Ghost cell odontogenic carcinoma.

Ghost cell odontogenic carcinoma (GCOC) is the malignant counterpart of calcifying cystic odontogenic tumour and dentinogenic ghost cell tumour. This is the case of a middle-aged male who presented with a slow-growing maxillary tumour. He was asymptomatic until pain symptoms developed prior to initial presentation. The excised tumour was diagnosed as a ghost cell odontogenic carcinoma. More case reports are needed for further understanding of this rare malignant odontogenic tumour.

Leiomyosarcomas of the oral cavity. Report of four cases and review of the literature.

Leiomyosarcomas (LMS) make up 7% of all soft tissue sarcomas. In the oral cavity, the LMS is rare due to the paucity of smooth muscle in that region. Four cases of intraoral LMS are reported, plus 34 cases found in the literature reviewed. There were 24 males and 14 females. The age range at presentation was 10 months to 88 years with no predilection for any particular age group. The commonest presenting symptom was a mass. The intraoral LMS occurred most commonly in the jaws (59% of cases). The mass was painful in 61% of cases. Follow-up of the case reports was assessed. Recurrence occurred in 36% of cases. Distant metastases occurred in 39% of cases, most commonly to the lungs. Cervical nodal metastasis was reported in 15% of cases. The 5-year survival rate determined on cases with adequate follow-up (n = 13) was 23% free of disease, 8% alive with disease and 69% who died of disease. In comparison with stage I and stage II tongue squamous cell carcinomas, the intraoral LMS is very aggressive. It is best treated surgically, early and aggressively.

Multiple endocrine neoplasia syndrome--type 2b. Case report and review.

The multiple endocrine neoplasia syndromes are an association of tumours of 2 or more endocrine glands. Multiple endocrine neoplasia type 2b (MEN 2b) patients develop medullary thyroid carcinoma and pheochromocytomas as well as unique physical characteristics. Most commonly, MEN2b is inherited with an autosomal dominant pattern although sporadic cases are not uncommon. If untreated the disease may be lethal. The facial, oral and ocular characteristics are reliable markers of the disease. These patients give a history most commonly of slipped capital femoral epiphysis, hypertension and life-long diarrhoea and/or constipation. MEN2b is most commonly characterised by nodules on the anterior aspect of the tongue, thickened lips with nodules, thickened upper eyelids, broadened nasal bridge, thickened corneal nerves and dilated, symmetrical, pedunculated nodules on the cheek mucosa. The patient described has most of these characteristics. Radiographic features of the jaws which have not been previously described are reported. These include a markedly enlarged and bifurcated inferior alveolar canal and shortened roots of the lower incisor teeth. Due to the lethality of the disease, patients who present with the above physical characteristics must be further investigated to exclude MEN2b.