Clinical Scenario

A 60‐year‐old man is referred to you for an urgent assessment by his medical practitioner. The doctor was examining the patient’s throat at the homeless shelter, when he coincidentally noticed a large swelling in the upper left quadrant of the mouth which he is worried may be caused by a cancer. The patient is not aware of the area and has no associated symptoms.

Structured Learning

1. What would you include in your differential diagnosis for the maxillary enlargement in the upper left quadrant?
   

   

   • Paget disease of the bone
   • Fibrous dysplasia
   • Osteoma
   • Osteosarcoma (occurs in less than 1% of patients with Paget disease)
2. There do not appear to be any signs of infection. Why does the area of maxillary enlargement feel warm to the touch?
   • Paget disease is associated with increased vascularity when bone turnover is high
3. How may the patient’s recent symptoms of headaches and loss of hearing be related to his Paget disease?
   • Neurological complications occur in the majority of patients over the course of the disease
   • Mechanical compression or reduced blood flow of neural tissue may result in these symptoms
   • This patient has radiologically confirmed skull involvement of Paget disease, and hence neurological symptoms are more likely
   • Cranial involvement is hence likely and may lead to various types of headache, hearing loss or other cranial nerves deficits, hyperaemia of skull and basilar invagination and its consequences
4. You explain to the patient that you need to investigate further and undertake an orthopantomogram. What features on an orthopantomogram would be suggestive of Paget disease?
   • Cotton wool appearance of the bone caused by irregular areas of focal osteosclerosis
   • Loss of lamina dura, hypercementosis, resorption, replacement of tooth roots by bone
5. What factors are considered important in assessing the risk of managing this patient?
   • Social
     • Homeless – no fixed address or contact details
     • Lack of financial resources has led to lack of access to regular healthcare (medical and dental)
     • No family/next of kin/escort
   • Medical
     • Paget disease has been untreated and unmonitored for ~5 years
     • Recent development of headaches/hearing loss
     • Malnutrition may be present due to the lack of financial resources
   • Dental
     • Unable to regularly access facilities and oral healthcare aids which allow him to brush his teeth
     • History of bisphosphonate use associated with the risk of medication‐related osteonecrosis of the jaw (MRONJ) (see Chapter 16.2)
6. The patient asks if you can biopsy the area on same day. How would you respond?
   • Oral cancer is not your primary diagnosis
   • Consultation with a physician is required
   • Further bone scans and blood tests are advisable (e.g. serum alkaline phosphatase levels)
   • A bone biopsy may not be required if the investigations confirm that Paget disease is the likely cause of the maxillary enlargement
   • If an incisional biopsy of the region is still considered necessary, it should be undertaken in a hospital setting as there is increased risk due to:
     • Hypervascularity and hence risk of increased bleeding
     • Risk of MRONJ (see Chapter 16.2
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