the british society for oral and maxillofacial pathology slide seminar - belfast 2008 nose and...

The British Society for Oral and Maxillofacial

Pathology

Slide Seminar - Belfast 2008Nose and Paranasal Sinuses

The British Society for Oral and Maxillofacial Pathology

•Newcastle PR7250/08•Male 81 years•Right nasal polyp extending

into posterior channel•? Inverted papilloma

PANEL DIAGNOSIS•Schneiderian papilloma

(oncocytic type)•Granulomatous inflammation

(significance uncertain)

FINAL DIAGNOSIS•Schneiderian papilloma

(oncocytic type)•Granulomatous inflammation

(no systemic disease)

SUGGESTED DIAGNOSIS• Schneiderian papilloma with

granulomas (sarcoid, reactive, other) x 2

• Granulomatous inflammation x 2• Inflammatory nasal polyp,

oncocytic change

• East Grinstead 293/07• Male 82 years• Nasal mass, ?recurrence from 2

years previously, treated DXT• Tumour filling nasal cavity but

not penetrating cribriform plate

Panel Diagnosis: adenocarcinoma

• Intestinal type, colonic (mod differentiated)

• Should be CK20 positive, focal CK7, endocrine cells also

• Was original the same?

SUGGESTED DIAGNOSIS•Sinonasal adenocarcinoma,

intestinal type x 4•?Possible metastasis•Adenocarcinoma

Adenocarcinomas of nasal cavity WHO 2005

• Salivary• Intestinal type, Barnes classification papillary: very low grade colonic: mod differentiated solid mucinous mixed• Non-intestinal type ( low grade: no necrosis; high

grade: solid growth)

FINAL DIAGNOSIS• Adenocarcinoma of Intestinal type

• Glasgow 07/2454• Male 12 years• Short history of obstruction (R)

nasal passage• Focal bone loss lateral wall• ? Nasal polyp but a bit odd

DIFFERENTIAL DIAGNOSIS•Sinonasal

haemangiopericyoma•Paraganglioma•Meningioma

PANEL DIAGNOSIS•Sinonasal

haemangiopericytoma

(probably…)

SUGGESTED DIAGNOSES•Nasal paraganglioma x 2•Alveolar rhabdomyosarcoma•Meningioma

• Dr R Reid fell into “falls into spectrum of haemangiopericyoma/glomus tumour”

• Actin, FXIIIA +ve; CD34, Bcl2, FVIII –ve but variable

• Slow-growing• Mit>4/10 HPF, necrosis, pleo++,

>5cm in more malignant cases

•Belfast J07/14723• Male 50 years• Polyp from right maxillary sinus• ? Simple inflammatory polyp

DIFFERENTIAL DIAGNOSIS•Clump of altered mucus•Mycetoma•Allergic fungal sinusitis

SUGGESTED DIAGNOSES• “Snotoma” x 2

– A few fungal hyphae• Fungal sinusitis• Slough, can’t see anything else• Eosinophilic goo with Charcot-

Leyden crystals

PANEL DIAGNOSIS•Allergic fungal sinusitis

CLINICAL FEATURES of AFRS

• First described by Safirstein in 1976• Accounts for 5-10% of chronic rhinosinusitis cases• More common in warm and humid climates• Adolescents and young adult males• Unilateral nasal obstruction• Nasal crusts and coloured nasal secretions• Nasal polyposis later• Unresponsive to antihistamines, antibiotics and topical nasal

steroids• Initially responds to systemic steroids but then relapses• Can cause proptosis, telecanthus and intracranial extension

RADIOLGICAL FEATURES of AFRS

• Opaque antrum/ethmoids with mucocele formation on CT• Often unilateral• Expansile nature with bone erosion in 98% of cases• Dura and periorbita not involved• High attenuation areas• Calcium and heavy metal deposits• MRI features

– Central hypointesity on T1

– Signal void on T2

– Peripheral enhancement

Allergic Fungal SinusitisBent and Kuhn criteria (1994)

