1/8. Malignant peripheral nerve sheath tumour in the maxilla associated with von Recklinghausen's disease.Malignant transformation of neurofibromatosis is one of the most serious complications of von Recklinghausen's disease (VRD). The most common associated malignancy is the malignant peripheral nerve sheath tumour (MPNST). Few cases of MPNST associated with VRD in the maxillary region have been reported. This report describes a rare case of MPNST in the maxilla and the aggressive nature of MPNST associated with VRD.- - - - - - - - - - ranking = 1keywords = fibromatosis (Clic here for more details about this article) |
2/8. Aggressive infantile (desmoid-type) fibromatosis of the maxilla: a case report and new classification.This paper describes the clinical, radiographic and histologic findings of an aggressive infantile (desmoid-type) fibromatosis of the face in a seven-year-old black Jamaican male. This condition is rare in the head and neck region and its occurrence in the maxilla is exceptional. The differential diagnosis, management and long term follow-up of this case are also mentioned The need for a less aggressive surgical management in this child and long-term follow-up is stressed. Also, its occurrence in someone of African descent has not been reported previously. The absence of recurrence, eight years after surgery is significant. This paper discusses the differential diagnosis and treatment of aggressive infantile fibromatosis and suggests a classification of the condition.- - - - - - - - - - ranking = 6keywords = fibromatosis (Clic here for more details about this article) |
3/8. Oral neurofibrosarcoma associated with neurofibromatosis type I.One of the most feared complications of neurofibromatosis type I (NF-I) is development of cancer, which is estimated to occur in about 5% of cases. The most common associated malignancy is the neurofibrosarcoma (NFS). HOwever, oral NFS in association with NF-I has rarely been reported. We report two cases of oral NFS arising in patients with NF-I. Both patients died of their tumors. Oral NFS arising in association with NF-I appears to have an extremely poor prognosis, as do these tumors at other sites of the body.- - - - - - - - - - ranking = 5keywords = fibromatosis (Clic here for more details about this article) |
4/8. Aggressive fibromatosis of the maxilla. Report of a case and review of the literature.Aggressive fibromatosis is a rare tumor of the jaws. Its occurrence in the maxilla is even more exceptional. The case of a 14-year-old girl with a peripheral fibromatosis of the palatal process of the maxilla is described. The lesion manifested as a painless swelling. On initial excision, a diagnosis of fibroma was made. After recurrence and reevaluation of the microscopic slides, a final diagnosis of aggressive fibromatosis was established. The features of this case and 12 similar lesions of the maxilla previously described in the literature are analyzed and discussed.- - - - - - - - - - ranking = 7keywords = fibromatosis (Clic here for more details about this article) |
5/8. Nonsurgical treatment of aggressive fibromatosis in the head and neck.Aggressive fibromatosis is a poorly defined, locally aggressive, yet histologically benign fibroblastic proliferative lesion that may occur in the head and neck. The lesion is highly cellular and locally infiltrative and has a propensity to invade and erode bone, compromising vital structures within the head and neck. However, it is not a true malignancy because it does not have malignant cytologic characteristics nor does it metastasize. We present two cases of aggressive fibromatosis occurring in young adult men. The first case involved a rapidly enlarging mass of the anterior maxilla that involved the upper lip, nasal alae, nasal septum, inferior turbinates, and hard palate. The patient underwent incisional biopsy to confirm the diagnosis. Because of difficulty in determining the actual margins of this extensive lesion and the significant morbidity that would have resulted from surgical resection, we elected to treat this patient with chemotherapy and radiation therapy. The second case was an extensive lesion involving the right temporal bone, pterygomaxillary space, and infratemporal, temporal, and middle cranial fossae. Incisional biopsy confirmed the diagnosis. Because of the lack of functional and cosmetic deficits and the unavoidable morbidity of a surgical resection, this patient was treated with radiation therapy. Although wide field resection is the most satisfactory form of treatment, in situations in which this modality would result in unacceptable morbidity or if surgical margins are positive, then radiation therapy and chemotherapy should be considered. Support for these therapeutic modalities is found in larger series of cases outside the head and neck.- - - - - - - - - - ranking = 6keywords = fibromatosis (Clic here for more details about this article) |
6/8. A rare cause of maxillary tuberosity enlargement: Von Recklinghausen's neurofibromatosis.Oral involvement is unusual in cases of Von Recklinghausen's neurofibromatosis. A case is described of a patient who presented for treatment of an enlarged maxillary tuberosity and the management of such cases is discussed.- - - - - - - - - - ranking = 5keywords = fibromatosis (Clic here for more details about this article) |
7/8. Solitary neurofibromatosis in the maxilla: report of oral findings.Clinical and histological manifestations of von Recklinghausen disease are reviewed. The oral findings in a patient with a solitary maxillary plexiform neurofibroma are described.- - - - - - - - - - ranking = 4keywords = fibromatosis (Clic here for more details about this article) |
8/8. Pediatric sino-orbital desmoid fibromatosis.Desmoid fibromatosis is a rare tumor of the head and neck. Only five cases involving the sino-orbital region have been previously reported in the literature. In this report we describe the sixth such case, involving a 2-year-old boy, and review the literature.- - - - - - - - - - ranking = 5keywords = fibromatosis (Clic here for more details about this article) |