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Query: UMLS:C0029463 (osteosarcoma)
 16,637 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A young patient surviving four years after treatment for primary intracerebral small-cell osteosarcoma is reported. This 16-year-old girl presented with headaches, visual disturbance, and papilledema and was found to have a large, heavily calcified mass in the left parietal lobe. A gross total resection of the tumor was performed and pathologic analysis revealed a small-cell osteosarcoma. Thoracic computerized tomography (CT) scans and a complete bone scan showed no other lesions. The patient received adjuvant chemotherapy and radiotherapy. She continues to do well with no evidence of metastases or local recurrence 4 years after her initial presentation. The clinical, radiographic, surgical, pathologic, and immunohistochemical features of this case, along with therapeutic interventions and outcome, are presented.
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PMID:Primary intracerebral small-cell osteosarcoma in an adolescent girl: report of a case. 912 May 47

Two cases of calvarium sarcoma with intracranial extension, consecutive to Paget disease of the skull are presented. The neurosurgical implications of this rare complication of a common disease are reviewed. In both cases, the symptoms of malignant degeneration were unspecific and blurred by the presence of a well-known Paget disease. Increasing headaches, focal neurological deficits and signs of intracranial hypertension were observed. Skull X-rays and Computerised Tomography did not allow to make the difference between Pagetic bone and sarcoma. In the second case, Magnetic Resonance Imaging was the most accurate in determining the precise localisation of the tumour, and the subdural invasion. Extensive surgical resection was carried out, by craniectomy of the tumoural bone, followed by cranioplasty. Dural sinus involvement and tumour hypervascularisation caused important peroperative blood loss in both patients. In the second case, the tumour invaded the subdural space through a Pacchioni granulation. Survival of patients with Pagetic sarcoma seems shorter when compared to post-radiation or primitive osteosarcoma, despite adjuvant therapies, probably because of late diagnosis and incomplete surgical resection. The mean reported survival rate is 6 months. Although the first patient died within 4 months, in the second case, the authors obtained a more than two year survival with aggressive surgery alone. These cases also illustrate the polymorphism of sarcomatous degeneration of Pagetic calvarium, the interest of MRI, and the need for close surveillance of patients with known Pagets disease of the skull.
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PMID:Pagetic sarcoma of the calvarium: report of two cases. 992 24

A 28-year-old male presented with a primary osteosarcoma of the sphenoid bone with extensive periosteal extension manifesting as severe headache and right exophthalmos. Computed tomography (CT) and magnetic resonance imaging revealed an anterior middle cranial fossa tumor extending into both the orbit and the extracranial space. However, roentgenography and CT with bone windows showed no marked osteolytic changes of the sphenoid bone. Total removal of the tumor was performed via the orbitozygomatic approach. Surgery revealed that the tumor had extended periosteally without macroscopic bone destruction, but no obvious abnormalities of the skull. The histological diagnosis was osteosarcoma. The patient was treated with chemotherapy and radiation therapy, but died of tumor recurrence 10 months after the surgery.
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PMID:Primary osteosarcoma of the sphenoid bone with extensive periosteal extension--case report. 1097 65

A 56-year-old woman presented with an intracranial osteosarcoma at the site of previous radiosurgery, manifesting as sudden onset of headache and left hemiparesis with aphasia. She had a previous history of stereotactic radiosurgery for an intracranial tumor under a diagnosis of falx meningioma. Computed tomography showed intratumoral and peritumoral hemorrhage at the right parietofrontal region. Gross total resection of the tumor with hematoma was performed. The histological diagnosis was osteosarcoma. Sarcomatous change is a rare complication of radiotherapy. This case illustrates that osteosarcoma may develop years after radiosurgery for benign brain neoplasm.
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PMID:Intracranial osteosarcoma after radiosurgery--case report. 1495 34

