Dedifferentiated Chondrosarcoma with a High-Grade Mesenchymal Component Mimicking a Gastrointestinal Stromal Tumor


This report presents a dedifferentiated chondrosarcoma with a unique pathologic feature. A 63-year-old man was referred with pain and a soft tissue mass in the left groin. A plain radiograph showed a mineralization in the proximal femur with partially osteolytic foci and an abnormal shadow in the soft tissue. Magnetic resonance imaging scans showed an inhomogeneous lesion with intermediate to partially low signal intensity on T1-weighted image and intermediate to high signal intensity on T2-weighted image. Microscopically, the tumor in the femur is a low-grade chondrosarcoma and the component of soft tissue was a high-grade sarcomatous lesion with an epithelial arrangement of tumor cells. A diffuse immunoreactivity to both vimentin and c-kit (CD117) antibodies was detected in the high-grade component. A dedifferentiated component is similar to those of gastrointestinal stromal tumor (GIST). This is the first case of dedifferentiated chondrosarcoma with a high-grade component mimicking a GIST.

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T. Akisue, K. Kishimoto, T. Kawamoto, H. Hara and M. Kurosaka, "Dedifferentiated Chondrosarcoma with a High-Grade Mesenchymal Component Mimicking a Gastrointestinal Stromal Tumor," Open Journal of Pathology, Vol. 2 No. 3, 2012, pp. 90-95. doi: 10.4236/ojpathology.2012.23017.

Conflicts of Interest

The authors declare no conflicts of interest.


