Abstract
Low-grade central osteosarcoma (LGCOS) is a rare variant of osteosarcoma. We present a rare case of multifocal LGCOS located in two distinct skeletal sites, initially noted as incidental findings on imaging for distant traumatic pathology. Both sites seemed small and innocuous on initial imaging, and were quiescent clinically, illustrating the value of close interval multimodal surveillance scanning.
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References
Silverman G. Multiple osteogenic sarcoma. Arch Pathol. 1936;21(5):88–95.
Kurt AM, Unni KK, McLeod RA, Pritchard DJ. Low-grade intraosseous osteosarcoma. Cancer. 1990;65(6):1418–28.
Bertoni F, Bacchini P, Fabbri N, Mercuri M, Picci P, Ruggieri P, et al. Osteosarcoma: low-grade intraosseous-type osteosarcoma, histologically resembling parosteal osteosarcoma, fibrous dysplasia, and desmoplastic fibroma. Cancer. 1993;71(2):338–45.
Choong PF, Pritchard DJ, Rock MG, Sim FH, McLeod RA, Unni KK. Low grade central osteogenic sarcoma: a long-term followup of 20 patients. Clin Orthop Relat Res. 1996;322:198–206.
Andresen KJ, Sundaram M, Unni KK, Sim FH. Imaging features of low-grade central osteosarcoma of the long bones and pelvis. Skeletal Radiol. 2004;33(7):373–9.
Unni KK, Dahlin DC, McLeod RA, Pritchard DJ. Intraosseous well-differentiated osteosarcoma. Cancer. 1977;40(3):1337–47.
Anninga JK, Picci P, Fiocco M, Kroon HM, Vanel D, Alberghini M, et al. Osteosarcoma of the hands and feet: a distinct clinico-pathological subgroup. Virchows Arch. 2013;462(1):109–20.
Ellis JH, Siegel CL, Martel W, Weatherbee L, Dorfman H. Radiologic features of well-differentiated osteosarcoma. AJR Am J Roentgenol. 1988;151(4):739–42.
Okada K, Nishida J, Morita T, Kakizaki H, Ishikawa A, Hotta T. Low-grade intraosseous osteosarcoma in northern Japan: advantage of AgNOR and MIB-1 staining in differential diagnosis. Hum Pathol. 2000;31(6):633–9.
Malhas AM, Sumathi VP, James SL, Menna C, Carter SR, Tillman RM, et al. Low-grade central osteosarcoma: a difficult condition to diagnose. Sarcoma. 2012;2012:764796.
Kenan S, Ginat DT, Steiner GC. Dedifferentiated high-grade osteosarcoma originating from low-grade central osteosarcoma of the fibula. Skeletal Radiol. 2007;36(4):347–51.
Simpfendorfer CS et al. Does the presence of focal normal marrow fat signal within a tumor on MRI exclude malignancy? An analysis of 184 histologically proven tumors of the pelvic and appendicular skeleton. Skeletal Radiol. 2008;37(9):797–804.
Antonescu CR, Huvos AG. Low-grade osteogenic sarcoma arising in medullary and surface osseous locations. Am J Clin Pathol. 2000;114(Suppl):S90–103.
Enneking WF, Kagan A. “Skip” metastases in osteosarcoma. Cancer. 1975;36(6):2192–205.
Bonar SF. Central low-grade osteosarcoma: a diagnostic challenge. Skeletal Radiol. 2012;41(4):365–7.
Ogose A, Hotta T, Emura I, Imaizumi S, Takeda M, Yamamura S. Repeated dedifferentiation of low-grade intraosseous osteosarcoma. Hum Pathol. 2000;31(5):615–8.
Franceschina MJ, Hankin RC, Irwin RB. Low-grade central osteosarcoma resembling fibrous dysplasia: a report of two cases. Am J Orthop (Belle Mead NJ). 1997;26(6):432–40.
Muramatsu K, Hashimoto T, Seto S, Gondo T, Ihara K, Taguchi T. Low-grade central osteosarcoma mimicking fibrous dysplasia: a report of two cases. Arch Orthop Trauma Surg. 2008;128(1):11–5.
Franchi A, Bacchini P, Della Rocca C, Bertoni F. Central low-grade osteosarcoma with pagetoid bone formation: a potential diagnostic pitfall. Mod Pathol. 2004;17(3):288–91.
Klein MJ, Siegal GP. Osteosarcoma: anatomic and histologic variants. Am J Clin Pathol. 2006;125(4):555–81.
Steiner GC, Forest M, Vacher-Lavenu MC. Ultrastructure of low-grade intraosseous osteosarcoma of bone: a comparative study with fibrous dysplasia and parosteal osteosarcoma. Ultrastruct Pathol. 2006;30(4):293–9.
Pollandt K, Engels C, Kaiser E, Werner M, Delling G. Gsalpha gene mutations in monostotic fibrous dysplasia of bone and fibrous dysplasia-like low-grade central osteosarcoma. Virchows Arch. 2001;439(2):170–5.
Idowu BD, Al-Adnani M, O’Donnell P, Yu L, Odell E, Diss T, et al. A sensitive mutation-specific screening technique for GNAS1 mutations in cases of fibrous dysplasia: the first report of a codon 227 mutation in bone. Histopathology. 2007;50(6):691–704.
Park HR, Jung WW, Bertoni F, Bacchini P, Park JH, Kim YW, et al. Molecular analysis of p53, MDM2 and H-ras genes in low-grade central osteosarcoma. Pathol Res Pract. 2004;200(6):439–45.
