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Malignant Soft Tissue Lesions

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The Pediatric Upper Extremity

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Abstract

Soft-tissue sarcomas of the upper extremity are extremely rare, particularly within the pediatric population. Optimal management is heavily dependent upon early recognition, proper diagnostic evaluation, and referral to a pediatric sarcoma center for both tissue biopsy and definitive management. Cure is most likely realized in the setting of truly localized disease, where complete surgical resection can be accomplished. Despite advances in adjuvant therapy, surgery remains the most efficacious treatment modality for localized soft-tissue sarcomas, with radiation augmenting local control primarily for large high-grade lesions. Adherence to fundamental sarcoma surgery principles is critical to oncologic outcomes and should take priority over functional results. A thorough understanding of upper extremity compartmental anatomy is necessary in order to plan and execute complete tumor extirpation. The role for chemotherapy continues to evolve, though adjuvant administration for large high-grade tumors and palliative administration for metastatic disease are common. While over 50 types of soft-tissue sarcoma have been described, some of the more commonly encountered pediatric soft-tissue sarcomas of the upper extremity include alveolar rhabdomyosarcoma, epithelioid sarcoma, synovial sarcoma, infantile fibrosarcoma, dermatofibrosarcoma protuberans, and malignant peripheral nerve sheath tumors.

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References

  • Al Yami A, Griffin AM, Ferguson PC, Catton CN, Chung PW, Bell RS, Wunder JS, O’Sullivan B. Positive surgical margins in soft tissue sarcoma treated with preoperative radiation: is a postoperative boost necessary? Int J Radiat Oncol Biol Phys. 2010;77(4):1191–7.

    Article  PubMed  Google Scholar 

  • Anderson MW, Temple HT, Dussault RG, Kaplan PA. Compartmental anatomy: relevance to staging and biopsy of musculoskeletal tumors. AJR Am J Roentgenol. 1999;173(6):1663–71.

    Article  CAS  PubMed  Google Scholar 

  • Andrassy RJ, Wiener ES, Raney RB, Lawrence W, Lobe TE, Corpron CA, Maurer HM. Thoracic sarcomas in children. Ann Surg. 1998;227(2):170–3.

    Article  CAS  PubMed Central  PubMed  Google Scholar 

  • Andrassy RJ, Okcu MF, Despa S, Raney RB. Synovial sarcoma in children: surgical lessons from a single institution and review of the literature. J Am Coll Surg. 2001;192(3):305–13.

    Article  CAS  PubMed  Google Scholar 

  • Athanasian E. Biopsy of musculoskeletal tumors. In: Menendez L, editor. Orthopaedic knowledge update. Rosemont: American Academy of Orthopaedic Surgery; 2002. p. 29–34.

    Google Scholar 

  • Athanasian EA. Malignant bone and soft-tissue sarcomas of the hand. J Am Soc Surg Hand. 2004;4(2):60–72.

    Article  Google Scholar 

  • Bleyer WA. What can be learned about childhood cancer from “cancer statistics review 1973–1988”. Cancer. 1993;71(10 Suppl):3229–36.

    Article  CAS  PubMed  Google Scholar 

  • Bleyer WA. The U.S. pediatric cancer clinical trials programmes: international implications and the way forward. Eur J Cancer. 1997;33(9):1439–47.

    Article  CAS  PubMed  Google Scholar 

  • Bowne WB, Antonescu CR, Leung DH, Katz SC, Hawkins WG, Woodruff JM, Brennan MF, Lewis JJ. Dermatofibrosarcoma protuberans: a clinicopathologic analysis of patients treated and followed at a single institution. Cancer. 2000;88(12):2711–20.

    Article  CAS  PubMed  Google Scholar 

  • Brien EW, Terek RM, Geer RJ, Caldwell G, Brennan MF, Healey JH. Treatment of soft-tissue sarcomas of the hand. J Bone Joint Surg Am. 1995;77(4):564–71.

    CAS  PubMed  Google Scholar 

  • Bryan RS, Soule EH, Dobyns JH, Pritchard DJ, Linscheid RL. Primary epithelioid sarcoma of the hand and forearm. A review of thirteen cases. J Bone Joint Surg Am. 1974;56(3):458–65.

    CAS  PubMed  Google Scholar 

  • Carli M, Ferrari A, Mattke A, Zanetti I, Casanova M, Bisogno G, Cecchetto G, Alaggio R, De Sio L, Koscielniak E, Sotti G, Treuner J. Pediatric malignant peripheral nerve sheath tumor: the Italian and German soft tissue sarcoma cooperative group. J Clin Oncol. 2005;23(33):8422–30.

    Article  PubMed  Google Scholar 

  • Chase DR, Enzinger FM. Epithelioid sarcoma. Diagnosis, prognostic indicators, and treatment. Am J Surg Pathol. 1985;9(4):241–63.