The ideal treatment of osteoporosis should preferably prevent fractures through normalization of bone mass and bone micro-architecture. Biosynthetic human parathyroid hormone 1-34 (teriparatide) was recently approved in the EU and the USA as the first anabolic treatment of osteoporosis. The effects of teriparatide are mediated by the G-protein-dependent, parathyroid hormone receptor-1 in the cell membrane. The binding of the ligand to the receptor activates adenylate cyclase and a number of phospholipases (A, C, and D) and increases intracellular levels of cAMP and calcium. Intermittent teriparatide increases the number of osteoblasts and bone formation by activation of pre-existing osteoblasts, increased differentiation of lining cells, and reduced osteoblast apoptosis. Anabolic effects of teriparatide on bone have been demonstrated in several species. It increases bone mass, structural integrity, bone diameter, and bone strength. Clinical efficacy was demonstrated in a randomized study comprising 1637 post-menopausal women with osteoporosis showing a 65% and 35% reduction of the relative risk of vertebral and appendicular fractures, respectively, during 18 months of treatment. Moreover, bone mineral density in the lumbar spine and hip increased by 9.7% and 2.6%, respectively. Similar effects on bone mineral density have been reported in men with osteoporosis and in glucocorticoid-induced osteoporosis, however, fracture data are limited in these groups. Direct comparison with alendronate revealed that teriparatide has a more pronounced effect on bone mineral density. Teriparatide should be used in combination with calcium plus vitamin D, and may be combined with hormonal replacement therapy. In contrast, alendronate attenuates the effect of teriparatide. The efficacy of other combinations remains uncertain. After termination of teriparatide, bone mineral density of the lumbar spine is reduced by approximately 2-3% after 2 1/2 years. This decrease is prevented by treatment with bisphosphonates. The most frequent adverse effects with teriparatide are nausea, headache, dizziness, and leg cramps, however, only the latter two differed significantly between the groups receiving teriparatide 20 microg/day and placebo. In the pivotal clinical study, reduced dosage or termination of therapy due to hypercalcaemia was necessary in 3% and 0.2%, respectively. In a rat toxicology study, in which teriparatide was administered in high dosages for an extended period of time, osteosarcoma was seen in a significant number of animals. However, none of the approximately 2800 patients in clinical trials has developed osteosarcoma. Teriparatide constitutes a break-through in the treatment of severe osteoporosis, although a number of issues about the optimal use of teriparatide remains unsettled. The published data provide proof of concept on anabolic therapy which changes several paradigms of bone physiology. Other parathyroid hormone analogues are being investigated in clinical trials and the development of non-peptide, small molecules targeted at the parathyroid hormone receptor may be envisaged.
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PMID:Teriparatide (biosynthetic human parathyroid hormone 1-34): a new paradigm in the treatment of osteoporosis. 1522 97

Primary extraskeletal osteosarcoma occurring in the brain parenchyma is distinctly uncommon, with only five cases having been reported. The authors describe the case of a 45-year-old man who presented with progressive headache and diplopia. Computerized tomography scanning and magnetic resonance imaging results revealed a pineal region tumor with obstructive hydrocephalus. The patient underwent partial resection of the tumor. The histological examination showed large pleomorphic tumor cells embedded in osteoid matrix. Immunohistochemical analysis was negative for various antibodies and thus excluded a glial, germ cell, epithelial, and lymphoid tumor origin. Only vimentin showed strong positivity in most of the tumor cells. Ultrastructurally, the tumor cells were rich in dilated rough endoplasmic reticula. Clear zones between tumor cells and osteoid matrix were observed. The osteoid matrix was made up of small collagen fibrils and hydroxyapatite deposits. The tumor was not attached to the bone structure of the skull. These findings are consistent with the features of extraskeletal osteosarcoma. Data from complete medical and radiological studies excluded a metastatic origin for this tumor. Partial resection and postoperative radiotherapy had provided tumor control at 11 months after the onset of symptoms. This is the first reported case of a primary extraskeletal osteosarcoma occurring in the pineal region.
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PMID:Primary extraskeletal osteosarcoma in the pineal region. Case report. 1559 71

