Sinus, Nasal and Nasopharyngeal Tumours.

Overview

Malignant = Rare ~ 5% of all H&N malignancies.
- 1 per 100K.
OS remains poor.

Clinical Features

Non-specific. Often like chronic rhinosinusitis.
But unilateral is key.
Later orbit or CN ( Vi or Vii) invasion.

Classification.

EPITHELIAL
1. Benign
  - Sinonasal Papilloma Inverted Type (=1%)
  - Pleomorphic Adenoma
2. Malignant
  - Squamous Cell Carcinoma (=52%)
    - Keratinising SCC
    - Non-keratinising SCC
  - Sinonasal Undifferentiated Carcinoma SNUC
  - Neuroendocrine Carcinoma
  - Adenocarcinoma
    - Intestinal-type AC (=13%)
    - Non-intestinal type AC
  - Salivary Gland Type Carcinoma
    - Adenoid Cystic Carcinoma
    - Acinic Cell Carcinoma
    - Mucoepidermoid Carcinoma
SARCOMAS
- Rhabdomyosarcoma
- Angiosarcoma
- Chondrosarcoma
- Osteosarcoma
HAEMATOLYMPHOID TUMOURS
- Extranodal NK T-cell lymphoma
- DLBCL
- Extraosseous plasmacytoma
NEUROECTODERMAL / MELANOCYTIC TUMOURS
- Olfactory Neuroblastoma
- Mucosal malignant melanoma
METASTASES
- Renal
- Breast
- Lung
- Thyroid
- Prostate

Specific Tumours

Inverted Papilloma (IP)

Path: Ecto derived ciliated respiratory membrane (= Schneiderian membrane)
Leads to 3 types of papilloma =
1. Inverted (60%) : Benign & locally aggressive. +/- EBV/HPV ~20% have foci of Carcinoma.
2. Oncocytic
3. Fungiform

Demo: M 40-70.
Site: Lateral nasal wall in region of the middle turbinate / ethmoid recess.
grows into Max + Ethmoid sinuses.

Radiology
CT
- Solitary polyp => unilateral obstruction.
- Ca2+ = Common.
- Focal hyperostosis (65%). Often pear or plaque shaped.
MRI
- Convoluted Cribiform Pattern (CCP) 96% PPV.
- T2 = alt Inc/Dec bands.
PET
- FDG = High in Inverted and SCC degeneration.

Generally diff to tell IP and Ca even on histo due to foci of Ca.

Rx: Surgical Resection : mostly open, more endoscopic.
Recurrence: ~15%

Juvenile Angiofibroma

Path: Testosterone - sensitive myofibroblast.
Demo: Rare. Only M. If F then mosaic.
Locally invasive vascular tissue in fibrous stroma
Site: Sphenopalatine foramen => PPF
Spread:
- Medially => Nose & nasopharynx
- Laterally => Pterygomaxillary fissure => Infratemporal Fossa.
- Superiorly => IOF => Orbital apex
- Posteriorly => Vidian or Foramen Rotundem => MCF
Invades Sphenoid (60%), Maxillary (43%), Ethmoid (35%)

Radiology
CT = 2x characteristic features on CT
- Mass in Nose & PPF.
- Erosion of Bone behind Sphenopalatine foramen at root of medial pterygoid plate.
MR = Delineate full extent of tumour.
- T2 = Intermediate and heterogeneous signal with flow voids.
- Gd +++

Invasion into Sphenoid predicts recurrence.

Rx: Angio of ECA then resect.
Recurrence: 15-20% often in basisphenoid thus surveillance.
RT is used if unresectable.

AdenoCarcinoma AC

Demo: 3M:F 50-60 Risks= Wood 500-900x, Leather.
CF: Usual and epistaxis.
Path: From surface epithelium or minor salivary gland rests.
Intestinal-type Adenocarcinoma is most common type.
4 sub-types:
1. Colonic (40%)
2. Solid
3. Papillary
4. Mucinous / Mixed type.
Site:
- Ethmoid: 40%
- Nasal Cavity 30%
- Maxillary 25%
Radiology
- Poorly defined mass.
- Bone destruction > remodelling.
- Enhancement ++
- FDG useful for Met vs 1y AC
Prognosis:
- = f(grade)
- 8% LN at presentation.
- 13% distant mets at presentation.
- 5y OS = 50%. 50% will recur => RIP.

