Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0029463 (osteosarcoma)
 16,637 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Of approximately 1,999 cases of osteogenic sarcomas at the Mayo Clinic, 25 were diagnosed as telangiectatic osteogenic sarcomas. Of the 25 patients involved, 16 were males and 9 were females, and their ages ranged from 6 to 49 years. Six patients had had pathologic fracture. The lesions were typically located centrally and usually in the distal femur or proximal humerus and roentgenographically were large and purely lytic with destruction of cortex. Grossly, the lesions were cystic and contained clotted blood. Histologically, cystic spaces that contained blood were lined with anaplastic spindle cells and benign giant cells; sometimes, there were so few malignant cells that diagnosis was difficult. Usually, fine, lacelike osteoid was present. Of the 25 patients, 23 have died of metastatic disease, and another has developed pulmonary metastasis 11 months after amputation. Only one patient has survived for more than five years; however, he has developed pneumothorax. Data from this series suggest that the outlook in telangiectatic osteogenic sarcoma is more bleak than in conventional osteosarcoma.
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PMID:Telangiectatic osteogenic sarcoma. 106 3

Osteosarcoma is the most frequent primary malignant bone tumor. It usually occurs in the second decade of life. Pathologic fracture as the primary manifestation of an osteosarcoma is rather unusual, especially under the age of 10. The authors present the case of a 9-year-old boy with a diaphyso-metaphyseal femoral fracture, in whom the radiological features of an osteosarcoma were not recognized at the time of the fracture as they were not clearly visible, and at follow-up initially were considered as proliferative callus formation.
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PMID:Osteosarcoma appearing as a pathologic fracture. 177 23

Twelve dogs with appendicular osteosarcoma were treated with 24-40 Gy of cobalt 60 radiation and two doses of intraarterial cisplatin. Improvement in limb function occurred in four dogs, and three dogs, which had only mild initial lameness, had no worsening of their lameness post-treatment. In nine dogs in which local control was evaluable, eight had local failure, with the median (95% CI) duration of local control being 5.9 (4.6, 6.7) months. Two dogs had metastatic disease before therapy, and an additional nine dogs had metastatic disease at a median time of 6.4 months. Pathologic fracture was present in four dogs; two fractures occurred before treatment and two were documented at the time of tumor recurrence. Median (95% CI) survival time for all 12 dogs was 4.9 (3.4, 6.8) months. Excluding the two dogs with preexisting metastatic disease, median survival time was 6.7 months. Three dogs survived longer than 1 year. This mode of therapy was well tolerated and may be considered an alternative to amputation or limb-sparing surgical procedures in selected dogs with appendicular osteosarcoma.
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PMID:Treatment of canine appendicular osteosarcoma using cobalt 60 radiation and intraarterial cisplatin. 177 23

Radiation and pagetic osteogenic sarcomas should be distinguished from classical osteogenic sarcoma. Both occur in older patients with significantly greater comorbidity. Roentgenographically, radiation osteogenic sarcoma is typically sclerotic, whereas pagetic osteogenic sarcoma is lytic and associated with pathologic fracture. Radical resections give the best result, local control, and survival. Chemotherapy has not proven effective to date. Improvements in tumor imaging and more intensive chemotherapy regimens may permit limb-sparing surgery. Overall results remain poor, with approximately 15% five-year survival in each condition.
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PMID:Radiation and pagetic osteogenic sarcomas. 188 31

The authors assessed the impact of two cycles of preoperative chemotherapy (POCT) with intraarterial cisplatin (120 mg/m2) and continuous intravenous doxorubicin hydrochloride (Adriamycin; 20 mg/m2/day x 3 days) on the decision to perform a limb-sparing procedure (LSP) or amputation in 22 patients with high-grade bone sarcomas of the extremities. The tumor types were osteosarcoma (17), malignant fibrous histiocytoma (three), leiomyosarcoma (one), and malignant schwannoma (one). Surgical stages were IIA (three), IIB (17), and IIIB (two). The prechemotherapy surgical options chosen were 12 amputations (55% of patients) and ten LSPs (45%). The initial decisions to amputate were based on a combination of the following: improper biopsy (five cases), large tumors (ten) and those with neurovascular encroachment (six), and pathological fracture (one). Following chemotherapy, 18 LSPs (81%) and four amputations (19%) were performed. Nine of 12 patients (75%) initially deemed unresectable were converted to LSP. The median tumor response (necrosis; range, 0%-100%) was 70%; ten of 22 specimens had necrosis greater than 95%. Median tumor necrosis for the patients treated by amputation and LSPs was 45% and 88%, respectively. Following surgery, all patients received four additional cycles of cisplatin and doxorubicin. The median follow-up period is 30 months; six patients have developed metastatic disease, with a median disease-free interval of 16.6 months. The rate of local tumor control is 95% (21 of 22 patients).(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Impact of two cycles of preoperative chemotherapy with intraarterial cisplatin and intravenous doxorubicin on the choice of surgical procedure for high-grade bone sarcomas of the extremities. 188 42