1. Type -1 hypersensitivity

2. Nasal polyposis

3. Characteristic CT findings

4. Eosinophilic mucus without fungal invasion

5. Positive fungal stain of surgically removed tissue +/- +ve fungal culture

ALLERGIC FUNGAL RHINOSINUSITIS(AFRS)

• DEMATIACEOUS FUNGI ( contain melanin in the cell wall)– Bipolaris– Alternaria– Cladosporium– Curvularia– Drechslera

• HYALINE MOULDS– Aspergillus

– Fusarium

• ZYGOMYCETES– Mucor

– Rhizopus

LABORATORY and PATHOLOGICAL features of AFRS

• Elevated IgE levels, typically in excess of 1000U/ml• RAST +ve for fungal antigens

• Thick, tenacious highly viscous mucin containing:– Noninvasive branching fungal hyphae

– Fontana-Mason stain good for Dematiaceous fungi

– Eosinophils

– Charcot-Leyden crystals

• Fungal cultures may be negative, if positive they don’t necessarily prove AFRS

ALLERGIC FUNGAL SINUSITIS• HSI (IgE) and HSIII (IgG) reactions• Impacted “allergic” mucin in sinus(es)

– Laminated, eosinophils++, CL crystals

• Many species of fungus– Aspergillus, Bipolaris, Curvularia

• Suggest AFS even if no hyphae?

FINAL DIAGNOSIS•Allergic Fungal Sinusitis

•Raigmore RP6547/95•Male 43 years •Nasal polyps ? Inverted

papilloma•No preoperative radiology

DIFFERENTIAL DIAGNOSIS•Ameloblastoma•Craniopharyngioma

PANEL DIAGNOSIS•Ameloblastoma

– Imaging–Clinical history

SUGGESTED DIAGNOSES•Ameloblastoma x 4•Craniopharyngioma

FINAL DIAGNOSIS•Craniopharyngioma•Arose high in posterior aspect

of nasal cavity

• Dublin 16877/07• Female 36• Nasal obstruction developed

during pregnancy. Large maxillary tumour, erosion of roots. ?Malignant

PANEL DIAGNOSIS•Fibro-osseous lesion

SUGGESTED DIAGNOSES• Fibro-osseous fibrous dysplasia• Ossifying fibroma• Bland spindle cell tumour ?type• Solitary fibrous tumour or Neurofibroma –

markers• Pseudosarcomatous giant cell tumour

FINAL DIAGNOSIS•Ossifying Fibroma

•Newcastle PR9644/08•Male 37 years•Previously fit, one month

right-sided headache•Bilateral papilloedema

•CT – Enhancing lesion arising in the ethmoidal sinus extending into the right frontal lobe

PANEL DIAGNOSIS•Nasal teratocarcinosarcoma

(atypical teratoid rhabdoid tumour)

SUGGESTED DIAGNOSES• Malignant blue cell tumour – olfactory

neuroblastoma• Embryonal germ cell with rhabdomyo

differentiation• Rhabdomyosarcoma with dedifferatiation vs.

carcinosarcoma• Embryonal rhabdomyosarcoma• Tumour ?what

FINAL DIAGNOSIS•Nasal teratocarcinosarcoma•3 year survival <30%

•King’s/Guy’s 595/08•Male 45 years•Right ulcerated nasal mass

with referred otalgia•?Carcinoma, Wegener’s or TB

• 45 year old lady – Bangladeshi Origin

• 3 month h/o right otalgia

• MH unremarkable

• O/E: both ears normal

• Right nostril: fleshy, nodular mass seen

• Rest of ENT examination – normal

PANEL DIFFERENTIAL DIAGNOSIS• Infective: Respiratory scleroma,

Sporidiosis, Atypical Mycobacteria

• Myospherulosis• ?Immunosuppression• Wegener’s granulomatosis

PANEL DIAGNOSIS•Respiratory scleroma

–Warthin-Starry (Steiner)–Serology–Clinical history

SUGGESTED DIAGNOSES• V tough, looks inflammatory

rather than plasmacytoma• Kleb Rhino• Wegener’s granulomatosis• Exotic infection x 2

– ?Leishmaniasis

• FINAL DIAGNOSIS– Respiratory scleroma

• Referred to the infectious diseases team.

