Malignant peripheral nerve sheath tumour in a 12 years girl: a case report

Malignant peripheral nerve sheath tumours [MPNSTs] are rare, aggressive soft tissue sarcomas associated with poor prognosis, that most commonly affect patients aged 20 to 50 years, but have also been reported in children. The tumour is usually found in lower extremities and only 10% to 20% of all lesions occur in head and neck region thus making it a rare entity. Central involvement, particularly in the jawbones is quite unusual. There is little reported in literature on these tumors in Africa. Here we report a rare case of intraosseous MPNSTS occurring in the mandible in a 12-years old girl. The biological behavior and diagnostic challenges of this rare malignancy are discussed

Early and delayed clinical manifestation

Pyknodysostosis [PKD], also called Maroteaux-Lamy disease or Toulouse-Lautrec syndrome is a rare osteosclerosing skeletal disorder that has an autosomal recessive trait. It is characterized by short stature, brachycephaly, short stubby fingers, open cranial sutures and fontanelles, diffuse osteosclerosis with attendant multiple fractures of long bones and osteomyelitis of the jaw; but with rare visceral manifestations. In this article we present three cases of PKD with diverse classical clinico-radiological features. Worldwide PKD have been reported in 9-months to 55 years but remarkably, we have presented amongst the youngest diagnosed cases, at 8months with visceral manifestations. Understanding early and delayed clinic-radiological manifestations of PKD is very important as accurate diagnosis of PKD avoid misdiagnosing it as hydrocephalous, cleidocranial dysostosis and osteopetrosis as the conditions may resemble each other clinically and radiologically

Intra-oral minor salivary glands neoplasms; the pattern and management

To determine the relative frequency and distribution of various types of intra-oral minor salivary gland tumours and their treatment modalities. Retrospective descriptive study. Muhimbili National Hospital [MNH], TANZANIA; at the department of Oral Surgery and Oral pathology. From 1993 to 2008. [16 years]. Hospital based data The information was retrieved from files and included; age, gender as well as their location, histological types and treatment modalities. Microscopic slides of all cases were reviewed and where necessary paraffin sections were re-cut and subsequently stained by haematoxylin and eosin. Special stained such as Periodic acid Schiff and/or mucicarmine were also performed in controversial cases. Tumours were classified according to the World Health Organization's Histological Typing of salivary gland tumours. Data were entered in the computer and analysed by SPSS. Statistical analysis was performed by student's t-test, and the difference were considered at 0.05 significance level. A total of 33 cases of intra-oral minor salivary gland tumours were studied. Out of these, 13 cases were benign and 20 were malignant. The male to female ratio for both benign and malignant tumours was 1:1.7. The age range for those with benign tumours was 16-78 years while for malignant ones was 17-76 years. Females were more affected than males in both benign and malignant neoplasms. There was a statistically significant difference in the mean age of occurrence of intra-oral minor salivary gland tumours in female compared to males [P=0.003]. With regard to the location of minor salivary gland tumours, the palate was the most affected site [51.5%]. Pleomorphic adenoma was the most frequent tumour located on the palate [52.9%] Similarly, the majority of malignant tumours were located on the palate [38%] followed by the Cheek [23.8%] and included adenocarcinoma, adenoidcystic carcinoma and mucoepidermoid carcinoma. Most malignant tumours were treated by adjuvant radiation therapy. Pleomorphic adenoma located on the palate was treated by excision with 1cm clinical margins at its periphery and including the overlying epithelium and periosteum. However, pleomorphic adenoma located on other mucosa sites was treated by peripheral excision with 1cm margin. With regard to monomorphic adenoma, conservative surgical excision including a rim or margin of normal uninvolved tissue was done. Palate was the common site for intraoral minor salivary gland tumours and that the percentage of the palatal cases were higher in benign than in malignant tumours. The mean age of occurrence of minor intraoral salivary gland tumours was higher in female patients than that of male patients