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)

Bone morphogenetic proteins (BMPs), inducers of ectopic bone formation in vivo, are present in a number of osteosarcomas. BMPs are responsible for reactive bone formation, including periosteal reactions by normal osteoblasts, rather than production of tumorous osteoid by tumor cells. Osteosarcomas producing BMPs contain less-differentiated mesenchymal cells, resulting in a poorer prognosis for those patients. BMPs are also expressed in malignant fibrous histiocytomas (MFHs) of bone and dedifferentiated chondrosarcomas exhibiting undifferentiated features. However, BMPs in MFH do not show any osteoinductive activity in vivo, suggesting that those BMPs may be inactive forms and have additional functions unrelated to bone formation. Among benign bone tumors, BMPs are expressed in osteoid osteomas or osteoblastomas and effect reactive bone formation such as a surrounding sclerosis. BMPs and a BMP receptor (BMPRIB) are also detected in the cartilage cap in osteochondroma, suggesting that BMP signaling via BMPRIB might be involved in the pathogenesis of osteochondroma. Clinically, BMPs have utility as diagnostic and prognostic markers for characterizing the stage of differentiation of mesenchymal cells and mesenchymal tumors, and they may be of value in predicting the prognosis of sarcoma patients. This article reviews the accumulated information on BMPs in bone tumors, including the most recent findings, and discusses the biological and clinical significance of BMPs in bone tumors.
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PMID:Bone morphogenetic proteins in bone tumors. 1516 94

This study was conducted to determine the outcome of patients who develop a second neoplasm after radiotherapy (RT) for a childhood solid tumor. From 1956 to 1998, 429 children with a malignant solid tumor were treated at a single radiation oncology facility. The medical records and radiotherapy charts were reviewed to determine if the patient developed a secondary neoplasm after treatment for malignancy. Twenty-three (5.4%) patients developed a secondary neoplasm. There were 12 males and 11 females with a median age at RT of 6.6 years (range, 2 months to 20 years). There were 14 malignant neoplasms in 13 (3.0%) and 14 benign neoplasms in 11 patients (2.6%). The types of initial solid tumors treated with RT were Ewing sarcoma in 6, Wilms tumor in 6, medulloblastoma in 5, neuroblastoma in 3, and other in 3. Median RT dose was 45 Gy (range, 12.3 to 60 Gy) using 4 MV in 9, 1.25 MV in 8, 250 KV in 4, and 6 MV photons in 1 patient. One child was treated using 15-MeV electrons. Fourteen had chemotherapy. Median follow-up was 23.2 years (range, 5.3 to 44.4 years). For the 14 malignant neoplasms, the median time interval from initial tumor to second malignancy was 10.1 years. The 14 second malignant neoplasms (SMN) were osteosarcoma in 3, breast carcinoma in 2, melanoma in 2, malignant fibrous histiocytoma in 1, dermatofibrosarcoma in 1, leiomyosarcoma in 1, mucoepidermoid carcinoma in 1, colon cancer in 1, chronic myelogenous leukemia in 1, and basal cell carcinoma in 1. Ten of the 14 SMN (71%) were at the edge or inside the RT field. The 5- and 10-year overall survival rate after diagnosis of an SMN was 69.2%; it was 70% for children with a SMN at the edge or inside the RT field and 66.7% for those outside of the RT field. The 14 benign neoplasms appeared at a median time of 16.9 years and included cervical intraepithelial neoplasia in 3, osteochondroma in 3, thyroid adenoma in 1, duodenal adenoma in 1, lipoma in 1, cherry angioma in 1, uterine leiomyoma in 1, ovarian cystadenofibroma in 1, and giant cell tumor in 1. Only 5 (36%) of the 14 benign tumors occurred in the RT field, with osteochondroma being the most common. Of 189 deaths occurring in 429 patients, only 3 (1.6%) were secondary to radiation-induced malignancy. Not all SMN in children receiving RT occur in the irradiated field. More than two-thirds of children with a radiation-induced malignancy are alive 10 years after the diagnosis of a SMN.
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PMID:Secondary neoplasms after radiotherapy for a childhood solid tumor. 1580 94

Periosteal osteoblastoma is an extremely rare bone-forming neoplasm located on the surface of cortical bone. Of the fewer than 30 cases of periosteal osteoblastomas found in the literature, 2 have been reported to be located in cranial bone, and these have not been documented in detail with clinical history, radiographic findings, macroscopic features, and microscopic findings. Although the differential diagnoses of periosteal lesions include parosteal and periosteal osteosarcoma, periosteal chondroma and chondrosarcoma, osteochondroma, osteoid osteoma, periostitis ossificans, and myositis ossificans, an important differential diagnosis both radiologically and pathologically of such a lesion in the cranium is meningioma. We report an unusual case of periosteal osteoblastoma located in the frontal cranial bone that was radiologically consistent with a meningioma. The differential diagnosis of metaplastic meningioma with differentiation toward bone is discussed.
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PMID:A rare case of periosteal osteoblastoma located in the frontal cranial bone. 1591 30

Osteochondromas are common lesions in the metaphyseal segments of the long bones and are known to be able to degenerate into chondrosarcoma. We present the case of a 20-year-old patient with an osteosarcoma at the base of a cartilaginous exostosis and discuss the causal relationship between the two lesions on the basis of the radiologic and pathologic findings.
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PMID:Radiology-pathology conference: osteosarcoma in a cartilaginous exostosis of the femur. 1663 58

