Conventional and dedifferentiated parosteal osteosarcoma: Diagnosis, treatment, and outcome

Sheth, Dhiren S.; Yasko, Alan W.; Raymond, A. Kevin; Ayala, Alberto G.; Carrasco, C. H.; Benjamin, Robert S.; Jaffe, Norman; Murray, J. A. H.

doi:10.1002/(sici)1097-0142(19961115)78:10<2136::aid-cncr14>3.3.co;2-y

Cited by 23 publications

(49 citation statements)

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“…These rates fall within the reported rates of dedifferentiation in juxtacortical osteogenic sarcomas which range from 16% to 43% [2,8,9,13]. The largest reported series of low-grade intramedullary osteogenic sarcoma had a rate of dedifferentiation of 25% [4].…”

Section: Discussionsupporting

confidence: 75%

A Comparison of Intramedullary and Juxtacortical Low-grade Osteogenic Sarcoma

Schwab

Antonescu

Athanasian

et al. 2008

Clinical Orthopaedics &Amp; Related Research

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While low-grade juxtacortical and low-grade intramedullary osteogenic sarcomas are histologically indistinguishable, they have been studied as separate entities. We retrospectively reviewed the clinical, radiographic, histologic features and treatment of 59 patients treated surgically to compare the rate of local recurrence, grade progression, and survival between low-grade intramedullary and low-grade juxtacortical osteogenic sarcoma. Forty-five (76%) patients were treated for low-grade juxtacortical osteogenic sarcoma and 14 (24%) were treated for low-grade intramedullary osteogenic sarcoma. Local recurrence rates of 7% were similar for both groups studied. The rate of distant metastases was also similar for both groups. . The rate of dedifferentiation for the entire group was 29%. Dedifferentiated lesions were treated with adjuvant chemotherapy in 16 of 17 cases. Recurrence preceded dedifferentiation in four cases. Five-year survival was over 90% in both groups. Low-grade intramedullary and low-grade juxtacortical osteogenic sarcoma were clinically indistinguishable with identical rates of local recurrence, distant metastases, dedifferentiation, and survival.

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Section: Discussionsupporting

confidence: 75%

A Comparison of Intramedullary and Juxtacortical Low-grade Osteogenic Sarcoma

Schwab

Antonescu

Athanasian

et al. 2008

Clinical Orthopaedics &Amp; Related Research

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“…Parosteal OS, central low-grade OS, and periosteal OS are morphologically and clinically distinct OS subtypes with an improved prognosis 7 and constitute less than 5% of cases of OS. [8][9][10][11][12][13][14] The age at presentation for parosteal and periosteal OS is usually in the fourth and fifth decades of life (patients are usually in the 30-to 40-year age range). The microscopic diagnosis of OS rests on the identification of production of osteoid matrix by the neoplastic cells (Figure 1, top).…”

Section: Biology and Pathology Osteosarcomamentioning

confidence: 99%

Common Musculoskeletal Tumors of Childhood and Adolescence

Arndt¹,

Rose²,

Folpe³

et al. 2012

SciVee

129

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Osteosarcoma, Ewing sarcoma, and rhabdomyosarcoma are the most common malignant musculoskeletal tumors in children and adolescents. Today, most patients can be cured. Numerous factors have contributed to improved outcome for these patients over the past several decades. These include multidisciplinary care involving oncologists, radiation oncologists, surgeons, pathologists, and radiologists and enrollment of patients in clinical trials. Better understanding of molecular mechanisms of disease have resulted in studies using molecular targets in addition to standard chemotherapeutic agents, which hopefully will lead to better outcomes in the future. Moreover, new orthopedic techniques and devices as well as new technologies in radiation oncology hold promise for better local control of primary tumors and the potential for fewer late adverse effects. Despite this progress, patients must undergo lifelong follow-up for possible late effects of intense chemotherapy and radiation therapy. We review the diagnosis, prognosis, staging, multidisciplinary therapy, new directions in therapy, and long-term complications of treatment for these tumors. For this review, we searched MEDLINE using the terms rhabdomyosarcoma, osteosarcoma, Ewing sarcoma, biology, and humans and limited the search to articles from 2000 to September 2011. Additional references found in these articles were utilized as appropriate, as well as references from the background information in current therapeutic studies of the Children's Oncology Group. The same database and time frame were searched for articles written by leading authorities in the field.

