neoplasia syndrome. As the oral signs may precede the development of more serious
aspects of this condition (such as carcinoma of the thyroid), children presenting with
multiple lesions should be referred to an endocrinologist.
Orofacial granulomatosis
Orofacial granulomatosis (OFG) is not a tumour in the true sense nor a distinct
disease entity, but describes a clinical appearance. Typically there is diffuse swelling
of one or both lips and cheeks, folding of the buccal reflected mucosa and
occasionally gingival swelling and oral ulceration (1021HFig. 15.14 (a) and (b)). This may
represent a localized disturbance due to an allergic reaction to foodstuffs, toothpaste,
or even dental materials. Alternatively, the appearance may be due to an underlying
systemic condition such as sarcoidosis or Crohn's disease.
MELKERSSON-ROSSENTHAL SYNDROME
This is a condition that generally begins during childhood. It consists of chronic facial
swelling (usually the lips), facial nerve paralysis, and fissured (scrotal) tongue.
Malignant tumours of the oral soft tissues
EPITHELIAL TUMOURS
Malignant tumours of the oral epithelium, such as squamous-cell carcinoma, are rare
in children. Malignant salivary neoplasms are also uncommon, although
mucoepidermoid carcinomas have been reported in young patients.
LYMPHOMAS
Hodgkin's and non-Hodgkin's lymphomas have been reported in children; however,
they are relatively rare in the paediatric age group. An exception is Burkitt's
lymphoma, which is endemic in parts of Africa and occurs in those under 14 years of
age. Indeed, in these areas the condition accounts for almost half of all malignancy in
children. Burkitt's lymphoma is multifocal, but a jaw tumour (more often in the
maxilla) is often the presenting symptom. Burkitt's lymphoma is strongly linked to the
Epstein-Barr virus as a causal agent.
RHABDOMYOSARCOMAS
These malignant tumours of skeletal muscle present in patients around 9 to 12 years
of age. The usual site is the tongue. Metastases are common and the prognosis is poor.
1022H
Fig. 15.10 Squamous-cell papilloma in
a 9-year-old girl. (By kind permission
of Dental Update.)1023H
Fig. 15.11 A fibrous epulis in a 10-