DEFINITION AND PATHOGENESIS
- A conventional chondrosarcoma juxtaposed next to a high-grade (non-cartilaginous) sarcoma
- Two groups of precursor lesions have been identified
- Low-grade (to high-grade) malignant chondrosarcoma
- Moderate- to high-grade malignant chondrosarcoma, osteosarcoma, MFH, fibrosarcoma, or unspecified and anaplastic spindle-cell sarcoma
- Very aggressive and high grade chondrosarcoma that must be aggressively txd
- Transformation to a higher grade occurs in ~11% of chondrosarcomas
- 6th decade disease on the average (wide age range)
- Mean age 59
- M (53%) > F
- Pain, swelling
- 10-29% present with pathologic fx (rare for conventional chondrosarcoma)
- Most in femur or pelvis
- Unusual location
- Second area with aggressive characteristics
- Soft tissue mass with penetrated/destroyed cortex (72%) adjacent to a less aggressive area
- Type 1: resembles conventional central chondrosarcoma with cortical penetration, soft tissue mass, pathological fx, angiographic contrast uptake, extraosseous ossification, rapid progression
- Type 2: resembles conventional central chondrosarcoma with an aggressive component
- Type 3: no resemblance radiographically to conventional central chondrosarcoma
- Nearly 2/3 may be associated with a secondary chondrosarcoma
- Reported in a thumb PP fxd 15 yrs previously through an enchondroma
- Ollier's disease
- Most often > 7cm
- ± pathological fx
- Diaphyseal or metaphyseal
- Typically in the pelvis, femur, humerus
- Has been reported arising in a solitary osteochondroma
- dedifferentiated component can be leiomyosarcoma, osteosarcoma, MFH, and fibrosarcoma
- 3 categories of plain radiographic appearance
- Type I: cortex not deformed, no periosteal reaction, calcific matrix, and an indolent osteolytic reion with indistinct margins
- Type II: cortical expansion and/or with osteolytic areas
- Type III: cortex destroyed with osteolytic regions predominate
- Two areas may be distinctly visible on cross sectioning
- High-grade spindle cell sarcoma (not recognizable as cartilaginous in origin) next (an abrupt interface) to low-grade cartilage tissue
- Precursor lesion may be seen
- Dedifferentiated tumor in LR of previously low-grade chondrosarcoma (review previous pathology)
DIFFERENTIAL CLINICOPATHOLOGIC DIAGNOSIS
- Sarcomatous transformation of a bone infarct
- Conventional chondrosarcoma
- GCT due to lucency when in metaepiphyseal location
DISEASE COURSE AND TREATMENT
- Dx with FNA reported in a pelvic location
- (Presence of both low and high grade components documented)
- Wide resection and chemotx
- Wide range of chemotherapeutic regimens have been used without proven benefit or standardization
- Doxorubicin and cisplatin
- Doxorubicin and ifosfamide
- Wide range of chemotherapeutic regimens have been used without proven benefit or standardization
- XRT may be used preop in some of the larger lesions where margins may be close; the theory is that the tumor cells will not be able to seed the wound if a positive margin is obtained
- Lack of response to chemotx may be related to high likelihood of P-glycoprotein expression
- Pulmonary, skeletal, and visceral metastases occur with high risk
- ~7-24% 5-yr survivals have been reported (median survival < 9 months)
- With no metastases at presentation, overall survival 28% at 10 yrs reported
- Best prognosis in radiologic type 1 lesions
- Grade of dedifferentiated sarcoma the most important predictor of biological behavior (rather than type of tumor differentiation--osteoblastic or fibroblastic)
- MS0812 cell line in nude mice reportedly with fibrosarcoma with chondroid components
Abenoza P, Neumann P, Manivel JC, Wick MR: Dedifferentiated chondrosarcoma: an ultrastructural study of two cases, with immunocytochemical correlations. Ultrastruct Pathol 10:529-38,1986.
Aigner T, Unni KK: Is dedifferentiated chondrosarcoma a 'de-differentiated' chondrosama? J Pathol 189:445-7,1999.
Akahane T, Shimizu T, Isobe K, Yoshimura Y, Kato H: Dedifferentiated chondrosarcoma arising in a solitary osteochondroma with leiomyosarcomatous component: a case report. Arch Orthop Trauma Surg. 2008 Sep;128(9):951-3.
