Familial Giant Cell Lesions of the Mandible-Unusual Occurence

Thursday, October 10, 2013
Khari K. Lewis , Royal Sussex County Hospital, Brighton, United Kingdom
Jim Herold , Royal Sussex County Hospital, Brighton, United Kingdom
Giant Cell Granuloma (GCG) is an uncommon bony lesion in the head and neck region, most commonly affecting the maxilla and mandible with a female predilection. The clinical behaviour of central Giant Cell Granulomas ranges from a slowly growing asymptomatic swelling to locally destructive aggressive lesions. Hereditary giant cell lesions of the facial skeleton, in the absence of a clinical syndrome, are rare.

 Materials and Methods:

A twenty year old female with relevant medical history attended the maxillofacial surgery department with a persistent swelling of the right posterior mandible. This had displaced the lower right second premolar. The patient reported a family history of tumours of the lower jaw wit her mother and maternal uncle having surgery in the past. In view of her family history, serum parathyroid hormone levels and bone chemistry were performed on mother and daughter. The parathyroid hormone levels, calcium and phosphate levels were all within normal limits. Imaging of the neck showed no pathology of the thyroid and parathyroid glands. The patient underwent curettage of the lesion in the lower right 56 region under general anaesthesia.

 Results:

Histopathology reported giant cell lesion/ benign fibro-osseus lesion. A second opinion from the Royal National Orthopaedic Hospital, London of the patient pathology stated the lesion was a giant cell granuloma. We also obtained a second opinion of the patient’s mother’s histopathology from the Royal National Orthopaedic Hospital of biopsies from 2000 and 2011 and reported similar features to that of the daughter. The histopathology of the maternal uncle was also giant cell granuloma and remains recurrnec frr 15 years post-op.   The patients mother had surgical curettage in 2000 with a second procedure in 2011 for recurrence. No evidence of recurrence 2 years post treatment.  The patient remains recurrence free 2 yrs post-op.

Conclusion :

Familial Giant cell lesions of the mandible are rare. It is important to rule out other differential diagnoses including Hereditary Hyperparathyroidism jaw tumour syndrome

References:

Kutcher MR, Rigby MH, Bullock M, Trites J, Taylor SM, Hart RD. Hyperparathyroidism jaw tumour syndrome Head Neck. 2012 Feb 2. doi: 10.1002/hed.22918.

2011 Dec;5(4):385-8. doi: 10.1007/s12105-011-0297-4. Epub 2011 Sep 4.
 
 
Valentine JC, Nelson BL. Central Giant Cell lesion. 2011 Dec;5(4):385-8.