1/25. Bone giant cell tumour in neuropathological practice. A fifty year overview.We report a case of a 29-year-old female patient who suffered from visual disturbance, resulting from a lesion in the sphenoid bone which, histologically, proved to be a giant cell tumour. Reviewing our laboratory practice over a 50 year period, only 7 cases of true giant cell tumour were found and they were in two major locations, i.e. the skull and vertebrae. These few cases led us to focus on the problem raised by the lack of histological patterns of malignancy. In agreement with Mazabraud's theory, it seems that a vertebral location is associated with a good prognosis and that gene mapping of chromosome 17 in relation to p53 mutations could be a valuable tool in the diagnosis of potential malignant behaviour.- - - - - - - - - - ranking = 1keywords = behaviour (Clic here for more details about this article) |
2/25. Childhood periosteal chondroma.We report here three fully documented cases of periosteal chondroma diagnosed and treated in our hospital. There have been few references since Lichtenstein first described this condition as a separate tumour, and none of them concerned children under 10 years old. deSantos accurately describes the radiological features of the tumour. All of our patients were under 10 years old at the moment of the appearance of the lesion, which was always detected in the long bones of the upper limbs. The study included a previous histological examination in two cases and a follow-up in all of them. From this study we learned that invasive diagnosis procedures are unnecessary as we could follow the behaviour and evolution of this cartilaginous benign tumour for a long period of time.- - - - - - - - - - ranking = 1keywords = behaviour (Clic here for more details about this article) |
3/25. Clinics in diagnostic imaging. Sacrococccygeal chordoma.A 41-year-old man with a sacrococcygeal chordoma is presented with emphasis on its morphological and imaging features. Examples of chordomas at other sites situated along the skull base and spinal axis are illustrated. Bone erosion and expanding soft tissue mass are invariably present on both enhanced computed tomography and magnetic resonance imaging. Sacrococcygeal chordomas are usually large on initial presentation and treatment is targeted at complete surgical excision since incomplete resection invariably leads to recurrence and distant metastases. skull base chordomas are smaller but are less accessible to complete removal. Adjuvant radiotherapy is offered under these circumstances with the view to delay recurrence. The biological behaviour and prognostic factors for survival are summarised.- - - - - - - - - - ranking = 1keywords = behaviour (Clic here for more details about this article) |
4/25. Cutaneous metastases from Ewing's sarcoma: report of two cases.Ewing's sarcoma is a malignant osseous neoplasm that affects mostly children and young adult males. Clinically, the neoplasm presents with oedema, swelling, and pain of the involved area. Histopathologically, Ewing's sarcoma consists of solid sheets of small round cells, with vesicular nuclei and scant cytoplasm, arranged in irregular masses separated by strands of fibrous tissue, with areas of necrosis en masse intermingled with intratumoural haemorrhage. Ewing's sarcoma is an extremely aggressive neoplasm and metastases to sites such as lung, pleura, other bones, central nervous system, liver, and regional lymph nodes frequently develop in early stages of the disease. Surprisingly, despite the highly aggressive biological behaviour of this neoplasm, cutaneous metastases from Ewing's sarcoma are very uncommon. We report two patients with Ewing's sarcoma of the bone who developed cutaneous metastases. As in other internal malignancies, the onset of cutaneous metastases in patients with Ewing's sarcoma indicates a poor prognosis.- - - - - - - - - - ranking = 1keywords = behaviour (Clic here for more details about this article) |
5/25. Metastasising clear cell odontogenic carcinoma: a case report and review of the literature.Primary odontogenic carcinomas are rare and examples which have metastasised are even more uncommon. We describe the first reported case of a clear cell odontogenic carcinoma which metastasised to distant bones, namely the 5th lumbar vertebra and hip, 3 years after initial diagnosis. The initial incisional biopsy was thought to represent a calcifying epithelial odontogenic tumour, but in the subsequent resection the tumour showed a prominent clear cell component admixed with squamous cells showing peripheral palisading, widespread infiltration and necrosis indicating a malignant neoplasm. Radiologically guided biopsy revealed a metastatic lesion in L5 vertebrae and left hip, confirmed by immunohistochemistry. The metastatic lesion had similar appearances to the first biopsy, and diagnosis was confirmed by comparison of histological features, immunohistochemistry and exclusion of a second primary lesion by clinical examination and imaging. The diagnosis of clear cell odontogenic carcinoma is a difficult one to make. The behaviour of these tumours is unpredictable. This case confirms that clear cell odontogenic carcinomas have the potential for distant metastasis and require long-term follow up.- - - - - - - - - - ranking = 1keywords = behaviour (Clic here for more details about this article) |