Lee WI, Bacchni P, Bertoni F, Maeng YH, Park YK. Quantitative assessment of HER2/neu expression by real-time PCR and fluorescent in situ hybridization analysis in low-grade osteosarcoma. Oncol Rep. 2004;12(1):125–8.
Yoshida A, Ushiku T, Motoi T, Shibata T, Beppu Y, Fukayama M, et al. Immunohistochemical analysis of MDM2 and CDK4 distinguishes low-grade osteosarcoma from benign mimics. Mod Pathol. 2010;23(9):1279–88.
Dujardin F et al. MDM2 and CDK4 immunohistochemistry is a valuable tool in the differential diagnosis of low-grade osteosarcomas and other primary fibro-osseous lesions of the bone. Mod Pathol. 2011;24(5):624–37.
Yoshida A, Ushiku T, Motoi T, Beppu Y, Fukayama M, Tsuda H, et al. MDM2 and CDK4 immunohistochemical coexpression in high-grade osteosarcoma: correlation with a dedifferentiated subtype. Am J Surg Pathol. 2012;36(3):423–31.
Ragazzini P, Gamberi G, Benassi MS, Orlando C, Sestini R, Ferrari C, et al. Analysis of SAS gene and CDK4 and MDM2 proteins in low-grade osteosarcoma. Cancer Detect Prev. 1999;23(2):129–36.
Nishio J, Iwasaki H, Takagi S, Seo H, Aoki M, Nabeshima K, et al. Low-grade central osteosarcoma of the metatarsal bone: a clinicopathological, immunohistochemical, cytogenetic and molecular cytogenetic analysis. Anticancer Res. 2012;32(12):5429–35.
Jeon DG, Koh JS, Cho WH, Song WS, Kong CB, Cho SH, et al. Clinical outcome of low-grade central osteosarcoma and role of CDK4 and MDM2 immunohistochemistry as a diagnostic adjunct. J Orthop Sci. 2015;20(3):529–37.
Lietman SA, Ding C, Levine MA. A highly sensitive polymerase chain reaction method detects activating mutations of the GNAS gene in peripheral blood cells in McCune-Albright syndrome or isolated fibrous dysplasia. J Bone Joint Surg Am. 2005;87(11):2489–94.
Lee SE, Lee EH, Park H, Sung JY, Lee HW, Kang SY, et al. The diagnostic utility of the GNAS mutation in patients with fibrous dysplasia: meta-analysis of 168 sporadic cases. Hum Pathol. 2012;43(8):1234–42.
Carter JM, Inwards CY, Jin L, Evers B, Wenger DE, Oliveira AM, et al. Activating GNAS mutations in parosteal osteosarcoma. Am J Surg Pathol. 2014;38(3):402–9.
Iemoto Y, Ushigome S, Fukunaga M, Nikaido T, Asanuma K. Case report 679: central low-grade osteosarcoma with foci of dedifferentiation. Skeletal Radiol. 1991;20(5):379–82.
Mirra JM. Osseous tumors of intramedullary origin. In: Bone Tumors. Clinical, radiologic and pathologic correlations. Vol 1. London: Lea and Febiger Philadelphia; 1989. p 143–488.
Price CH, Truscott DE. Multifocal osteogenic sarcoma; report of a case. J Bone Joint Surg (Br). 1957;39-b(3):524–33.
Amstutz HC. Multiple osteogenic sarcomata: metastatic or multicentric? Report of two cases and review of literature. Cancer. 1969;24(5):923–31.
Daffner RH, Kennedy SL, Fox KR, Crowley JJ, Sauser DD, Cooperstein LA. Synchronous multicentric osteosarcoma: the case for metastases. Skeletal Radiol. 1997;26(10):569–78.
Enneking WF, Springfield D, Gross M. The surgical treatment of parosteal osteosarcoma in long bones. J Bone Joint Surg Am. 1985;67(1):125–35.
Hatori M, Ohtani H, Yamada N, Uzuki M, Kokubun S. Synchronous multifocal osteosarcoma with lymphatic spread in the lung: an autopsy case report. Jpn J Clin Oncol. 2001;31(11):562–6.
Bacci G, Picci P, Ferrari S, Mercuri M, Briccoli A, Donati D, et al. Synchronous multifocal osteosarcoma: results in twelve patients treated with neoadjuvant chemotherapy and simultaneous resection of all involved bones. Ann Oncol. 1996;7(8):864–6.
Meyers PA, Heller G, Healey J, Huvos A, Lane J, Marcove R, et al. Chemotherapy for nonmetastatic osteogenic sarcoma: the Memorial Sloan-Kettering experience. 1992;10(1):5–15.
McCarthy EF, Tolo VT, Dorfman HD. Case report 446. Multicentric, metachronous, low-grade, sclerosing osteogenic sarcoma. Skeletal Radiol. 1987;16(7):592–6.
Arkader A, Huvos AG, Healey JH. Multifocal synchronous low-grade intramedullary and juxtacortical osteogenic sarcoma. Clin Orthop Relat Res. 2007;465:265–70.
Acknowledgements
Prof Adrienne Flanagan and Dr Fernanda Amary at Royal National Orthopaedic Hospital, UK for GNAS testing.
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All authors have made substantial contributions to all three categories established by the ICMJE to include: conception and design, or acquisition of data, or analysis and interpretation of data; drafting the article or revising it critically for important intellectual content; and final approval of the version to be published.
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Boyle, R., Giuffre, B. & Bonar, S.F. Multifocal low-grade central osteosarcoma: a rare case. Skeletal Radiol 45, 997–1006 (2016). https://doi.org/10.1007/s00256-016-2384-9
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DOI: https://doi.org/10.1007/s00256-016-2384-9