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“…Dedifferentiation affects 16-43% of parosteal osteosarcomas with the characteristic gene amplifications being retained and can be present at the first presentation (synchronous type) or at the time of recurrence (metachronous type). [7][8][9] Bone tumors analyzed previously for GNAS alterations include fibrous dysplasia (N = 405), [10][11][12][13][14][15][16] ossifying fibroma (N = 65), 10,12,[15][16] osteofibrous dysplasia (N = 19), 10,12-13 low-grade central osteosarcoma (N = 12) 10,13-14 , and parosteal osteosarcoma (N = 10). 10 GNAS mutations have been reported in fibrous dysplasia and have not been described until recently in any other fibrous osseous lesions with the exception of a single low-grade central osteosarcoma.…”

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confidence: 99%

GNAS mutations are not detected in parosteal and low-grade central osteosarcomas

et al. 2015

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Parosteal osteosarcoma, low-grade central osteosarcoma, and fibrous dysplasia share similar histological features that may pose a diagnostic challenge. The detection of GNAS mutations in primary bone tumors has been useful in clinical practice for diagnosing fibrous dysplasia. However, the recent report of GNAS mutations being detected in a significant proportion of parosteal osteosarcoma challenges the specificity of this mutation. As the number of cases reported in this study was small we set out to determine if these results could be reproduced. We studied 97 formalin-fixed paraffin-embedded low-grade osteosarcomas from 90 patients including 62 parosteal osteosarcomas, of which MDM2 amplification was detected in 79%, 11 periosteal osteosarcomas and 24 low-grade central osteosarcoma samples. The mutational status of GNAS was analyzed in codons p.R201, p.Q227, and other less common GNAS alterations by bidirectional Sanger sequencing and/or next generation sequencing using the Life Technologies Ion Torrent platform. GNAS mutations were not detected in any of the low-grade osteosarcomas from which informative DNA was extracted. Our findings therefore support prior observations that GNAS mutations are highly specific for fibrous dysplasia and occur rarely, if ever, in parosteal and other low-grade osteosarcomas. Parosteal and low-grade central osteosarcomas are rare low-grade primary malignant bone tumors comprising o4% of all osteosarcomas. In contrast, fibrous dysplasia is one of the most common benign fibro-osseous tumor-like lesions in medullary bone. Morphologically, there is considerable overlap between these lesions all showing variably shaped bony trabeculae surrounded by spindled-shaped cells, with little to no cytologic atypia. 1 In the majority of cases, imaging studies can easily differentiate parosteal osteosarcoma from fibrous dysplasia and central low-grade osteosarcoma: parosteal osteosarcoma is a surface tumor that may secondarily invade bone marrow, and low-grade central osteosarcoma and fibrous dysplasia are centrally based. Occasionally, fibrous dysplasia arises eccentrically in the marrow space, presenting as an exophytic surfacebased lesion. Such lesions are referred to as fibrous dysplasia protuberans. 2 Hence, distinguishing fibrous dysplasia protuberans from parosteal osteosarcoma can be challenging and differentiating between lowgrade central osteosarcoma and fibrous dysplasia can also be difficult.Parosteal osteosarcoma typically harbors one or more supernumerary ring chromosomes with amplification of the MDM2, SAS, and CDK4 genes. 3,4 MDM2 amplification is reported to occur in 93% (14/15) of low-grade central osteosarcomas 3,5 and in 79% (68/86) of parosteal osteosarcomas. 5,6 Dedifferentiated parosteal osteosarcoma is a distinct tumor variant in which a (high-grade) sarcoma coexists with a conventional parosteal osteosarcoma. Dedifferentiation affects 16-43% of parosteal osteosarcomas

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Conventional and dedifferentiated parosteal osteosarcoma: Diagnosis, treatment, and outcome

Abstract: Wide surgical excision alone is adequate treatment for patients with c-POS. Recognition of dedifferentiated areas with angiography and percutaneous biopsy of hypervascular areas should prompt the administration of chemotherapy and wide local excision to optimize patient outcome.

Cited by 23 publications

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A Comparison of Intramedullary and Juxtacortical Low-grade Osteogenic Sarcoma

A Comparison of Intramedullary and Juxtacortical Low-grade Osteogenic Sarcoma

Common Musculoskeletal Tumors of Childhood and Adolescence

GNAS mutations are not detected in parosteal and low-grade central osteosarcomas

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