Nonrhabdomyosarcoma Soft Tissue Sarcomas Differential Diagnoses
- Author: Justine K Walker, MD; Chief Editor: Robert J Arceci, MD, PhD more...
Diagnostic Considerations
Other malignancies that cause masses in children must be considered during evaluation. Examples include lymphomas, osteosarcoma, Ewing sarcoma, rhabdomyosarcoma, and neuroblastoma. Benign lesions (eg, lipomas, rhabdomyomas) should be considered as well.
Differential Diagnoses
- Aggressive fibromatosis (Desmoid tumor)
- Cysts
- Dermatofibroma
- Ewing Sarcoma and Primitive Neuroectodermal Tumors
- Langerhans cell histiocytosis
- Lipoma
- Neuroblastoma
- Neurofibroma
Loh ML, Ahn P, Perez-Atayde AR, et al. Treatment of infantile fibrosarcoma with chemotherapy and surgery: results from the Dana-Farber Cancer Institute and Children's Hospital, Boston. J Pediatr Hematol Oncol. 2002;24 (9):722-6. [Medline].
McArthur GA, Demetri GD, van Oosterom A, Heinrich MC, Debiec-Rychter M, Corless CL. Molecular and clinical analysis of locally advanced dermatofibrosarcoma protuberans treated with imatinib: Imatinib Target Exploration Consortium Study B2225. J Clin Oncol. Feb 1 2005;23(4):866-73. [Medline].
Brecht IB, Ferrari A, Int-Veen C, et al. Grossly-resected synovial sarcoma treated by the German and Italian Pediatric Soft Tissue Sarcoma Cooperative Groups: discussion on the role of adjuvant therapies. Pediatric Blood and Cancer. 2006;47:11-17. [Medline]. [Full Text].
Okcu MF, Munsell M, Treuner J, et al. Synovial sarcoma of childhood and adolescence: a multicenter, multivariate analysis of outcome. J Clin Oncol. 2003;21:1602-11. [Medline].
Faulkner LB, Hajdu SI, Kher U, et al. Pediatric desmoid tumor: retrospective analysis of 63 cases. J Clin Oncol. 1995;13:2813-8. [Medline].
Skapek SX, Ferguson WS, Granowetter L, et al. Vinblastine and methotrexate for desmoid fibromatosis in children: results of a Pediatric Oncology Group Phase II Trial. J Clin Oncol. 2007;25 (5):501-6. [Medline].
Daller JA, Bueno J, Gutierrez J, et al. Hepatic hemangioendothelioma: clinical experience and management strategy. J Pediatr Surg. 1999;34:98-105. [Medline].
Randall RL, Albritton KH, Ferney BJ, Layfield L. Malignant fibrous histiocytoma of soft tissue: an abandoned diagnosis. Am J Orthop (Belle Mead NJ). Dec 2004;33(12):602-8. [Medline].
Erlandson RA, Antonescu CR. The rise and fall of malignant fibrous histiocytoma. Ultrastruct Pathol. Sep-Dec 2004;28(5-6):283-9. [Medline].
Federman N, Feig SA. PET/CT in evaluating pediatric malignancies: a clinician's perspective. J Nucl Med. Dec 2007;48(12):1920-2. [Medline].
McCarville MB, Christie R, Daw NC, et al. PET/CT in the evaluation of childhood sarcomas. AJR Am J Roentgenol. Apr 2005;184(4):1293-304. [Medline].
American Joint Committee on Cancer. AJCC Cancer Staging Manual. 6th. New York, NY: Springer; 2002.
Krasin MJ, Davidoff AM, Spunt SL, et al. Preliminary results from a prospective study using limited margin radiotherapy in pediatric and young adult patients with high-grade nonrhabdomyosarcoma soft-tissue sarcoma. Int J Radiat Oncol Biol Phys. Mar 2010;76 (3):874-8. [Medline].
Phase III Study of Risk-Based Therapy Comprising Observation Only, Adjuvant Radiotherapy, or Adjuvant Chemoradiotherapy or Neoadjuvant Chemoradiotherapy, Surgery, and Adjuvant Chemotherapy With or Without Radiotherapy in Young Patients With Non-Rhabdomyosarcoma Soft Tissue Sarcoma. Available at http://www.hemonc.theclinics.com/article/S0889-8588(05)00034-1/abstract.
Stacchiotti S, Tamborini E, Marrari A, et al. Response to sunitinib malate in advanced alveolar soft part sarcoma. Clin Cancer Res. 2009;15 (3):1096-104. [Medline].
Sleijfer S, Ray-Coquard I, Papai Z, et al. Pazopanib, a multikinase angiogenesis inhibitor, in patients with relapsed or refractory advanced soft tissue sarcoma: a phase II study from the European organisation for research and treatment of cancer- soft tissue and bone sarcoma group (EORTC study 62043). J Clin Oncol. 2009;27 (19):3126-32. [Medline].
