The nose (Figure 4.7) and paranasal sinuses are the most com- monly involved organs, but any part of the body can be affected, mainly the lungs. It can start simply with nasal congestion, crust- ing, epistaxis or facial pain. If untreated, it progresses rapidly to involve other parts, and can be fatal. The symptoms depend on the organ involved, but the patient is generally unwell and pyrexial, with weight loss. Nasal examination reveals thickened mucosa, ulceration, crusting, septal perforation or nasal collapse.
Sarcoidosis
This is also a multisystem disease that can affect many parts of the body but often has ENT manifestations. A ‘strawberry
Causes of granulomatous conditions Infective
Bacterial Tuberculosis and atypical mycobacteria Syphilis Actinomycosis Rhinoscleroma Leprosy Fungal Aspergillus Phyco- or mucormycosis Rhinosporidiosis Histoplasmosis Parasitic Leishmaniasis Toxoplasmosis
Traumatic Pyogenic granuloma Foreign body granuloma
Inflammatory Wegener’s granulomatosis Sarcoidosis
Cholesterol granuloma Eosinophilic granuloma Churg–Strauss syndrome
Neoplastic T-cell lymphoma
skin’ appearance of the nasal mucosa has been described and congestion, crusting and bleeding are common. A purulent discharge is a feature of sec-
ondary infection. A mass can be seen externally when the disease penetrates and de- stroys the nasal bones (lupus pernio).
Investigation
The key is to have a low level of suspicion and question gen- eralised symptoms.
• Blood tests include FBC, urea and electrolytes, liver function tests, erythrocyte sedimentation rate, C-reactive protein, c-ANCA for Wegener’s granulomatosis and serum ACE levels for sarcoidosis
• Culture and sensitivity when infective causes are suspected • Chest X-ray and lung function test for pulmonary involve-
ment
• Urinalysis for renal involvement
• Biopsy taken from affected nasal mucosa shows non- caseating granulomatous inflammation
• Cross-sectional imaging is useful in assessing the extent of the disease and for surgical planning.
Figure 4.7 Wegner’s granulomatosis affecting the nose and surrounding area. The dye marks the area where the skin biopsy was taken.
Nasal crusting, especially with bloodstained discharge, should alert one to the possibility of granulomatous inflammation and prompt appropriate investigations.
Rhinology Sinonasal tumours 145
Management
Management is multidisciplinary, involving the relevant medi- cal specialties.
• Rheumatologists treat the inflammatory conditions with a mixture of steroids and various immune-modulating agents
• Antimicrobials are used to treat the infective causes and secondary infections
• Symptomatic treatment of nasal symptoms, especially crusting, includes saline douches, topical creams and steroids
• Surgery is generally to be avoided in active We- gener’s granulomatosis and sarcoidosis, except for taking biopsies or decrust- ing, but is effective in for- eign body, pyogenic and cholesterol granulomas.
Prognosis
Patients with Wegener’s granulomatosis and sarcoidosis will require lifelong treatment, with considerable side effects or relapses. Kidney, eye, CNS and heart involvement carries a poor prognosis. Neoplastic aetiologies require radical treatment, and the prognosis depends highly on the stage at presentation. In- fective and traumatic causes in general carry a better outcome.
4.8 Sinonasal tumours
These are benign or malignant neoplasms of the nose and sinuses. They may be epithelial or non-epithelial in origin.
Epidemiology
Malignant sinonasal tumours are rare and comprise less than 10% of head and neck cancers. The incidence is less than 1 in Granulomatous conditions affecting the nose can be locally destructive leading to nasal collapse and septal perforation. Rhinoplasty surgery for nasal augmentation or closure of a septal perforation should not be considered until the disease has remained inactive for several years.
100 000. In males it is double that in females, and patients often present in the fifth decade of life. Fifty per cent of nasal cavity tumours are benign and 50% are malignant. The majority of paranasal sinus tumours are malignant.
Causes and pathogenesis
The sinonasal region exhibits a wide diversity of tumour subtypes compared to other regions in the body (Table 4.8). Adenocarcinoma of the ethmoid sinuses is related to the hardwood industry and leather tanning chemicals. Other carcinogenic agents implicated include nickel refining fumes, mineral oils, radium, lacquer paint, chromium and cigarette smoking. Epstein–Barr virus may be responsible in the aetiol- ogy of nasopharyngeal malignancy. Dietary factors have been implicated in the high prevalence of nasopharyngeal malignan- cies in southern China.
Clinical features
Sinonasal tumours present with nasal blockage (especially unilateral), epistaxis, anosmia, pain and nasal discharge. Diplopia is a serious feature suggesting orbital involvement.
Epithelial Nonepithelial Lymphoreticular tumors Squamous cell carcinoma (SCC) Transitional cell carcinoma (TCC) Adenocarcinoma Adenoid cystic carcinoma Melanoma Olfactory neuroblastoma Undifferentiated carcinoma Soft-tissue sarcoma Rhabdomyosarcoma Leiomyosarcoma Fibrosarcoma Liposarcoma Angiosarcoma Myxosarcoma Hemangiopericytoma Connective tissue sarcoma Chondrosarcoma Osteosarcoma Synovial sarcoma Lymphoma Plasmacytoma Giant cell tumor Metastatic carcinoma
Rhinology Sinonasal tumours 147 A full examination is necessary, including the face, oral cavity, and oropharynx, with anterior rhinoscopy, neck examination and examination of the nasal cavity with a flexible nasendo- scope or rigid Hopkins rod telescope. Particular attention is paid to unilateral nasal polypi/masses. It is necessary to assess and document their size, surface, colour and position in the nose.
Investigation
Plain radiography has been superseded by CT scans of the nose and paranasal sinuses. These can be particularly valu- able in suspected malignancy when combined with MRI, as this helps differentiate fluid from solid matter (Figure 4.8). All unilateral masses in the nose and sinuses must be biopsied and assessed by examination under anaesthesia, or by endo- scopic sinus surgery for histology if the sinuses are involved.
Management
Management depends on the histology of the tumour.