Arce FP, Pinto J, Portero I, Echevarria S, Val-Bernal JF: Cutaneous metastases as initial manifestation of dedifferentiated chondrosarcoma of bone. An autopsy case with review of the literature. J Cutan Pathol 27:262-7,2000.
Arora R, Sharma A, Dinda AK: Dedifferentiated chondrosarcoma of the femur mimicking a conventional giant cell tumor: a diagnostic pitfall. Indaina J Pathol Microbiol. 2008 Oct-Dec;51(4):561-2.
Bertoni F, Present D, Bacchini P, Picci P, Pignatti G, Gherlinzoni F, Campanacci M: Dedifferentiated peripheral chondrosarcomas. A report of seven cases. Cancer 63:2054-9,1989.
Bertoni F, Present D, Picci P, Bacchini P: Case report 301. Diagnosis: Dedifferentiated chondrosarcoma of the upper end of the humerus. Skletal Radiol 13:228-32,1985.
Bovee JV, Cleton-Jansen AM, Rosenberg C, Taminiau AH, Cornelisse CJ, Hogendoom PC: Molecular genetic characterization of both components of a dedifferentiated chondrosarcoma, with implications for its histogenesis. J Pathol 189:454-62,1999.
Bridge JA, DeBoer J, Travis J, Hohansson SL, Elmberger G, Noel SM, Neff JR: Simultaneous interphase cytogenetic analysis and fluorescence immunophenotyping of dedifferentiated chondrosarcoma. Implications for histopathogenesis. Am J Pathol 144:215-20,1994.
Campanacci M, Bertoni F, Capanna R: Dedifferentiated chondrosarcomas. Ital J Orthop Traumatol 5:331-41,1979.
Capanna R, Bertoni F, Bettelli G, Picci P, Bacchini P, Present D, Giunti A, Capanacci M: Dedifferentiated chondrosarcoma. J Bone Joint Surg 70A:60-9,1988.
Casorzo L, Chiecchio L, Pisacane A, Fumero S, Canavese F, Torchio B, Risjo M: Cytogenetic findings in a case of dedifferentiated chondrosarcoma. Cancer Genet Cytogenet 144:61-4,2003.
Dahlin DC, Beabout JW: Dedifferentiation of low-grade chondrosarcoma. Cancer 28:461-6,1971.
Daly PJ, Sim FH, Wold LE: Dedifferentiated chondrosarcoma of bone. Orthopedics 12:763-7,1989.
Dee S, Meneses M, Ostrowski ML, Murakami M, Horowitz M, Graf W: Pleomorphic ("dedifferentiated") chondrosarcoma. Report of a case initially examined by fine needle aspiration biopsy. Acta Cytol 35:467-71,1991.
de Lange EE, Pope TL, Fechner RE: Dedifferentiated chondrosarcoma: radiographic features. Radiology 161:489-92,1986.
Dervan PA, O'Loughlin J, Hurson BJ: Dedifferentiated chondrosarcoma with muscle and cytokeratin differentiation in the anaplastic component. Histopathology 12:517-26,1988.
Dickey ID, Rose PS, Fuchs B, Wold LE, Okuno SH, Sim FH, Scully SP: Dedifferentiated chondrosarcoma: the role of chemotherapy with updated outcomes. J Bone Joint Surg 86A:2412-8,2004.
Doganavsargil B, Argin M, Sezak M, Sabah D, Sarsik B, Omur O, Oztop F: Dedifferentiated chondrosarcoma of the thumb: a case report. Arch Orthop Trauma Surg. 2009 Feb;129(2):161-6.
Dornauer K, Soder S, Inwards CY, Bovee JV, Aigner T: Matrix biochemistry and cell biology of dedifferentiated chondrosarcomas. Pathol Int. 2010 May;60(5):365-72. Pubmed
Du YK, Shih HN, Wang JM, Shih CH: Dedifferentiated chondrosarcoma arising from osteochondromatosis. A case report. Changgeng Yi Xue Za Zhi 14:130-5,1991.
Estrada EG, Ayala AG, Lewis V, Czerniak B, Valerie L: Dedifferentiated chondrosarcoma with a noncartilaginous component mimicking a conventional giant cell tumor of bone. Ann Diagn Pathol 6:159-63,2002. Erratum in: Ann Diagn Pathol 7:336,2003.