Sinonasal Undifferentiated Carcinoma SNUC

Demo: 3M:F 8-85y. x= 50s. Rare.
Path: Uncertain histiogenesis.
Site: Nasal cavity => Ethmoid > Maxillary cavity.
Radiology:
- Large (>4cm)
- Invasive +
- Bone Destruction ++
Prog:
- 25% LN at presentation.
- Mets -> Bone, Brain & liver.
- 5y OS = 35%

Minor Salivary Gland Tumours

Adenoid Cystic Carcinoma = most common.

Demo: 20-91y
CF: Occ Facial numbness due to V2 invasion.
Path:
- Low Grade, thus insidious, thus 2/3 T4 at presentation.
- 50% have perineural spread.
Site
- 60% Maxillary sinus.
- 25% Nasal Cavity
Radiology:
- Low grade = Solid, enhancing, well-defined mass +/- bone remodelling (?polyps)
- High grade = heterogeneous, enhancing, poorly defined mass with bone Destruction
- FDG = Low uptake.

Olfactory Neuroblastoma ON

Demo: Rare=2% of Sinonasal Tumours, 2-90y x= 40y.
Path: Olfactory mucosa, thus Cribiform plate but also ectopic = lower nasal cavity or sinuses.Rarely intracranial.
CF: Occ paraneoplastic inc Cushings & SIADH.
Staging: KADISH
- A:In nasal cavity.
- B: Nasal cavity & Sinuses.
- C: Beyond Sinonasal Cavity.
- D:Regional nodes (6%) or mets (7%)
Radiology:
- Dumb-bell shaped in superior nasal cavity with waist at cribiform plate.
- Bone-remodelling > Destruction
- T1:Low/Int T2: Int/High. Occ cystic from necrosis.
- Enhance: +++ homogeneous.
- FDG: Often used as upstages 36%.
- FU: Gd MRI inc Neck = 4m for 2y, 6m for 2-5y, then 9m for life.
Prognosis:
- 5/10y OS = 62% / 46%. - 10y OS for A = 83% but D = 13%
- Local recurrence upto 60%
- Mets -> Brain, dura, lung, liver, parotid, bone, cord, skin in 15% at usually 1-20 months post but can be upto 10ys
Rx:
- Craniofacial resection & RT and neoadj Platinum chemoRx
- No neck dissection unless indicated.

Mucosal Melanoma MMHN

Demo: M=F 60s. Rare = 4% of SN tumours & <1% of all MMs(but 8% in Japan). Risks: Formaldehyde
Path: Neuroectodermal
- No ref point to measure invasion thus all are T3+
- Sites: 52% Nasal cavity, 20% Paranasal sinuses.
- 15% Regional Nodes at presentation. <10% Mets. - 50% End up with distant mets.
Radiology:
- Often polypoid.
- T1 High / T2 low
- Amelanotic = Intermediate mostly
- Gd +++
Rx: Surgery not RT. Then immunotherapy. RT for recurrences occasionally
Prognosis: 5yOS = 30%

Sinonasal NHL

Demo: 3M:F x=53y for NK Tcell. M:F x=63y for DLBCL. 14% of all SN tumours.
CF: Mostly local signs +/- B symptoms.
Rare in NK T-cell is Haemophagocytic syndrome with pancytopenia.
Path: 2 main types:
- DLBCL = Most
- NK / T-cell lymphoma = in Asia / S. America . 100% EBV but role not clear.
Radiology
- CT: High. But variable.
- Gd++
- Bone destruction is common.
- FDG: High for both.

Sinonasal Chondrosarcoma

Demo: Rare 0.1% of all H+N, but most common sarcoma. M>F 60s.
Radiology
- CT: 'Rings and arcs' Ca2+ with Low matrix.
- MR: Matrix is High T2.
- Gd: +/++
Prognosis Good but late recurrence is common.

Sinonasal Osteosarcoma

Demo: M:F 20-30
Path:
- Site: Jaw > Maxilla (alveolar ridge)
Radiology
- Osteoid matrix
- Sunburst periosteal reaction.
- T1 = look for marrow infiltration.

Tumour Table

Name	Demo	CF	Path	Radiology	Other
Inverted Papilloma	M 40-70	Nothing specific	Ecto derived ciliated respiratory membrane (Schneiderian) => 3 types of papilloma. Inverted is 60%. Benign and locally aggressive. 20% foci of Ca. Site: Lateral nasal wall near middle turbinate or ethnoid recess. => Max / Ethm sinuses.	CT: Solitary Polyp Ca2+ = Common Focal hyperostosis, often pear/plaque shaped. MRI: Convoluted Cribiform Pattern (CCP) 96% PPV T2 = alternating Inc/Dec bands. FDG: High in IPs & SCC degen.	Recurrence = 15% Rx = Surgical Resection