Also called indolent fungal sinusitis
Associated with diabetes, immunocompromise or endemic areas (Sudan, Saudi Arabia)
Micro: fungi with surrounding foreign body inflammatory response or granulomas and tissue invasion; no vascular invasion by definition
5% of patients have upper airway involvement, usually in nasal septum or inferior turbinate
Micro: granulomas without caseous necrosis
DD: tuberculosis
Micro: granulomas with amorphous area (residual injected substance) surrounded by palisading histiocytes
Rare; usually associated with cervical lymphadenopathy
Usually isolated infection and not secondary spread from pulmonary infection
Diagnosis: culture or PCR
Gross: ulcerated or polypoid lesion of nasal septum or inferior turbinate; variable septal perforation
Micro: poorly formed granulomas, usually no necrosis; organisms rare
Rapidly progressive condition involving nasal cavity, lungs and kidney
Biopsies of 5 mm or more improve diagnostic yield
Note: lymphocyte rich biopsies are unlikely to be Wegener’s and more likely represent lymphoma
Diagnostic for Wegener’s: granulomatous inflammation, necrosis and vasculitis, in addition to clinical involvement of lung or kidney; OR two of three microscopic features and lung and kidney involvement
Probable: two of three microscopic features and (lung or kidney involvement) but not both
Suggestive: one of three microscopic features and lung and kidney involvement
Suspicious: one of three microscopic features and (lung or kidney involvement) but not both
Nonspecific: no microscopic features, either lung or kidney involvement
Gross: early disease usually lacks destruction of cartilage or bone
Micro: leukocytoclastic vasculitis of arterioles, small arteries and veins with geographic necrosis surrounded by palisading histiocytes, variable poorly formed granulomas with minimal lymphocytes; variable giant cells; granulomas and giant cells are distant from vessels or adjacent/within vessel wall; also epithelial ulcers; vessel may be obliterated by inflammatory cells and thrombus and be difficult to identify without elastic stain; all stages of vasculitis are present (acute: neutrophils with fibrinoid necrosis; chronic/healed: narrowed or obliterated lumina with concentric rims of perivascular collagen); usually no significant lymphoid infiltrate
Positive stains: elastic stains to identify badly damaged vessels
Negative stains: EBV
DD: Churg-Strauss syndrome, NK/T cell lymphoma, idiopathic midline destructive disease, tuberculosis, lymphoma, eosinophilic angiocentric fibrosis, cocaine-related lesions, systemic lupus erythematosus, necrotizing vasculitis from other causes