[1] D. C. Dahlin and J. W. Beabout, “Dedifferentiation of Low-Grade Chondrosarcomas,” Cancer, Vol. 28, No. 2, 1971, pp. 461-466. doi:10.1002/1097-0142(197108)28:2<461::AID-CNCR2820280227>3.0.CO;2-U
[2] F. J. Frassica, K. K. Unni, J. W. Beabout and F. H. Sim, “Dedifferentiated Chondrosarcoma. A Report of the Clinicopathological Features and Treatment of Seventy-Eight Cases,” Journal of Bone & Joint Surgery, Vol. 68, No. 8, 1986, pp. 1197-1205.
[3] I. D. Dickey, P. S. Rose, B. Fuchs, L. E. Wold, S. H. Okuno, F. H. Sim and S. P. Scully, “Dedifferentiated Chondrosarcoma: The Role of Chemotherapy with Updated Outcomes,” Journal of Bone & Joint Surgery, Vol. 86, No. 11, 2004, pp. 2412-2418.
[4] J. Bruns, W. Fiedler, M. Werner and G. Delling, “Dedifferentiated Chondrosarcoma—A Fatal Disease,” Journal of Cancer Research and Clinical Oncology, Vol. 131, No. 6, 2005, pp. 333-339. doi:10.1007/s00432-004-0648-6
[5] K. Okada, T. Hasegawa, U. Tateishi, M. Endo and E. Itoi, “Dedifferentiated Chondrosarcoma with Telangiectatic Osteosarcoma-Like Features,” Journal of Clinical Pathology, Vol. 59, No. 11, 2006, pp. 1200-1202. doi:10.1136/jcp.2005.029629
[6] J. Sopta, A. Dordevic, G. Tulic and V. Mijucic, “Dedifferentiated Chondrosarcoma: Our Clinico-Pathological Experience and Dilemmas in 25 Cases,” Journal of Cancer Research and Clinical Oncology, Vol. 134, No. 2, 2008, pp. 147-152. doi:10.1007/s00432-007-0262-5
[7] R. N. Astorino and H. Tesluk, “Dedifferentiated Chondrosarcoma with a Rhabdomyosarcomatous Component,” Human Pathology, Vol. 16, No. 3, 1985, pp. 318-320. doi:10.1016/S0046-8177(85)80022-8
[8] J. D. Reith, T. W. Bauer, D. F. Fischler, M. J. Joyce and K. E. Marks, “Dedifferentiated Chondrosarcoma with Rhabdomyosarcomatous Differentiation,” American Journal of Surgical Pathology, Vol. 20, No. 3, 1996, pp. 293-298. doi:10.1097/00000478-199603000-00005
[9] P. L. Munk, D. G. Connell and N. F. Quenville, “Dedifferentiated Chondrosarcoma of Bone with Leiomyosarcomatous Mesenchymal Component: A Case Report,” Canadian Association of Radiologists Journal, Vol. 39, No. 3, 1988, pp. 218-220.
[10] L. A. Littrell, D. E. Wenger, L. E. Wold, F. Bertoni, K. K. Unni, L. M. White, R. Kandel and M. Sundaram, “Radiographic, CT, and MR Imaging Features of Dedifferentiated Chondrosarcomas: A Retrospective Review of 174 de Novo Cases,” Radiographics, Vol. 24, No. 5, 2004, pp. 1397-1409. doi:10.1148/rg.245045009
[11] T. Akahane, T. Shimizu, K. Isobe, Y. Yoshimura and H. Kato, “Dedifferentiated Chondrosarcoma Arising in a Solitary Osteochondroma with Leiomyosarcomatous Component: A Case Report,” Archives of Orthopaedic and Trauma Surgery, Vol. 128, No. 9, 2008, pp. 951-953. doi:10.1007/s00402-008-0567-0
[12] H. A. Sissons, J. A. Matlen and M. M. Lewis, “Dedifferentiated Chondrosarcoma. Report of an Unusual Case,” Journal of Bone & Joint Surgery, Vol. 73, No. 2, 1991, pp. 294-300.
[13] T. Ishida, H. D. Dorfman and E. T. Habermann, “Dedifferentiated Chondrosarcoma of Humerus with Giant Cell Tumor-Like Features,” Skeletal Radiology, Vol. 24, No. 1, 1995, pp. 76-80. doi:10.1007/BF02425959
[14] E. G. Estrada, A. G. Ayala, V. Lewis and B. Czerniak, “Dedifferentiated Chondrosarcoma with a Noncartilaginous Component Mimicking a Conventional Giant Cell Tumor of Bone,” Annals of Diagnostic Pathology, Vol. 6, No. 3, 2002, pp. 159-163. doi:10.1053/adpa.2002.33905
[15] R. Arora, A. Sharma and A. K. Dinda, “Dedifferentiated Chondrosarcoma of the Femur Mimicking a Conventional Giant Cell Tumor: A Diagnostic Pitfall,” Indian Journal of Pathology and Microbiology, Vol. 51, No. 4, 2008, pp. 561-562. doi:10.4103/0377-4929.43763
[16] M. E. Pring, K. L. Weber, K. K. Unni and F. H. Sim, “Chondrosarcoma of the Pelvis. A Review of Sixty-Four Cases,” Journal of Bone & Joint Surgery, Vol. 83, No. 11, 2001, pp. 1630-1642.
[17] M. T. Mazur and H. B. Clark, “Gastric Stromal Tumors. Reappraisal of Histogenesis,” American Journal of Surgical Pathology, Vol. 7, No. 6, 1983, pp. 507-519. doi:10.1097/00000478-198309000-00001
[18] S. Hirota, K. Isozaki, Y. Moriyama, K. Hashimoto, T. Nishida, S. Ishiguro, K. Kawano, M. Hanada, A. Kurata, M. Takeda, G. M. Tunio, Y. Matsuzawa, Y. Kanakura, Y. Shinomura and Y. Kitamura, “Gain-of-Function Mutations of c-kit in Human Gastrointestinal Stromal Tumors,” Science, Vol. 279, No. 5350, 1998, pp. 577-580. doi:10.1126/science.279.5350.577
[19] L. G. Kindblom, H. E. Remotti, F. Aldenborg and J. M. Meis-Kindblom, “Gastrointestinal Pacemaker Cell Tumor (GIPACT): Gastrointestinal Stromal Tumors Show Phenotypic Characteristics of the Interstitial Cells of Cajal,” American Journal of Pathology, Vol. 152, No. 5, 1998, pp. 1259-1269.
[20] S. Eustace, N. Baker, H. Lan, A. Wadhwani and D. Dorfman, “MR Imaging of Dedifferentiated Chondrosarcoma,” Clinical Imaging, Vol. 21, No. 3, 1997, pp. 170-174. doi:10.1016/S0899-7071(96)00016-2
[21] F. MacSweeney, A. Darby and A. Saifuddin, “Dedifferentiated Chondrosarcoma of the Appendicular Skeleton: MRI-Pathological Correlation,” Skeletal Radiology, Vol. 32 No. 12, 2003, pp. 671-678. doi:10.1007/s00256-003-0706-1
[22] A. D. Mitchell, K. Ayoub, D. C. Mangham, R. J. Grimer, S. R. Carter and R. M. Tillman, “Experience in the Treatment of Dedifferentiated Chondrosarcoma,” Journal of Bone & Joint Surgery, British Volume, Vol. 82, No. 1, 2000, pp. 55-61. doi:10.1302/0301-620X.82B1.9020
[23] R. J. Grimer, G. Gosheger, A. Taminiau, D. Biau, Z. Matejovsky, Y. Kollender, M. San-Julian, F. Gherlinzoni and C. Ferrari, “Dedifferentiated Chondrosarcoma: Prognostic Factors and Outcome from a European Group,” European Journal of Cancer, Vol. 43, No. 14, 2007, pp. 2060-2065. doi:10.1016/j.ejca.2007.06.016
[24] E. L. Staals, P. Bacchini, M. Mercuri and F. Bertoni, “Dedifferentiated Chondrosarcomas Arising in Preexisting Osteochondromas,” Journal of Bone & Joint Surgery, Vol. 89, No. 5, 2007, pp. 987-993. doi:10.2106/JBJS.F.00288

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