Flashcards in Pathology of ENT Deck (24):
What is a vestibular schwannoma?
This is a benign primary intracranial tumor of the myelin-forming cells of the vestibulocochlear nerve (8th cranial nerve)
What are the clinical features of a vestibular schwannoma/acoustic neuroma?
-what to consider if bilateral and young?
• Assoc. with vestibular portion of the vestibulocochlear nerve
• Occurs within the temporal bone
• Represents 80-90% of cerebellopontine angle tumours
• 95% are sporadic and unilateral.
• Equal gender distribution
• If bilateral and young – consider neurofibromatosis (NF) type 2
What are the signs/symptoms of vestibular schwannoma?
Unilateral sensorineural hearing loss!Acoustic neuroma until proven otherwise.
Impaired facial sensation (involvement of CN V)
Ataxia (likely CPA mass)
Signs of increased ICP
What are the investigations for vestibular sschwannoma?
Audiology to test for hearing loss, and MRI (much better than CT in this case)
What is the management of vestibular schwannoma?
Watch and monitor
Although sitting back and watching may sound too conservative, a recent study showed that only 4% of small neuromas actually showed rapid growth over a period of time. If the patient is asymptomatic then avoiding the 1% mortality risks associated with surgery may be the best option. As with most neurosurgery other risks include; meningitis, hearing loss, stroke and epilepsy.
What is NF1 - what is seen?
¥ 1:3000. Autosomal dominant. Widespread neurofibromas. Bony defects, café au lait spots, axillary freckling, Lisch nodules
¥ NF-1 gene encode neurofibromin
What is NF2 - what is seen?
¥ AD but usually sporadic mutation. 1:40000 incidence. NF-2 gene encodes merlin protein at Ch22q12
¥ Bilateral vestibular schwannoma
¥ Multiple meningiomas
¥ Café au lait
Granulomatosis with polyangitis:
-what is this?
¥ Autoimmune disorder of unkown aetiology characterised by a small vessel vasculitis limited to respiratory tract and kidneys
What are the clinical features of granulomatosis with polyangitis?
¥ Rare. Usually white patients and >40years old.
¥ Present with pulmonary, renal disease or nasal symptoms of congestion, septal perforation etc.
What is the diagnosis of granulomatosis with polyangitis?
¥ Characterised by high frequency of anti-neutrophil antibody levels – ANCA +ve
¥ pANCA – microscopic polyangiitis
¥ cANCA – Wegener’s
What benign and malignant lesions are found in the nose?
¥ Relatively rare
¥ Benign lesions – squamous papillomas, “Schneiderian” papillomas. Angiofibromas
¥ Malignant lesions – Most commonly Squamous cell carcinoma
¥ Also primary adenocarcinoma, nasopharyngeal carcinoma, neuroblastoma, lymphoma
Nasopharyngeal carcinoma - what is the epidemiology?
¥ Low incidence in UK. Very high in far east.
¥ Strong association with Epstein Barr virus as well as volatile nitrosamines in food
Describe how EBV is implicated in carcinogenesis?
¥ Seen in association with Burkitt’s lymphoma, other B-cell lymphomas and Hodgkin’s lymphoma
¥ Infects epithelial cells of oropharynx and B-cells
¥ Ubiquitous infection – most subclinical. Occasional infectious mononucleosis
¥ EBV hi-jacks and mimicks helper T-cell responses leading to proliferation and survival of B-cells. Mediated largely by latent membrane protein 1 (LMP-1). EBV encodes EBNA-2 activating cyclin D and promoting transition from G0 to G1.
-are these common?
-what is this?
-what is this caused by?
¥ Infrequent - <1% of population.
¥ Reactive change in laryngeal mucosa secondary to - vocal abuse, infection and smoking. Occ. in hypothyroidism.
¥ Nodules and polyps are largely interchangeable terms. Nodules are usually seen in young women and are bilateral on middle 1/3 to posterior 1/3 on vocal cord. Polyps are unilateral and pedunculated.
What is a paraganglioma?
¥ Tumours arising in clusters of neuroendocrine cells dispersed throughout the body.
What are the two different types of paraganglioma?
¥ Chromaffin positive – sympathetic nervous system and can secrete catecholamines. Usually adrenal medulla or paravertebral – organ of Zuckerkandl
¥ Non-chromaffin – carotid bodies, aortic bodies, jugulotympanic ganglia, ganglia nodosum of vagus and clusters around oral cavity, nose, nasopharynx, larynx and orbit.
What are the clinical features of paraganglioma?
¥ Rare tumours
¥ Can arise at any age but usually >50
¥ Can occur as part of multiple endocrine neoplasia 2. Autosomal dominant – usually multiple and a higher incidence of malignant forms
What type of carcinoma is seen in the head and neck? what are the risk factors for this?
Squamous cell carcinoma
¥ Common tumour in head and neck – nose, sinuses, pharynx, larynx and oral cavity.
¥ Risk factors – majority relate to smoking and alcohol
¥ Also tumours related to human papilloma virus (HPV). Largely in the oropharynx.
What are the general rules for salivary gland tumours?
¥ Parotid most common site for almost all tumours
¥ Tumours in smaller glands are more likely to be malignant
¥ If young and a painful mass – think malignant
What are the different types of salivary gland tumours?
¥ Pleomorphic adenoma - most common
¥ Warthin’s tumour - second most common
¥ Adenoid cystic
- who is affected?
¥ Most common tumour
¥ Usually females over 60 in parotid with a long history.
¥ Can occur at any age in any gland.
¥ Difficult to excise – recurrence
¥ If longstanding, risk of malignant transformation
-who is affected?
-where does this occur?
¥ Second most common benign tumour
¥ Usually males over 50. Rare outwith the parotid. Strong association with smoking. Often bilateral and multicentric.
-which type is the most common worldwide?
-which type is most common in the UK?
¥ Worldwide the most common is a mucoepidermoid carcioma
¥ In the UK adenoid cystic carcinoma