Common malignant tumors of the musculoskeletal system in children,adolescents, and young adults (<30 years old) include rhabdomyosarcoma,osteosarcoma, and Ewing's sarcoma. Together they constituteabout 10 percent of newly diagnosed cancers in children, adolescents,and young adults (about 1000 cases annually in the United States).Although relatively common in young persons, these tumors arerare in older people.
Rhabdomyosarcomas constitute more than half of all soft-tissuesarcomas in children.1 Other soft-tissue sarcomas are highlyheterogeneous and individually rare (e.g., synovial sarcomaand sarcomas of soft parts); thus, they are not discussed further.Osteosarcoma comprises several high-grade histologic variantsand . . . [Full Text of this Article]
Epidemiology
Clinical Features
Diagnosis and Staging
Morphologic and Immunohistochemical Features
Genetic Features
Rhabdomyosarcoma
Osteosarcoma
Ewing's Sarcoma
Treatment
Principles of Therapy
Rhabdomyosarcoma
Osteosarcoma
Ewing's Sarcoma
Treatment of Relapse
Future Directions
Source Information
From the Department of Pediatric and Adolescent Medicine, Mayo Clinic, Mayo Foundation, and Mayo Medical School, Rochester, Minn.
Address reprint requests to Dr. Arndt at the Department of Pediatric and Adolescent Medicine, Mayo Clinic, 200 First St. SW, Rochester, MN 55905, or at arndt.carola@mayo.edu.
Choy, E., Digumarthy, S. R., Koplin, S. A.
(2009). Case 36-2009 -- A 23-Year-Old Man with Cough, Hoarseness, and Abnormalities on Chest Imaging. NEJM
361: 2080-2087
[Full Text]
Laffan, E. E., Ngan, B.-Y., Navarro, O. M.
(2009). Pediatric Soft-Tissue Tumors and Pseudotumors: MR Imaging Features with Pathologic Correlation: Part 2. Tumors of Fibroblastic/Myofibroblastic, So-called Fibrohistiocytic, Muscular, Lymphomatous, Neurogenic, Hair Matrix, and Uncertain Origin1. RadioGraphics
29: e36-e36
[Abstract][Full Text]
Laffan, E. E., Ngan, B.-Y., Navarro, O. M.
(2009). Pediatric Soft-Tissue Tumors and Pseudotumors: MR Imaging Features with Pathologic Correlation: Part 2. Tumors of Fibroblastic/Myofibroblastic, So-called Fibrohistiocytic, Muscular, Lymphomatous, Neurogenic, Hair Matrix, and Uncertain Origin. RadioGraphics
0: e36-e
[Abstract][Full Text]
Bielack, S., Carrle, D., Casali, P. G., On behalf of the ESMO Guidelines Working Group,
(2009). Osteosarcoma: ESMO Clinical Recommendations for diagnosis, treatment and follow-up. Ann Oncol
20: iv137-iv139
[Full Text]
Nishijo, K., Chen, Q.-R., Zhang, L., McCleish, A. T., Rodriguez, A., Cho, M. J., Prajapati, S. I., Gelfond, J. A.L., Chisholm, G. B., Michalek, J. E., Aronow, B. J., Barr, F. G., Randall, R. L., Ladanyi, M., Qualman, S. J., Rubin, B. P., LeGallo, R. D., Wang, C., Khan, J., Keller, C.
(2009). Credentialing a Preclinical Mouse Model of Alveolar Rhabdomyosarcoma. Cancer Res.
69: 2902-2911
[Abstract][Full Text]
Dantonello, T. M., Int-Veen, C., Harms, D., Leuschner, I., Schmidt, B. F., Herbst, M., Juergens, H., Scheel-Walter, H.-G., Bielack, S. S., Klingebiel, T., Dickerhoff, R., Kirsch, S., Brecht, I., Schmelzle, R., Greulich, M., Gadner, H., Greiner, J., Marky, I., Treuner, J., Koscielniak, E.
(2009). Cooperative Trial CWS-91 for Localized Soft Tissue Sarcoma in Children, Adolescents, and Young Adults. JCO
27: 1446-1455
[Abstract][Full Text]
Chen, F., Miyahara, R., Bando, T., Okubo, K., Watanabe, K., Nakayama, T., Toguchida, J., Date, H.
(2008). Prognostic factors of pulmonary metastasectomy for osteosarcomas of the extremities. Eur. J. Cardiothorac. Surg.