Osteosarcoma (OS) is the most common malignant bone tumor in children. To identify a plasma proteomic signature that can detect OS, we used SELDI MS to perform proteomic profiling on plasma specimens from 29 OS and 20 age-matched osteochondroma (OC) patients. Nineteen statistically significant ion peaks that were differentially expressed in OS when compared with OC patients were identified (p < 0.001 and false discovery rate < 10%). Using the proteomic profiles, we constructed a multivariate 3-nearest neighbors classifier to distinguish OS from OC patients with a sensitivity of 97% and a specificity of 80% based on external leave-one-out crossvalidation. Permutation test showed that the classification result was statistically significant (p < 0.00005). One of the proteins (m/z 11 704) in the proteomic signature was identified as serum amyloid protein A (SAA) by PMF. The higher plasma level of SAA in OS patients was further validated by Western blotting when compared to that of osteochrondroma patients and normal subjects as reference. The classifier based on this plasma proteomic signature may be useful to differentiate malignant bone cancer from benign bone tumors and for early detection of OS in high-risk individuals.
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PMID:Identification of a plasma proteomic signature to distinguish pediatric osteosarcoma from benign osteochondroma. 1667 37

Surface lesions of bone usually present little diagnostic dilemma because the majority are conventional osteochondromas. Other surface bone lesions include periosteal chondroma, periosteal chondrosarcoma, and parosteal osteosarcoma. Mineralized soft tissue lesions such as myositis ossificans, synovial chondroma, and synovial sarcoma may present in a similar fashion when they occur in a juxtaarticular position. The soft tissue osteochondroma or paraarticular osteochondroma may simulate some of these more aggressive tumors, and its recognition is important to avoid overtreatment. A case of an 11-year-old male with a soft tissue osteochondroma is reported to illustrate the characteristic radiographic and histological features of this rare entity. No prior reports have examined soft tissue osteochondroma for expression of parathyroid hormone related protein, an established cartilage tumor proliferative mitogen.
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PMID:Soft tissue osteochondroma: case report and immunohistochemistry for parathyroid hormone-related protein. 1684 64

Extraskeletal osteochondroma in the nape of the neck is rare and its pathological diagnosis is based on radiological and histopathological examination. It is vital that such a diagnosis be considered when a discrete, ossified mass is localised in soft tissues, even at atypical sites. Differential diagnoses include myositis ossificans, a lipomatous lesion, a pseudomalignant osseous tumour, an ossifying fibromyxoid tumour, an extraskeletal chondroma with endochondral ossification, synovial (osteo) chondromatosis, tumoural calcinosis, a synovial sarcoma, and an extraskeletal osteosarcoma. Clinical awareness of this benign entity is important as no malignant transformation or metastasis has been reported. Marginal excision with histopathological identification is the treatment of choice.
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PMID:Extraskeletal osteochondroma in the nape of the neck: a case report. 1691 87

"Bizarre parosteal osteochondromatous proliferations" are extremely rare manifestations first described by Nora and only a few more than 100 cases have been described in the international literature. In spite of clinically resembling an osteochondroma, a subungual exostosis or a parosteal osteosarcoma, the lesion is characterised by distinct radiological and histological findings. The lesion is defined as a reactive heterotopic ossification and is mostly found in the hands and feet of adults in the third decade of life. A high rate of local recurrence of up to 50% has been described, but since it is defined as a benign lesion no metastases can be found. We report the instructive case of a BPOP ("Nora's lesion") in the area of the proximal phalanx of the big toe in which, after performing a biopsy, a resection of the lesion under preservation of the big toe was performed. At the last follow-up examination (4 years postoperatively) the patient showed no sign of recurrence of the lesion. The clinical, radiological and MRI appearance of the lesion are described together with the distinct clinical findings.
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PMID:[The interesting case: Nora's lesion of the big toe]. 1760 32

The rapid development of an osteosarcoma, after surgical resection of an osteochondroma, has not been yet reported. We present here the case of a 12-year-old girl that had, in less than 2 months, an osteosarcoma at the initial site of a treated osteochondroma. Comparative Genomic Hybridization analyses showed that the 2 tumors were genetically distinct, suggesting a distant, if any, relationship. The possible implication of a deregulated tissue homeostasis caused by the surgical intervention is discussed. Proangiogenic factors involved in the tissue healing could be the triggering factor favoring tumor angiogenesis and explaining the very rapid progression of the tumor.
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PMID:Rapid development of an osteosarcoma after surgical resection of an osteochondroma. 1771 63

Parosteal osteosarcoma is a well-differentiated, predominantly fibro-osseous variant of osteosarcoma, accounting for 5% of all osteosarcomas. We report a case of parosteal osteosarcoma in the metaphyseal area of right femur, of 2 years' duration in a male aged 35 years. It was mistaken for osteochondroma in the initial biopsy, and the possibility of bizarre parosteal osteochondromatous proliferation (BPOP) was also considered. Subsequent excision of the tumor showed it to be a parosteal osteosarcoma. The patient had recurrence of the lesion after 1 year, and he attended a cancer institute. Follow-up showed metastases of the tumor on bone scan.
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PMID:Osteochondroma-like parosteal osteosarcoma. 1841 58


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