Estrada-Villaseñor E, Rico-Martinez G, Linares-Gonzalez LM: Diagnosis of a dedifferentiated chondrosarcoma of the pelvis by fine needle aspiration. A case report. Acta Cytol. 2010 Mar-Apr;54(2):217-20.
Eustace S, Baker N, Lan H, Wadhwani A, Dorfman D: MR imaging of dedifferentiated chondrosarcoma. Clin Imaging 21:170-4,1997.
Frassica FJ, Unni KK, Beabout JW, Sim FH: Dedifferentiated chondrosarcoma. A report of the clinicopathological features and treatment of seventy-eight cases. J. Bone Joint Surg. 68-A:1197-1205,1986.
Frassica FJ, Unni KK, Sim FH: Case report 347. Dedifferentiated chondrosarcoma: grade 4 fibrosarcoma arising in grade 1 chondrosarcoma (femur). Skeletal Radiol 15:77-81,1986.
Garbyal RS, Gupta P, Kumar P, Mitra S, Bohra A: Fine needle aspiration cytology of a metastatic skin nodule diagnosed as a dedifferentiated chondrosarcoma. Acta Cytol. 2007 Nov-Dec;51(6):940-1.
Grimer RJ, Gosheger G, Taminiau A, Biau D, Matejovsky Z, Kollender Y, San-Julian M, Gherlinzoni F, Ferrari C: Dedifferentitated chondrosarcoma: prognostic factors and outcome from a European group. Eur J Cancer. 2007 Sep;43(14):2060-5. Pubmed
Grote HJ, Schneider-Stock R, Neumann W, Roessner A: Mutation of p53 with loss of heterozygosity in the osteosarcomatous component of dedifferentiated chondrosarcoma. Virchows Arch 436:404-7,2000.
Hackel C, Czerniak B, Ayala AG, Radig K, Roessner A: Expression of plasminogen activators and plasminogen activator inhibitor 1 in dedifferentiated chondrosarcoma. Cancer 79:53-8,1997.
Herman TE, McAlister WH, Dehner LP, Kaufman BA: Dedifferentiated chondrosarcoma in childhood: report of a case. Pediaatr Radiol 25(Suppl 1):S140-2,1995.
Huvos AG, Marcove RC: Chondrosarcoma in the young. A clinicopathologic analysis of 79 patients younger than 21 years of age. Am J Surg Pathol 11:930-42,1987.
Ishida T, Dorfman HD, Habermann ET: Dedifferentiated chondrosarcoma of humerus with giant cell tumor-like features. Skletal Radiol 24:76-80,1995.
Ishida T, Kuwada Y, Motoi N, Oka T, Machinami R: Dedifferentiated chondrosarcoma of the rib with a malignant mesenchymomatous component: an autopsy case report. Pathol Int 47:397-403,1997.
Jaworski RC: Dedifferentiated chondrosarcoma. An ultrasturctural study. Cancer 53:2674-8,1984.
Jaworski RC, Gibson M: Test and teach. Number forty-three. Diagnosis: dedifferentiated chondrosarcoma. Pathology 15:364-5,1983.
Johnson S, Tetu B, Ayala AG, Chawla SP: Chondrosarcoma with additional mesenchymal component (dedifferentiated chondrosarcoma). I. A clinicopathological study of 26 cases. Cancer 58:278-86,1986.
Karabela-Bouropoulou V, Markaki S, Kypparidou L, Stefis A, Prevedorou D: Dedifferentiated chondrosarcoma: a clinicopathological and immunohistochemical study of six cases. Arch Anat Cytol Pathol 36:218-25,1988.
Kahn LB: Chondrosarcoma with dedifferentiated foci. A comparative and ultrastructural study. Cancer 37:1365-75,1976.
Kilpatrick SE, Pike EJ, Ward WG, Pope TL: Dedifferentiated chondrosarcoma in patients with multiple osteochondromatosis: report of a case and review of the literature. Skletal Radiol 26:370-4,1997.
Kohli K, Lallawmzuali D, Kawatra V, Khurana N: Dedifferentiated chondrosarcoma of the hand: case report. Pathology. 2009;41(5):491-4.
Kumar S, Ferns S, Pereira I, Rao S: Dedifferentiated chondrosarcoma--an unusual presentation: a case report. Indian J Pathol Microbiol 47:46-7,2004.