34: 1235-1239
[Abstract][Full Text]
Kucharczak, J., Charrasse, S., Comunale, F., Zappulla, J., Robert, B., Teulon-Navarro, I., Pelegrin, A., Gauthier-Rouviere, C.
(2008). R-Cadherin Expression Inhibits Myogenesis and Induces Myoblast Transformation via Rac1 GTPase. Cancer Res.
68: 6559-6568
[Abstract][Full Text]
Mackall, C. L., Rhee, E. H., Read, E. J., Khuu, H. M., Leitman, S. F., Bernstein, D., Tesso, M., Long, L. M., Grindler, D., Merino, M., Kopp, W., Tsokos, M., Berzofsky, J. A., Helman, L. J.
(2008). A Pilot Study of Consolidative Immunotherapy in Patients with High-Risk Pediatric Sarcomas. Clin. Cancer Res.
14: 4850-4858
[Abstract][Full Text]
Dantonello, T. M., Int-Veen, C., Winkler, P., Leuschner, I., Schuck, A., Schmidt, B. F., Lochbuehler, H., Kirsch, S., Hallmen, E., Veit-Friedrich, I., Bielack, S. S., Niggli, F., Kazanowska, B., Ladenstein, R., Wiebe, T., Klingebiel, T., Treuner, J., Koscielniak, E.
(2008). Initial Patient Characteristics Can Predict Pattern and Risk of Relapse in Localized Rhabdomyosarcoma. JCO
26: 406-413
[Abstract][Full Text]
Kurmasheva, R. T., Easton, J. B., Houghton, P. J.
(2008). Combined Targeting of mTOR and the Insulin-like Growth Factor Pathway. Am Soc Clin Oncol Ed Book
2008: 460-464
[Abstract][Full Text]
Skubitz, K. M., D'Adamo, D. R.
(2007). Sarcoma. Mayo Clin Proc.
82: 1409-1432
[Abstract][Full Text]
Langenau, D. M., Keefe, M. D., Storer, N. Y., Guyon, J. R., Kutok, J. L., Le, X., Goessling, W., Neuberg, D. S., Kunkel, L. M., Zon, L. I.
(2007). Effects of RAS on the genesis of embryonal rhabdomyosarcoma. Genes Dev.
21: 1382-1395
[Abstract][Full Text]
Dormans, J. P., Moroz, L.
(2007). Infection and Tumors of the Spine in Children. JBJS
89: 79-97
[Full Text]
Galindo, R. L., Allport, J. A., Olson, E. N.
(2006). A Drosophila model of the rhabdomyosarcoma initiator PAX7-FKHR. Proc. Natl. Acad. Sci. USA
103: 13439-13444
[Abstract][Full Text]
Srivastava, A., Fuchs, B., Zhang, K., Ruan, M., Halder, C., Mahlum, E., Weber, K., Bolander, M. E., Sarkar, G.
(2006). High WT1 Expression Is Associated with Very Poor Survival of Patients with Osteogenic Sarcoma Metastasis.. Clin. Cancer Res.
12: 4237-4243
[Abstract][Full Text]
Whelan, J. P., Zembowicz, A.
(2006). Case records of the Massachusetts General Hospital. Case 19-2006. A 22-month-old boy with the rapid growth of subcutaneous nodules.. NEJM
354: 2697-2704
[Full Text]
Weisstein, J. S., Goldsby, R. E., O'Donnell, R. J.
(2005). Oncologic Approaches to Pediatric Limb Preservation. J Am Acad Orthop Surg
13: 544-554
[Abstract][Full Text]
Clark, M. A., Fisher, C., Judson, I., Thomas, J. M.
(2005). Soft-Tissue Sarcomas in Adults. NEJM
353: 701-711
[Full Text]
Rodeberg, D. A., Nuss, R. A., Elsawa, S. F., Celis, E.
(2005). Recognition of Six-Transmembrane Epithelial Antigen of the Prostate-Expressing Tumor Cells by Peptide Antigen-Induced Cytotoxic T Lymphocytes. Clin. Cancer Res.
11: 4545-4552
[Abstract][Full Text]
Kempf-Bielack, B., Bielack, S. S., Jurgens, H., Branscheid, D., Berdel, W. E., Exner, G. U., Gobel, U., Helmke, K., Jundt, G., Kabisch, H., Kevric, M., Klingebiel, T., Kotz, R., Maas, R., Schwarz, R., Semik, M., Treuner, J., Zoubek, A., Winkler, K.