• Ciprofloxacin for 6 weeks

• ENT review (24/04/2008): significant regression of the mass and improvement of symptoms

• Further follow up arranged in 6 weeks

• Oslo 10408/99• Male 47 years• Teeth 15, 16 extracted due to severe

periodontitis 10 and 6 months previously. Now OAF 17 and sinus perforation. Tooth 17 extracted and thickened periosteum removed. ?Chronic sinusitis

PANEL DIAGNOSIS•Adenoid cystic carcinoma

SUGGESTED DIAGNOSES•Adenoid cystic carcinoma•Metastatic adenocarcinoma•Adenocarcinoma•Ameloblastoma

Oslo Case 10408/99Oslo Case 10408/99

Histological diagnosis:Histological diagnosis:Adenocarcinoma, moderately to well differentiated

Further CT head & neck Further CT head & neck information:information:

Perforation of the orbital floor

Posterior maxillary sinus wall destruction

Possible tumour invasion of the nasoethmoidal region

Muscular tumour invasion in the infratemporal fossa

Oslo Case 10408/99

Therapy:Therapy:

The tumour was inoperable, T4 NO MO

On the patient’s request, hemimaxillectomy was performed

Tumour positive margins

Bone and soft tissue transplant

Post-operative radiation therapy 64 Gy

Further follow upFurther follow up:: No recurrence 7 1/2 years post-

operatively

Several surgical corrective operations

5 1/2 years post-operatively osteoradionecrosis of posterior right mandibular region

Partial mandibular resection with bone reconstruction

Severe functional and cosmetic problems

Further plastic corrections not planned due to possible complications (infection, necrosis)

• Leicester PR11152/04• Female 40 years• Nasal discharge• Necrotising lesion of hard palate with

perforation• Destructive lesion of nasal cavity• ?Wegener’s ??SCC

Diagnosis – first immunos! keratin, S100, CD45, CD20, CD3, CD34, CD56, CD30,

EBV ( EBER), CD38, CD43, myeloperoxidase

• Most probably malignant lymphoma• Setting and morphology suggest extranodal

NK/T cell lymphoma, nasal type (CD3,CD56,EBER) (angiocentric T cell lymphoma REAL. Lethal midline granuloma)

• Epithelial hyperplasia, necrosis, admixed inflammatory cells and variable cytology typical

• Diffuse large B cell lymphoma• Granulocytic sarcoma (myeloid leukemia)• Atypical myeloma

PANEL DIAGNOSIS•NK/T cell lymphoma

SUGGESTED DIAGNOSES• Lymphoma (“midline lethal

granuloma”)• Probable lymphoma• TNK lymphoma• Malignant lymphoma T-cell• NK/T cell lymphoma

FINAL DIAGNOSIS• NK/T cell lymphoma

DIFFERENTIAL DIAGNOSIS•Non-Hodgkin’s Lymphoma

–NK/T-cell

•Could it be Churg-Strauss or Wegener’s?

SUGGESTED DIAGNOSES•Eosinophilic vasculitis•Eosinophilic angiocentric

fibrosis•Churg-Strauss > ALHE•Vasculitis

PANEL DIAGNOSIS•Non-Hodgkin’s Lymhoma

–NK/T-cell

(probably…)

NK/T-CELL LYMPHOMA•Commonest primary lymphoma•CD2, CD3 (cytopl), CD56 +ve•TIA1, granzymeB +ve•EBER +ve in 95%