Littrell LA, Wenger DE, Wold LE, Bertoni F, Unni KK, White LM, Kandel R, Sundaram M: Radiographic, CT, and MR imaging features of dedifferentiated chondrosarcoma: a retrospective review of 174 de novo cases. Radiographics 24:1397-409,2004.
MacSweeney F, Darby A, Saifuddin A: Dedifferentiated chondrosarcoma of the appendicular skeleton: MRI-pathological correlation. Skletal Radiol 32:671-8,2003.
Marco RAW, Gitelis S, Brebach GT, Healey JH: Cartilage tumors: evaluation and treatment. J Am Acad Orthop Surg 8:292-304,2000.
McFarland GB Jr, McKinley LM, Reed RJ: Dedifferentiation of low grade chondrosarcomas. Clin Orthop 122:157-64,1977.
Mercuri M, Picci P, Campanacci L, Rulli E: Dedifferentiated chondrosarcoma. Skeletal Radiol 24:409-16,1995.
Mikhail MG, Lim KB: Dedifferentiated chondrosarcoma metastasizing to the pancreas in pregnancy. Acta Obstet Gynecol Scand 68:467-8,1989.
Mirra JM: Dedifferentiated chondrosarcoma. Report of an unusual case. J Bone Joint Surg 73:1579-80,1991.
Mirra JM, Marcove RC: Fibrosarcomatous dedifferentiation of primary and secondary chondrosarcoma. Review of five cases. J Bone Joint Surg 56:285-96,1974.
Mirra JM, Picci P, and Gold RH. Intramedullary cartilage- and chondroid-producing tumors. In Bone Tumors. Clinical, radiologic and pathologic correlations. Lea & Febiger. Philadelphia, 1989, pp 439-690.
Mitchell AD, Ayoub K, Mangham DC, Grimer RJ, Carter SR, Tillman RM: Experience in the treatment of dedifferentiated chondrosarcoma. J Bone Joint Surg 82B:55-61,2000.
Mitchel A, Rudan JR, Fenton PV: Juxtacortical dedifferentiated chondrosarcoma from a primary periosteal chondrosarcoma. Mod Pathol 9:279-83,1996.
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.
Munk PL, Connell DG, Quenville NF: Dedifferentiated chondrosarcoma of bone with leiomyosarcomatous mesenchymal component: a case report. Can Assoc Radiol J 39:218-20,1988.
Munshi A, Atri SK, Pandey KC, Sharma MC: Dedifferentiated chondrosarcoma of the maxilla. J Cancer Res Ther. 2007 Jan-Mar;3(1):53-5.
Niezabitowski A, Edel G, Roessner A, Grundmann E, Timm C, Wuisman P: Rhabdomyosarcomatous component in dedifferentiated chondrosarcoma. Pathol Res Pract 182:275-82,1987.
O'Malley DP, Opheim KE, Barry TS, Chapman DB, Emond MJ, Conrad EU, Norwood TH: Chromosomal changes in a dedifferentiated chondrosarcoma: a case report and review of the literature. Cancer Genet Cytogenet 124:105-11,2001.
Ozaki T, Lindner N, Blasius S: Dedifferentiated chondrosarcoma in Albright syndrome. A case report and review of the literature. J Bone Joint Surg 79:1545-51,1997.
Park YK, Yang MH, Ryu KN, Chung DW: Dedifferentiated chondrosarcoma arising in an osteochondroma. Skletal Radiol 24:617-9,1995.
Radhi JM, Loewy J: Dedifferentiated chondrosarcoma with features of telangiectatic osteosarcoma. Pathology 31:428-30,1999.
Reith JD, Bauer TW, Fischler DF, Joyce MJ, Marks KE: Dedifferentiated chondrosaromca with rhabdomyosarcomatous differentiation. Am J Surg Pathol 20:293-8,1996.
Remagen W, Jani L, Luthi A, Schuman L: Atypical (dedifferentiated) chondrosarcoma or osteosarcoma with preponderant chondroblastic differentiation? J Cancer Res Clin Oncol 101:177-82,1981.
Ropke M, Boltze C, Neumann HW, Roessner A, Schneider-Stock R: Genetic and epigenetic alterations in tumor progression in a dedifferentiated chondrosarcoma. Pathol Res Pract 199:437-44,2003.
Saifuddin A, Mass BS, Mahroof S, Pringle JA, Briggs TW, Cannon SR: Dedifferentiated chondrosarcoma: use of MRI to guide neeld biopsy. Clin Radiol 59:268-72,2004.