6/25. Aggressive psammomatoid ossifying fibroma of the inferior turbinate and lateral nasal wall.OBJECTIVE: Fibroosseous lesions are rare entities of the nose and paranasal sinuses, the terminology and classification of which are still confusing. Psammomatoid or aggressive (juvenile) ossifying fibroma is a benign messenchymal tumor usually met in the young age (5-15 years), in the sinonasal tract, bearing distinctive histomorphologic features and a tendency towards locally aggressive behaviour. METHODOLOGY: We report here a rare case of an aggressive psammomatoid ossifying fibroma of the inferior turbinate and the lateral nasal wall, with obstruction of the nasolacrimal duct, in a 68-year-old woman. RESULTS: diagnosis was based on physical examination, CT scan imaging and histopathological examination. Treatment consisted of endoscopic intranasal resection of the tumor accompanied by removal of the lateral nasal wall. CONCLUSIONS: Fibro-osseous tumors of the nose and paranasal sinuses require aggressive surgical approach in order to avoid recurrence. Complete surgical excision may not always be possible mainly due to the tumor's extent and location.- - - - - - - - - - ranking = 1keywords = behaviour (Clic here for more details about this article) |
7/25. chondroblastoma of the lumbar spine with cauda equina syndrome.STUDY DESIGN: Case report. OBJECTIVE: To describe the clinical presentations, radiological features and clinical progress of a rare case of chondroblastoma of the lumbar spine. SETTING: Regional Hospital, hong kong, china. METHOD: A 54-year-old male patient presented with low back pain and left sciatica. X-ray and MRI revealed tumour infiltration of the fifth lumbar vertebrae and left paraspinal muscles, which was found to be a chondroblastoma by repeated open biopsies. The tumour was removed surgically by combined anterior and posterior approaches, followed by spinal fusion and instrumentation. RESULTS: The anterior tricortical bone graft was complicated with fracture and nonunion. Surgical re-exploration confirmed local recurrence of tumour macroscopically and histologically. The patient developed symptoms and signs of cauda equina syndrome gradually despite repeated surgery and irradiation. The patient eventually died of complications of local recurrence and neurological deficit at 3 years and 8 months after the first operation. CONCLUSION: This is the first case report of chondroblastoma of the lumbar spine. The clinical profile of this patient and the evidence from the literature review suggests that spinal chondroblastoma has a very aggressive behaviour with high recurrence and mortality rate.- - - - - - - - - - ranking = 1keywords = behaviour (Clic here for more details about this article) |
8/25. Pitfalls in the diagnosis and management of laryngeal chondrosarcoma.Chondrosarcomas of the larynx (CS) are uncommon and predominantly affect the cricoid cartilage. In the larynx they have a distinctive biological behaviour and require individual treatment. A retrospective study was made on three cases of CS, all presenting with compromise of the upper respiratory tract. The medical history varied from several weeks to six years. Correct diagnosis required open resection of the lesion in all cases. Due to intra-operative findings, all patients underwent total laryngectomy. CS grow slowly and are therefore frequently diagnosed late in the course of the disease. A subglottic bulging of the mucosa should indicate high-resolution-computed-tomography of the larynx to exclude affection of the laryngeal framework. Correct diagnosis of laryngeal cartilaginous tumours requires a complete examination of the entirely resected tumour. Only extended and de-differentiated CS indicate further radical surgery, the remainder call for conservative surgical management.- - - - - - - - - - ranking = 1keywords = behaviour (Clic here for more details about this article) |
9/25. Bizarre parosteal osteochondromatous proliferation of the hand in a young man.We report a case of a solitary osteochondromatous tumor on the hand of a 38 yr old man. This had radiological and histological features distinct from an osteochondroma and demonstrated the features first described as bizarre parosteal osteochondromatous proliferation. These lesions have a tendency for local recurrence but no metastatic behaviour has yet been reported. We highlight 2 histological features which have not been previously described.- - - - - - - - - - ranking = 1keywords = behaviour (Clic here for more details about this article) |
10/25. natural history of giant cell tumour of the bone.The clinical presentation and behaviour of giant cell tumours of bone vary. The progression of the disease and metastases are unpredictable, but the overall prognosis is good. We describe the natural history and different clinical presentations of two cases of giant cell tumour of bone where the patients had refused the initial treatment and presented several years later with the disease.- - - - - - - - - - ranking = 1keywords = behaviour (Clic here for more details about this article) |
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