FINAL DIAGNOSIS• Long history of systemic

involvement with vasculitic pattern, cANCA became +ve

• Responded intermittently to Cyclophosphamide and steroids

• Regarded as Wegener’s

•Glasgow 07/20582• Female 20 years• Bilateral nasal polyps

DIFFERENTIAL DIAGNOSIS•Follicular hyperplasia•? Cause

– Epstein-Barr Virus– Human Immunodeficiency Virus

PANEL DIAGNOSIS•Follicular hyperplasia•Probably EBV-driven…

SUGGESTED DIAGNOSES• ?Lymphoma ?Florid reactive• Exclude MZ lymphoma

– Underlying immune disorder• Follicular hyperplasia x 2

– ?ALPS, ?Selective IgA def., ??AIDS

• B9 lymphoid hyperplasia– B & T markers

FINAL DIAGNOSIS• Known case of Kartagener’s

syndrome• Mucociliary paralysis with situs

inversus• Similar lesions throughout

bronchial and URT

•N. Staffs 293786•Female 63 years•Left nasal mass

•MRI - Tumour roof of nasal cavity with enhancement of anterobasal medial aspect of frontal lobe just above level of posterior third orbital apex

PANEL DIAGNOSIS•Olfactory neuroblastoma

(Hyams grade 1)•Small blue round cell tumour

SUGGESTED DIAGNOSES• Olfactory neuroblastoma x 2

– Low grade

• Something cerebral – pinealoma, pituitary tumour, etc

• Neuroblastoma• MPNST

FINAL DIAGNOSIS• Olfactory neuroblastoma

– Low grade

•Newcastle PR41689/07•Male 22 years•Bleeding from nostril, visual

disturbance•Destructive tumour

PANEL DIAGNOSIS•Olfactory neuroblastoma•Small blue round cell tumour

SUGGESTED DIAGNOSES• Sinonasal undifferentiated CA• Clear cell pituitary something• Malignant carcinoid• ?Olfactory neuroblastoma x 2

•Ewing’s/PNET•Mesenchymal chondrosarcoma•Lymphoma•Malignant melanoma•Rhabdomyosarcoma•Monophasic synovial sarcoma

Immunohistochemistry • Synaptophysin, Chromogranin

Melan A, Desmin, Myo D1, CD45, cytokeratin panel, GFAP, SMActin -ve

• S100 patchy nuclear• CD99 ++

Cytogenetics

• FISH No 22q12 or 13q14• RT-PCR No 11;22 translocation• EWS/ERG fusion transcript +ve• t(21;22)(q22;q12) • FISH cryptic insertional fusion

FINAL DIAGNOSIS•Ewing’s sarcoma•Good response to five courses

of chemotherapy

• Sheffield 05/9498

• Female 14 years

• ?Inverted papilloma

Differential diagnosis CD99, CD45, Cd34, desmin, PAS, S100, CD43, keratin

• Ewing Family tumours: Extraskeletal Ewings/ PNET

• PNET more typically spindled and with rosettes• CD99, FLI1, EWS gene fusions due to chros translocation (11;22) (q24;q12), can be AE1/ AE3

positive • age typical, site unusual• Embryonal rhabomyosarcoma (desmin, can be

CD99 positive))• Leukemia/ lymphoblastic lymphoma (TdT,

CD79a)• (Olfactory/metastatic neuroblastoma CD99 neg)

Final diagnosis

• Morphology most like extraskeletal Ewings/PNET

• Need to exclude others

SUGGESTED DIAGNOSES• Ewing’s/PNET• Small cell carcinoma,

neuroendocrine type• PNET/Embryo.• High grade - ?lymphoma ??NPC

IHC Staining

CD99, BCL2 (+)ve strong

MNF116, AE1/3, Cam5.2 (+)ve variably

S100, desmin (+)ve focally

CD20, CD38, CD45, CD56,CD79a

• t(11,22) - (+)ve

• t(X,18) - (-)ve

• FINAL DIAGNOSIS = Ewing’s/PNET

• King’s/Guy’s 1892/07

• Female 49 years

• Mass in sphenoid sinus with bony erosion

DIFFERENTIAL DIAGNOSIS• Pituitary Adenoma• Other neuroendocrine neoplasm• Salivary-type adenocarcinoma

