1/9. Chondromyxoid fibroma of skull base: a tumour prone to local recurrence.Chondromyxoid fibroma of the skull base is extremely uncommon. Sometimes involvement of the nasal cavity may occur and the patients may present with nasal symptoms. The biological behaviour of this tumour has not been well studied, primarily because of the limited number of reported cases and the short duration of follow-up. We report a histologically confirmed case of chondromyxoid fibroma of the skull base that recurred repeatedly over a 10-year period after the initial operation. Histologically it showed identical morphology to the original tumour with no evidence of histological progression or dedifferentiation. Ultrastructurally, the spindle tumour cells in the fibromyxoid area showed dual chondroblastic and fibroblastic differentiation, suggesting that these spindle fibroblastic cells and the better differentiated chondroid cells were of the same cell type with different histological morphology.- - - - - - - - - - ranking = 1keywords = behaviour (Clic here for more details about this article) |
2/9. Primary cranial Ewing's sarcoma.Primary Ewing's sarcoma of the cranium is extremely rare, with only 17 cases reported so far. We describe a further case with involvement of the frontotemporal region and the orbit. The patient, an adult male, was admitted with headache and papilloedema, and later developed localised swelling and proptosis. He was treated with surgery, radiotherapy and chemotherapy, and was free from metastases during a follow-up of 14 months. The prognosis of Ewing's sarcoma is improving with radiotherapy and chemotherapy. Further cases are needed to study the biological behaviour of primary cranial Ewing's sarcoma.- - - - - - - - - - ranking = 1keywords = behaviour (Clic here for more details about this article) |
3/9. Multifocal cerebral hemangio-endothelioma. Case report and review of the literature.BACKGROUND: Intracranial hemangio-endotheliomas (HE) are rare vascular tumours that present histological features and biological behaviour of an intermediate malignancy. We report the first case of primary HE presenting as multiple intracranial masses. CASE DESCRIPTION: The patient is a 20-year-old female who presented with paresis of the left arm. magnetic resonance imaging (MRI) showed multiple lesions in both frontal lobes. Total surgical excision of the tumours was obtained; a histological diagnosis of hemangio-endothelioma was made. Five months later, MRI scan showed new intradiploic skull lesions whereas a scintigraphic study revealed multicentric extracranial bone involvement. Treatment based on interferon-alpha (IFN-alpha) administration was undertaken; this produced partial regression of the lesions. Approximately three years after the first operation, the patient is in good clinical condition. CONCLUSIONS: Surgery continues to play the leading role in the treatment of intracranial HE. However, adjuvant therapy with IFN represents a new and well-tolerated therapeutic method for residual intracranial lesions as well as multicentric extracranial disease.- - - - - - - - - - ranking = 1keywords = behaviour (Clic here for more details about this article) |
4/9. Ossifying fibroma of the temporal bone.A rare case of ossifying fibroma of the temporal bone is presented. Fibro-osseous lesions are benign neoplasms but may show an aggressive behaviour when invading important anatomical structures. The lack of experience in the treatment of those tumours is reflected in the small relative literature. The purpose of this paper is to contribute to the few cases already reported.- - - - - - - - - - ranking = 1keywords = behaviour (Clic here for more details about this article) |
5/9. Rare sellar region tumors.We present three cases of rare intracranial midline tumor in the sellar region, often mimicking pituitary adenomas clinically. We describe their symptoms, radiological and pathomorphological features. The first case is a pituitary adenoma producing growth hormone with ganglion cell differentiation. In addition, a rare intracranial granular cell tumor of sellar region and germinoma of pituitary fossa are also presented. All tumors were resected and histologically analyzed. Their biological behaviour was favorable with a 10-year follow-up demonstrating no recurrent tumor mass.- - - - - - - - - - ranking = 1keywords = behaviour (Clic here for more details about this article) |
6/9. Extensive primary Ewings' sarcoma in the greater wing of the sphenoid bone.We describe a rare case of an extensive primary cranial Ewing's sarcoma located in the greater wing of the sphenoid bone with extension to the orbit, the endocranium, the parapharyngeal and infratemporal space. The patient presented with diplopia, anosmia and prolapse of the left eye. He was given chemo- and radiotherapy and was free of symptoms on re-examination 1.5 years later. The prognosis of Ewing's sarcoma in the absence of surgery is uncertain, but prompt treatment appears to have a satisfactory therapeutic outcome. In the future, more cases should be studied in order to investigate the biological behaviour of a primary cranial Ewing's sarcoma.- - - - - - - - - - ranking = 1keywords = behaviour (Clic here for more details about this article) |
7/9. Hereditary intraosseous vascular malformation of the craniofacial region: imaging findings.Benign vascular lesions can be classified into two categories depending on clinical behaviour and endothelial cell characteristics: neoplasms (haemangiomas) and vascular malformations. However, intraosseous vascular anomaly, previously called intraosseous haemangioma, is a very rare malformation. In our previous study, we described the first hereditary form of intraosseous vascular malformation of the craniofacial region, vascular malformation osseous (VMOS). Characteristic findings are autosomal recessive inheritance, severe and diffuse intraosseous vascular malformation in all craniofacial bones without soft tissue involvement and associated mid-line abnormalities such as umbilical hernia and supra-umbilical raphe. In this paper, we discuss the imaging findings of this new disorder in detail.- - - - - - - - - - ranking = 1keywords = behaviour (Clic here for more details about this article) |
8/9. Malignant melanotic schwannoma of the bronchus.A case of malignant melanotic schwannoma arising in the right upper lobe bronchus of a 27 year old man is presented. Tumours of this type most commonly occur in spinal nerve roots and are generally considered to be benign. The behaviour of those originating elsewhere is less predictable. As far as we are aware this is the first reported case affecting the respiratory tract.- - - - - - - - - - ranking = 1keywords = behaviour (Clic here for more details about this article) |
9/9. Juvenile fibrosarcoma of the temporal bone.A case of juvenile fibrosarcoma arising from the head and neck region is described. This type of tumour should be considered as a separate entity different from the fibrosarcoma in adults because of the different clinical behaviour. The symptomatology, the radiographic features and the literature data are reviewed.- - - - - - - - - - ranking = 1keywords = behaviour (Clic here for more details about this article) |