Putnam JB Jr, Roth JA. Surgical treatment for pulmonary metastases from sarcoma. Hematol Oncol Clin North Am. 1995;9 (4):869-87. [Medline].
Albritton KH. Sarcomas in adolescents and young adults. Hematol Oncol Clin North Am. 2005;19:527-546. [Medline]. [Full Text].
Antonescu CR. The role of genetic testing in soft tissue sarcoma. Histopathology. Jan 2006;48(1):13-21. [Medline].
Blakely ML, Spurbeck WW, Pappo AS, et al. The impact of margin of resection on outcome in pediatric nonrhabdomyosarcoma soft tissue sarcoma. J Pediatr Surg. May 1999;34(5):672-5. [Medline].
Dillon PW. Nonrhabdomyosarcoma soft tissue sarcomas in children. Seminars in Pediatric Surgery. 1997;6(1):24-28. [Medline].
Kayton ML, Meyers P, Wexler LH, et al. Clinical presentation, treatment, and outcome of alveolar soft part sarcoma in children, adolescents, and young adults. J Pediatr Surg. 2006;41:187-193. [Medline].
Lanzkowsky P. Rhabdomyosarcoma and other soft tissue sarcomas. In: Manual of Pediatric Hematology and Oncology. 2005. 4th ed. San Diego, Calif: Academic; 561-584.
Loeb DM, Thornton K, Shokek O. Pediatric soft tissue sarcomas. Surg Clin North Am. Jun 2008;88(3):615-27, vii. [Medline].
Marcus KC, Grier HE, Shamberger RC, et al. Childhood soft tissue sarcoma: a 20-year experience. J Pediatr. Oct 1997;131(4):603-7. [Medline].
Marcus RB Jr. Current controversies in pediatric radiation oncology. Orthop Clin North Am. Jul 1996;27(3):551-7. [Medline].
Meyer WH, Spunt SL. Soft tissue sarcomas of childhood. Cancer Treat Rev. 2004;30:269-80. [Medline]. [Full Text].
Okeu M, Hicks J, Merchant TE, Andrassy RJ, Pappo AS, Horowitz ME. Non-rhabdomyosarcomatous soft tissue sarcomas. In: Pizzo PA, Poplack DG. Principles and Practice of Pediatric Oncology. 5th. Philadelphia: Lippincott Williams and Wilkins; 2006:1034-1073.
Pappo AS. Rhabdomyosarcoma and other soft tissue sarcomas in children. Curr Opin Oncol. Jul 1996;8(4):311-6. [Medline].
Paulides M, Kremers A, Stohr W, Bielack S, Jurgens H, Treuner J. Prospective longitudinal evaluation of doxorubicin-induced cardiomyopathy in sarcoma patients: a report of the late effects surveillance system (LESS). Pediatr Blood Cancer. Apr 2006;46(4):489-95. [Medline].
Paulino AC. Treatment options for children with nonrhabdomyosarcoma soft tissue sarcoma. Expert Rev Anticancer Ther. 2004;4:247-56. [Medline].
Sirvent N, Maire G, Pedeutour F. Genetics of dermatofibrosarcoma protuberans family of tumors: from ring chromosomes to tyrosine kinase inhibitor treatment. Genes Chromosomes Cancer. May 2003;37(1):1-19. [Medline].
Spunt S, Wolden S, Schofield D, Skapek S. Non-Rhabdomyosarcoma soft tissue sarcomas. In: Pappo, Alberto. Pediatric Bone and Soft Tissue Sarcomas. New York: Springer; 2006:133-162.
Spunt SL, Poquette CA, Hurt YS, et al. Prognostic factors for children and adolescents with surgically resected nonrhabdomyosarcoma soft tissue sarcoma: an analysis of 121 patients treated at St Jude Children's Research Hospital. J Clin Oncol. Dec 1999;17(12):3697-705. [Medline].
Spunt SL, Skapek SX, Coffin CM. Pediatric nonrhabdomyosarcoma soft tissue sarcomas. Oncologist. Jun 2008;13(6):668-78. [Medline].
Womer RB, Sinniah D. Soft tissue sarcomas. In: Practical Pediatric Oncology. 1992:318-23.
| Primary Tumor | Regional Lymph Nodes | Distant Metastasis | Histologic Grade | |
| Stage I | Any tumor size, superficial or deep | N0 | M0 | G1 or G2 |
| Stage II | T1a (tumor < 5 cm, superficial) | N0 | M0 | G3 |
| T1b (tumor < 5 cm, deep) | N0 | M0 | G3 | |
| T2a (tumor >5 cm, superficial) | N0 | M0 | G3 | |
| Stage III | T2b (tumor >5 cm, deep) | N0 | M0 | G3 |
| Stage IV | Any tumor size, superficial or deep | N1 | M0 or M1 | G1, G2, or G3 |
| Any tumor size, superficial or deep | N0 or N1 | M1 | G1, G2, or G3 |