(2005). Osteosarcoma Relapse After Combined Modality Therapy: An Analysis of Unselected Patients in the Cooperative Osteosarcoma Study Group (COSS). JCO
23: 559-568
[Abstract][Full Text]
Keller, C., Arenkiel, B. R., Coffin, C. M., El-Bardeesy, N., DePinho, R. A., Capecchi, M. R.
(2004). Alveolar rhabdomyosarcomas in conditional Pax3:Fkhr mice: cooperativity of Ink4a/ARF and Trp53 loss of function. Genes Dev.
18: 2614-2626
[Abstract][Full Text]
Zhang, J., Hu, S., Schofield, D. E., Sorensen, P. H. B., Triche, T. J.
(2004). Selective Usage of D-Type Cyclins by Ewing's Tumors and Rhabdomyosarcomas. Cancer Res.
64: 6026-6034
[Abstract][Full Text]
Tsukahara, T., Nabeta, Y., Kawaguchi, S., Ikeda, H., Sato, Y., Shimozawa, K., Ida, K., Asanuma, H., Hirohashi, Y., Torigoe, T., Hiraga, H., Nagoya, S., Wada, T., Yamashita, T., Sato, N.
(2004). Identification of Human Autologous Cytotoxic T-Lymphocyte-Defined Osteosarcoma Gene That Encodes a Transcriptional Regulator, Papillomavirus Binding Factor. Cancer Res.
64: 5442-5448
[Abstract][Full Text]
Paluska, S. A.
(2003). Persistent Knee Pain in a Recreational Runner. J Am Board Fam Med
16: 435-442
[Full Text]
Brenner, W., Bohuslavizki, K. H., Eary, J. F.
(2003). PET Imaging of Osteosarcoma. JNM
44: 930-942
[Abstract][Full Text]
Bielack, S. S., Kempf-Bielack, B., Delling, G., Exner, G. U., Flege, S., Helmke, K., Kotz, R., Salzer-Kuntschik, M., Werner, M., Winkelmann, W., Zoubek, A., Jurgens, H., Winkler, K.
(2002). Prognostic Factors in High-Grade Osteosarcoma of the Extremities or Trunk: An Analysis of 1,702 Patients Treated on Neoadjuvant Cooperative Osteosarcoma Study Group Protocols. JCO
20: 776-790
[Abstract][Full Text]
Bickert, B. M.
(2002). Treatment of Common Childhood Malignancies. Journal of Pharmacy Practice
15: 42-51
[Abstract]
Ahmad, S. A., Patel, S. R., Ballo, M. T., Baker, T. P., Yasko, A. W., Wang, X., Feig, B. W., Hunt, K. K., Lin, P. P., Weber, K. L., Chen, L. L., Zagars, G. K., Pollock, R. E., Benjamin, R. S., Pisters, P. W.T.
(2002). Extraosseous Osteosarcoma: Response to Treatment and Long-Term Outcome. JCO
20: 521-527
[Abstract][Full Text]
Braun, K. R., Zenel, J. A.
(2001). Visual Diagnosis: An Adolescent Who Has Swelling of the Foot. Pediatr. Rev.
22: 316-320
[Full Text]
Merino, M. E., Navid, F., Christensen, B. L., Toretsky, J. A., Helman, L. J., Cheung, N.-K. V., Mackall, C. L.
(2001). Immunomagnetic Purging of Ewing's Sarcoma From Blood and Bone Marrow: Quantitation by Real-Time Polymerase Chain Reaction. JCO
19: 3649-3659
[Abstract][Full Text]
Widhe, B., Widhe, T.
(2000). Initial Symptoms and Clinical Features in Osteosarcoma and Ewing Sarcoma. JBJS
82: 667-667
[Abstract][Full Text]
Puri, P. L., Wu, Z., Zhang, P., Wood, L. D., Bhakta, K. S., Han, J., Feramisco, J. R., Karin, M., Wang, J. Y.J.
(2000). Induction of terminal differentiation by constitutive activation of p38 MAP kinase in human rhabdomyosarcoma cells. Genes Dev.
14: 574-584
[Abstract][Full Text]
Mehta, P., Wingard, J. R., Arndt, C. A.S., Crist, W. M.
(1999). Musculoskeletal Tumors of Childhood. NEJM
341: 1701-1702
[Full Text]