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Secondary Chondrosarcoma, Exostotic

DEFINITION AND PATHOGENESIS

  • Primarily arising secondarily from a surface exostosis or periosteal chondroma, either low (Grade I) or intermediate (Grade II) in grade

IMPORTANCE

  • Must be differentiated from an osteochondroma or periosteal chondroma
  • A dx of multiple hereditary exostoses is a risk factor, also Ollier disease and Maffucci syndrome

CLINICAL FEATURES

  • In a pt with multiple hereditary osteochondromatoses, new onset of pain or growing mass, esp in the shoulder or hip/pelvic region should arouse the suspicion for malignant degeneration
  • Sudden growth in a cartilage cap with marked enlargement of the cap strongly suggests secondary chondrosarcoma
  • Unusual location:
  • M  > F
    • ~2:1 overall in secondary chondrosarcoma
  • Average age 34 yrs (younger than primary conventional chondrosarcoma) 

RADIOLOGIC FEATURES

  • Appears as an osteochondroma with an attached mass
  • Most occur in the pelvis, femur, humerus, tibia, ribs
  • Growth of previously stable osteochondroma, bone erosion, irregular or scattered calcification
  • Normal bone scan virtually excludes diagnosis of malignant transformation of an exostosis
  • Intramedullary extension may occur in up to 1/3 of cases

GROSS PATHOLOGY

  • Soft tissue mass associated wtih an exostosis
  • Thickened cartilage cap

HISTOLOGIC/MOLECULAR FEATURES

DIFFERENTIAL CLINICOPATHOLOGIC DIAGNOSIS

  • Osteochondroma with an overlying bursal sac
  • Large atypical osteochondroma

DISEASE COURSE AND TREATMENT

  • Rarely metastasizes
  • Should be widely resected, without violation of the cartilage cap or associated soft tissue mass

REFERENCES

Ahmed AR, Tan TS, Unni KK, Collins MS, Wenger DE, Sim FH. Secondary chondrosarcoma in osteochondroma: report of 107 patients. Clin Orthop Relat Res. 2003 Jun;(411):193-206. Pubmed

Altay M, Bayrakci K, Yildiz Y, Erekul S, Saglik Y. Secondary chondrosarcoma in cartilage bone tumors: report of 32 patients. J Orthop Sci. 2007 Sep;12(5):415-23. Pubmed
Aprin H, Riseborough EJ, Hall JE. Chondrosarcoma in children and adolescents. Clin Orthop Relat Res. 1982 Jun;(166):226-32.
Barrett AW, Hopper C, Speight PM. Oral presentation of secondary chondrosarcoma arising in osteochondroma of the nasal septum. Int J Oral Maxillofac Surg. 1996 Apr;25(2):119-21.
Chadha M, Singh AP. Secondary chondrosarcoma of the cuboid bone in a patient with multiple exostoses. Can J Surg. 2008 Feb;51(1):E5-6.

Coley BL, Higinbotham NL: Secondary chondrosarcoma. Ann Surg 139:547-55,1954.
de Andrea CE, Reijnders CM, Kroon HM, de Jong D, Hogendoorn PC, Szuhai K, Bovee JV: Secondary peripheral chondrosarcoma evolving from osteochondroma as a result of outgrowth of cells with functional EXT. Oncogen. 2011 Aug 1. doi: 10.1038/onc.2011.311. Pubmed

Garrison R, Unni K, McLeod R, Pritchard DJ, Dahlin DC: Chondrosarcoma arising in osteochondroma. Cancer 49:1890-7,1982.
Hudson TM, Springfield DS, Spanier SS, Enneking WF, Hamlin DJ: Benign exostoses and exostotic chondrosarcomas: evaluation of cartilage thickness by CT. Radiology 152:595-9,1984.
Kilpatrick SE, Pike EJ, Ward WG, Pope TR: Dedifferentiated chondrosarcoma in patients with multiple osteochondromatosis: report of a case and review of the literature. Skeletal Radiol 26:370-4,1997.

Kivioja A, Ervasti H, Kinnunen J, Kaitila I, Wolf M, Böhling T: Chondrosarcoma in a family with multiple hereditary exostoses. J Bone Joint Surg 82B:261-6,2000.

Lee FY, Zawadsky M, Parisien M, Ho M, Murty VV, Jayaraman T, Dick HM. Novel translocation (9;12)(q22;q24) in secondary chondrosarcoma arising from hereditary multiple exostosis. Cancer Genet Cytogenet. 2002 Jan 1;132(1):68-70.
Lin PP, Moussallem CD, Deavers MT: Secondary chondrosarcoma. J Am Acad Orthop Surg 18:608-615, 2010. Pubmed

Marco RAW, Gitelis S, Brebach GT, Healey JH: Cartilage tumors: evaluation and treatment. J Am Acad Orthop Surg 8:292-304,2000.

Merchan EC, Sanchez-Herrera S, Gonzalez JM. Secondary chondrosarcoma. Four cases and review of the literature. Acta Orthop Belg. 1993;59(1):76-80. Pubmed

Mirra JM, Picci P, and Gold RH. Parosteal tumors. In Bone Tumors. Clinical, radiologic and pathologic correlations. Lea & Febiger. Philadelphia, 1989, pp 1626-1683.
Moser RP, Jr:: Cartilaginous Tumors of the Skeleton. AFIP Atlas of Radiologic-Pathologic Correlations. Fascicle II. Davidson AJ (Ed). Mosby-Year Book, St. Louis, 1990.
Norman A, Sissons HA: Radiographic hallmarks of peripheral chondrosarcoma. Radiology 151:589- ,1984.
Saunders C, Szabo RM, Mora S: Chondrosarcoma of the hand arising in a young patient with multiple hereditary exostoses. J Hand Surg 22B:237-42,1997.
Springfield DS, Gebhardt MC, McGuire MH: Chondrosarcoma: a Review. J. Bone Joint Surg. 78-A:141-149, 1996.

Wuisman PI, Jutte PC, Ozaki T. Secondary chondrosarcoma in osteochondromas. Medullary extension in 15 or 45 cases. Acta Orthop Scand. 1997 Aug;68(4):396-400.
Xu B, Shi H, Wang S, Wang P, Yu Q. Secondary chondrosarcoma in the mandibular condyle. Dentomaxillofac Radiol. 2011 Jul;40(5):320-3.

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