The clinical, radiologic, and histologic features of 22 cases of Paget sarcoma were reviewed to determine in which patients with Paget disease these tumors are most likely to develop and what radiologic findings suggest the diagnosis. Clinical findings at presentation included pain and/or a mass (11 patients), pathologic fracture (seven), and neurologic symptoms (four). Survival time in 20 patients ranged from 5 days to 2.5 years. Two patients were lost to follow-up: one at 2 years and one at 8 years. There were 16 high-grade osteosarcomas, three chondrosarcomas, two fibrosarcomas, and one malignant fibrous histiocytoma. The most common site was the femur. Tumors also were observed in unusual sites. In one case of multifocal osteosarcoma, the tumor involved only pagetic bone. In 15 patients, Paget disease was polyostotic, clinically significant, and had been documented previously. In four patients, a sarcoma developed near the site of a fracture that had occurred between 2 months and 15 years previously. All cases showed radiologic evidence of a destructive lesion; other findings included a mass and evidence of tumor mineralization. Periosteal reaction was not observed. All but one tumor developed in a site of osteoblastic or mixed osteoblastic and lytic Paget disease. Our results suggest that sarcomas can develop in any part of any bone affected by Paget disease but are more likely to occur with advanced disease and to present with a destructive lesion without periosteal reaction.
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PMID:Sarcoma in Paget disease of bone: clinical, radiologic, and pathologic features in 22 cases. 202 67

We studied the value of MR imaging in monitoring the response of Ewing sarcoma and osteosarcoma to chemotherapy. Relative signal-intensity changes on MR images in the course of chemotherapy were compared with changes in tumor volume and histopathologic findings. MR scans (T1- and T2-weighted spin-echo images) were obtained in 20 patients with bone sarcoma. The first MR scan was obtained before the administration of chemotherapy in all patients. The follow-up scan was obtained in the course of treatment, before surgery. Tumor-volume and signal-intensity measurements of the intra- and extraosseous components of the tumor were analyzed. In 17 patients, histopathologic findings of the resected tumor were available for comparison with the MR images. In 12 of 17 patients there was complete agreement between changes in tumor volume, changes in the signal intensity of the extraosseous tumor component on T2-weighted images, and histopathology. In another four cases, changes in signal intensity correlated either with histopathology or with changes in tumor volume. In one patient with a pathologic fracture, no such correlation existed. A significant correlation was found between changes in signal intensities and pathologic response (r = .57, p = .02), as well as between changes in tumor volume and pathologic response (r = .53, p = .03). No correlation could be found between changes in signal intensity of the intraosseous tumor component and changes in tumor volume or histopathology. We conclude that the signal intensity of the extraosseous component of bone sarcomas on T2-weighted MR images in addition to changes in tumor volume may be useful in evaluating response to chemotherapy.
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PMID:The value of MR imaging in monitoring the effect of chemotherapy on bone sarcomas. 210 73

A 19-year-old man with telangiectatic osteosarcoma of the left proximal femur was started on a course of neoadjuvant chemotherapy consisting of intraarterial administration of cis-platinum. Within 72 hours of receiving the first intraarterial dose, the patient developed signs and symptoms of fat embolism syndrome (FES). A physical examination revealed cyanosis, tachycardia, and seizure activity. Laboratory studies demonstrated a pO2 of less than 65 mmHg, lipuria, and a drop in hematocrit of three percentage points. There was no clinical or roentgenographic evidence of pathologic fracture. Tumor necrosis secondary to intraarterial cis-platinum therapy in this patient with osteosarcoma may have caused a sudden release of free fatty acids and embolization of fat macroglobules that precipitated this episode of FES. FES in association with the intraarterial administration of cis-platinum seems not to have been previously reported.
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PMID:Fat embolism syndrome complicating intraarterial chemotherapy with cis-platinum. 232 45

Telangiectatic osteosarcoma is a rare and special variant of osteogenic sarcoma with distinct radiologic, gross and microscopic features. This tumor is predominantly lytic, destructive tumor without sclerosis on roentgenogram, and is soft and cystic on gross examination. Histologically aneurysmally dilated spaces lined or traversed by stromal cells producing osteoid are noted. This report concerns a case of telangiectatic osteosarcoma occurring in a 7 years old boy. He presented with pathologic fracture of the right distal tibia, followed by a purely lytic lesion on X-ray examination. This lesion recurred five times during a span of one year. Microscopic features of the biopsy specimen was difficult to differentiate from aneurysmal bone cyst because of prominant blood-filled cyst formation. It was finally identified as osteosarcoma from the below-knee amputation specimen through the close examination for anaplastic osteoid-producing stromal cells in the septa that separate the blood cysts.
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PMID:Telangiectatic osteosarcoma--a case report. 259 66

Thirty-three patients with high grade bone sarcomas of the knee and shoulder treated by limb sparing surgery were evaluated. The histological diagnoses were osteosarcoma (25), chondrosarcoma (3), malignant fibro-histiocytoma (3), fibrosarcoma (1) and unclassified (1). The Surgical Stages were: Stage IIA (3), Stage IIB (28) and Stage III (3). The operative procedure consisted of three phases: tumor resection, skeletal reconstruction and soft tissue reconstruction. All resections obtained negative margins and were classified as, marginal excision (3), intracompartmental resections (28) and radical resections (2). Overall survival was 77%. Four of 33 patients (12.4%) required a secondary amputation. Local recurrence was 6% (2/33) with an average follow-up of 37.2 months. The most common complications were flap necrosis (33%) and transient nerve palsies (33%). There were 2 infections and one prosthetic dislocation. We believe that limb salvage surgery for high grade bone sarcomas need not be reserved for only those without extraosseous extension. Careful preoperative selection and attention to the three stages of a limb sparing procedure are important for a successful outcome. Presently, we consider the following as contraindications to resection: vascular involvement, pathologic fracture, inappropriate biopsy and infection.
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PMID:Surgical technique and results of limb sparing surgery for high grade bone sarcomas of the knee and shoulder. 300 4


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