PANEL DIAGNOSIS• Pituitary Adenoma

SUGGESTED DIAGNOSES• Sinonasal papillary adenocarcinoma• Ectopic pituitary adenocarcinoma• Non-intestinal type adenocarcinoma• Probable lymphoma• Tumour ?what

FINAL DIAGNOSIS• Known pituitary adenoma• GH+ve on IHC• Ki-67 LI = 8%

PITUITARY ADENOMA• Classified on pattern of hormone IHC

– “acidophil adenoma of acromegaly” - OUT

• Large ones functionally silent

• “Invasive adenoma” carcinoma

• Necrosis, mitotic figures are rare

• North Staffordshire 2999178• Male 52 years• Large tumour in sphenoid sinus and

extending into nose • Large mass (L) parasellar region involving

the pituitary fossa, upper clivus, cavernous sinus and (L) Meckel’s cave, encircling the carotid artery

DIFFERENTIAL DIAGNOSIS•Pituitary adenoma•Olfactory neuroblastoma•Malignant melanoma• Other “sneaky” history

SUGGESTED DIAGNOSES• Trabecular• Neuroendocrine – prob. pituitary• Olfactory neuroblastoma x 2• Plasmacytoma with amyloid

– Serum Ig’s

PANEL DIAGNOSIS•Pituitary adenoma

(probably…)

FINAL DIAGNOSIS• Prolactinoma

• Referred for second opinion: Some pleomorphism but not regarded as malignant

(probably…)

• Dublin 4329/07 or 4950/07• Female 48 years• Surgery for olfactory neuroblastoma, grade

II. Due to have post-op XRT 3 months later when developed swelling lateral nasal skin and cheek large destructive nasal mass seen clinically and on imaging

• (Slides are of 1st or 2nd biopsy)

Postoperative course

• Uncomplicated initially

• While awaiting radiotherapy, approx 8 weeks post op, she began to develop hard swelling of the side of her nose and face adjacent to the scar

DIFFERENTIAL DIAGNOSIS• Sarcoma

– MPNST– NOS

• Melanoma??

PANEL DIAGNOSIS• Definitely either benign or

malignant

SUGGESTED DIAGNOSES• Fibrosarcoma• MFH-like tumour• High-grade sarcoma• Spindle cell sarcoma NOS• Neuromatous differentiation in

n’blastoma with bony metaplasia

Immunohistochemistry

• Keratin (MNF), EMA, smooth m actin, desmin, melan A, CD34, HMB45, S100, chromogranin, synaptophysin, CD56, ALK all negative.

• Vimentin and NSE positivity

Biopsy• Morphology suggests malignant lesion,

probably sarcoma• ? Dedifferentiation of olfactory neuroblastoma

• Proximity to surgery and the expected biologic behaviour of olfactory neuroblastoma raises possibility of post operative spindle cell lesion/ fibrous pseudotumour…..but no inflammatory cells, no myofibroblasts, necrosis unusual

Material sent to US by courier

… but delayed at Customs in JFK

Second opinions

1: Malignant, probably a sarcoma of neural origin (Prof Dervan)

2. Malignant, possibly unusual melanoma, await immunos (Prof Fletcher)

• Patient recalled

Lesion disappeared!

Final second opinion, Prof Fletcher

• “Atypical spindle cell proliferation, very worrisome for unclassified sarcoma”

• Difficult to accept reactive lesion on morphology, would like to know follow up

? Final diagnosis

• Olfactory neuroblastoma, with very atypical spindle cell pseudotumour

• Patient 1 year post radiotherapy for original findings of residual olfactory neuroblastoma, no recurrence

the british society for oral and maxillofacial pathology slide seminar - belfast 2008 nose and...

british society

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inverted papilloma slide

oncocytic change slide

solid growth slide

systemic disease slide

cribriform plate slide

intestinal type x

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