Sakamoto A, Oda Y, Adachi T, Oshiro Y, Tamiya S, Tanaka K, Matsuda S, Iwamoto Y, Tsuneyoshi M: H-ras oncogene mutation in dedifferentiated chondrosarcoma: plymerase chain reaction-restriction fragment length polymorphism analysis. Mod Pathol 14:343-9,2001.
Sakamoto A, Oda Y, Iwamoto Y, Tsuneyoshi M: Expression of membrane type 1 matrix metalloproteinase 2 in human cartilaginous tumors with special emphasis on mesenchymal and dedifferentiated chondrosarcoma. J Cancer Res Clin Oncol 125:541-8,1999.
Sannerkin NG, Woods CG: Fibrosarcomata and malignant fibrous histiocytoma arising in relation to endochondromata. J Bone Joint Surg 61B:366-72,1979.
Simms WW, Ordonez NG, Johnston D, Ayala AG, Czerniak B: p53 expression in dedifferentiated chondrosarcoma. Cancer 76:223-7,1995.
Sissons HA, Matlen JA, Lewis MM: Dedifferentiated chondrosarcoma. Report of an unusual case. J Bone Joint Surg 73:294-300,1991.
Sopta J, Dordevic A, Tulic G, Mijucic V: Dedifferentiated chondrosarcoma: our clinico-pathological experience and dilemmas in 25 cases. J Cancer Res Clin Oncol. 2008 Feb;134(2):147-52.
Springfield DS, Gebhardt MC, McGuire MH: Chondrosarcoma: a review. J. Bone Joint Surg. 78-A:141-149,1996.
Staals EL, Bacchini P, Mercuri M, Bertoni F: Dedifferentiated chondrosarcomas arising in preexisting osteochondromas. J Bone Joint Surg 89:987-993,2007.
Steurer S, Huber M, Lintner F: Dedifferentiated chondrosarcoma of the lung: case report and review of the literature. Clin Lung Cancer. 2007 Jul;8(7):439-42.
Swarts SJ, Neff JR, Johansson SL, Bridge JA: Cytogenetic analysis of dedifferentiated chondrosaoma. Cancer Genet Cytogenet 89:49-51,1996.
Tarkkanen M, Wiklund T, Virolainen M, Elomaa I, Knuutila S: Dedifferentiated chondrosarcoma with t(9;22)(q34;q11-12). Genes Chromosomes Cancer 9:136-40,1994.
Terek RM, Schwartz GK, Devaney K, Glantz L, Mak S, Healey JH, Albino AP: Chemotherapy and P-glycoprotein expression in chondrosarcoma. J Orthop Res 16:585-90,1998.
Tetu B, Ordonez NG, Ayala G, Mackay B: Chondrosarcoma with additional mesenchymal component (dedifferentiated chondrosarcoma). II. An immunohistochemical and electron microscopic study. Cancer 58:287-98,1986.
Tsuchiya H, Tomita K, Yasutake H, Takagi Y, Ueda Y, Kadoya M: Maffucci's syndrome combined with dedifferentiated chondrosarcoma. Arch Orthop Trauma Surg 110:269-72,1991.
Van Hoe L, Lateur L, Sciot R: Case report: Metastatic dedifferentiated chondrosarcoma. Clin Radiol 51:373-6,1996.
Weber KL, Raymond AK: Low-grade/dedifferentiated/high-grade chondrosarcoma: a case of histological and biological progression. Iow Orthop J 22:75-80,2002.
Wick MR, Siegal GP, Mills SE, Thompson RC, Sawhney D, Fechner RE: Dedifferentiated chondrosarcoma of bone. An immunohistochemical and lectin-histochemical study. Virchows Arch A Pathol Anat Histopathol 411:23-32,1987.
Yang L, Chen Q, Zhang S, Wang X, Li W, Wen J, Huang X, Zheng J, Huang G, Huang X, Zheng J, Huang G, Huang T, Ju G: A novel mutated cell line with characteristics of dedifferentiated chondrosarcoma. Int J Mol Med. 2009 Oct;24(4):427-35.
Zalupski MM, Ensley JF, Ryan J, Selvaggi S, Baker LH, Wolman SR: A common cytogenetic abnormality and DNA content alterations in dedifferentiated chondrosarcoma. Cancer 66:1176-82,1990.