(1) Oral alendronic acid is the reference drug for women with osteoporosis and a previous vertebral fracture. In a placebo-controlled trial in women who were also taking calcium and vitamin D, treatment with alendronic acid for three years reduced the incidence of symptomatic vertebral fractures (2.3% versus 5%) and wrist fractures (2.2% versus 4.1%) and, albeit with a lower level of evidence, the incidence of hip fractures (1.1% versus 2.2%). (2) Teriparatide, a biotech drug, reproduces the 34 N-terminal amino acids of parathormone. It is marketed in Europe for subcutaneous treatment of "proven" postmenopausal osteoporosis. (3) The cornerstone of the clinical evaluation dossier is a randomised placebo-controlled double-blind trial in 1637 women also taking calcium and vitamin D. The two doses of teriparatide (20 micrograms/day and 40 micrograms/day), given for a median of 19 months, reduced the risk of new radiologically documented vertebral fractures (about 4% versus 14% in the placebo group) and spinal pain (about 16% versus 23% in the placebo group), but not the risk of hip fracture. (4) In a double-blind trial in 146 postmenopausal women also taking calcium and vitamin D, 40 micrograms/day teriparatide given subcutaneously for 14 months increased spinal mineral bone density significantly more than 10 mg/day alendronic acid given orally. The trial was not designed to show a difference in clinical outcome (fractures). (5) The main adverse effects of teriparatide reported to date are nausea, headache, cramp, hypercalcemia and hyperuricemia. (6) A rat study showed an increased risk of osteosarcoma. This tumour is rare in humans, and the number of patients so far enrolled in clinical trials is insufficient to document a possible increase in risk associated with teriparatide. (7) The need for daily subcutaneous injections and for refrigeration of the prefilled syringes are two notable disadvantages of teriparatide therapy. (8) In practice, alendronic acid is better assessed and remains the reference treatment, combined with calcium and vitamin D, for secondary prevention of osteoporotic fracture in postmenopausal women.
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PMID:Teriparatide: new preparation. Osteoporosis: less well evaluated than alendronic acid. 1574 48

Osteosarcoma, usually observed in long bones, is the second most frequent primitive malignant bone tumor after myeloma. The skull base is an exceptional localization. We report a case of skull base osteosarcoma managed in our department. A 23-year-old female was admitted for bilateral epistaxis, headache, decreasing visual acuity then blindness. Physical examination revealed bilateral blindness and exophthalmia. Cranial magnetic resonance imaging showed a voluminous mass in the skull base extending to the nasosinusal area. A rhinoseptal surgical approach was used but the tumor was so huge that excision was impossible. The biopsy identified at an osteoblastic osteosarcoma. Search for extension (chest computed tomography and abdominal ultrasonography) was negative. Chemotherapy was to be delivered before combination chemoradiotherapy but the patient died before any treatment could be started. Osteosarcoma of the skull base is very rare. The treatment is based on surgery which should be as complete as possible followed by chemoradiotherapy. Prognosis is poor. Median survival is around six months.
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PMID:[Osteosarcoma of the skull base: a case report]. 1806 Dec 13

We report a case of primary dural based osteosarcoma in the right fronto-temporal convexity in a 43-year-old female who presented with a short history of seizure and headache. Radiologic evaluation revealed a well defined brightly enhancing extra-axial lesion in the right fronto-temporal region with a dural tail around the sylvian fissure. The overlying bone was uninvolved. Paraffin section of the tumor showed plump cells with moderate nuclear and cellular pleomorphism with eosinophilic extracellular material (osteiod) between the cells. At a few places, lace like osteiod was seen encasing individual cells signifying osteiod being formed by tumor cells. Immunohistochemistry for EMA was focally positive and negative for S-100 protein and GFAP. A final histopathological diagnosis of dural based primary osteosarcoma of the right fronto-temporal region was rendered. To the best of our knowledge this will be the eighth such case in literature.
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PMID:Dural based primary osteosarcoma in right fronto-temporal region with review of literature. 2022 82

Mifamurtide (liposomal muramyl tripeptide phosphatidyl ethanolamine; Mepact) is an immunomodulator with antitumor effects that appear to be mediated via activation of monocytes and macrophages. In the EU, mifamurtide is indicated in children, adolescents, and young adults for the treatment of high-grade, resectable, non-metastatic osteosarcoma after macroscopically complete surgical resection; it is administered by intravenous infusion in conjunction with postoperative multiagent chemotherapy. In the US, mifamurtide is currently an investigational agent that holds orphan drug status for the treatment of osteosarcoma. In a large, randomized, open-label, multicenter, phase III trial, the addition of adjuvant (postoperative) mifamurtide to three- or four-drug combination chemotherapy (doxorubicin, cisplatin, and high-dose methotrexate with, or without, ifosfamide) was associated with a statistically significant improvement in overall survival in patients with newly diagnosed, high-grade, non-metastatic, resectable osteosarcoma. The pattern of outcome was generally similar in a small cohort of patients with metastatic disease who were enrolled in this trial. Mifamurtide is generally well tolerated; adverse events attributed to administration of the drug include chills, fever, headache, nausea, and myalgias. Based on the available data, mifamurtide can be considered for inclusion in treatment protocols for localized osteosarcoma.
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PMID:Mifamurtide: a review of its use in the treatment of osteosarcoma. 2048 44


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