    Article  CAS  PubMed  Google Scholar 

  • Chen Y, Yang Y, Wang C, Shi Y. Adjuvant chemotherapy decreases and postpones distant metastasis in extremity stage IIB/III synovial sarcoma patients. J Surg Oncol. 2012;106(2):162–8.

    Article  PubMed  Google Scholar 

  • Chung EB, Enzinger FM. Infantile fibrosarcoma. Cancer. 1976;38(2):729–39.

    Article  CAS  PubMed  Google Scholar 

  • Coffin CM, Dehner LP. Fibroblastic-myofibroblastic tumors in children and adolescents: a clinicopathologic study of 108 examples in 103 patients. Pediatr Pathol. 1991;11(4):569–88.

    Article  CAS  PubMed  Google Scholar 

  • Coffin CM, Jaszcz W, O’Shea PA, Dehner LP. So-called congenital-infantile fibrosarcoma: does it exist and what is it? Pediatr Pathol. 1994;14(1):133–50.

    Article  CAS  PubMed  Google Scholar 

  • Dal Cin P, Brock P, Casteels-Van Daele M, De Wever I, Van Damme B, Van den Berghe H. Cytogenetic characterization of congenital or infantile fibrosarcoma. Eur J Pediatr. 1991;150(8):579–81.

    Article  CAS  PubMed  Google Scholar 

  • de Visscher SA, van Ginkel RJ, Wobbes T, Veth RP, Ten Heuvel SE, Suurmeijer AJ, Hoekstra HJ. Epithelioid sarcoma: still an only surgically curable disease. Cancer. 2006;107(3):606–12.

    Article  PubMed  Google Scholar 

  • deCou JM, Rao BN, Parham DM, Lobe TE, Bowman L, Pappo AS, Fontanesi J. Malignant peripheral nerve sheath tumors: the St. Jude Children’s Research Hospital experience. Ann Surg Oncol. 1995;2(6):524–9.

    Article  CAS  PubMed  Google Scholar 

  • Demir HA, Varan A, Yalcn B, Akyuz C, Kutluk T, Buyukpamukcu M. Malignant peripheral nerve sheath tumors in childhood: 13 cases from a single center. J Pediatr Hematol Oncol. 2012;34(3):204–7.

    Article  PubMed  Google Scholar 

  • Dias P, Chen B, Dilday B, Palmer H, Hosoi H, Singh S, Wu C, Li X, Thompson J, Parham D, Qualman S, Houghton P. Strong immunostaining for myogenin in rhabdomyosarcoma is significantly associated with tumors of the alveolar subclass. Am J Pathol. 2000;156(2):399–408.

    Article  CAS  PubMed Central  PubMed  Google Scholar 

  • Doorn PF, Molenaar WM, Buter J, Hoekstra HJ. Malignant peripheral nerve sheath tumors in patients with and without neurofibromatosis. Eur J Surg Oncol. 1995;21(1):78–82.

    Article  CAS  PubMed  Google Scholar 

  • Ducatman BS, Scheithauer BW, Piepgras DG, Reiman HM, Ilstrup DM. Malignant peripheral nerve sheath tumors. A clinicopathologic study of 120 cases. Cancer. 1986;57(10):2006–21.

    Article  CAS  PubMed  Google Scholar 

  • Eilber FC, Brennan MF, Eilber FR, Eckardt JJ, Grobmyer SR, Riedel E, Forscher C, Maki RG, Singer S. Chemotherapy is associated with improved survival in adult patients with primary extremity synovial sarcoma. Ann Surg. 2007;246(1):105–13.

    Article  PubMed Central  PubMed  Google Scholar 

  • Enzinger FM. Epithelioid sarcoma. A sarcoma simulating a granuloma or a carcinoma. Cancer. 1970;26(5):1029–41.

    Article  CAS  PubMed  Google Scholar 

  • Evans DG, Baser ME, McGaughran J, Sharif S, Howard E, Moran A. Malignant peripheral nerve sheath tumours in neurofibromatosis 1. J Med Genet. 2002;39(5):311–4.

    Article  CAS  PubMed Central  PubMed  Google Scholar 

  • Fisher C. Synovial sarcoma. Ann Diagn Pathol. 1998;2(6):401–21.

    Article  CAS  PubMed  Google Scholar 

  • Fong Y, Coit DG, Woodruff JM, Brennan MF. Lymph node metastasis from soft tissue sarcoma in adults. Analysis of data from a prospective database of 1772 sarcoma patients. Ann Surg. 1993;217(1):72–7.

    Article  CAS  PubMed Central  PubMed  Google Scholar 

  • Gadd S, Beezhold P, Jennings L, George D, Leuer K, Huang CC, Huff V, Tognon C, Sorensen PH, Triche T, Coffin CM, Perlman EJ. Mediators of receptor tyrosine kinase activation in infantile fibrosarcoma: a Children’s Oncology Group study. J Pathol. 2012;228(1):119–30.

    CAS  PubMed  Google Scholar 

  • Gronchi A, Lo Vullo S, Colombo C, Collini P, Stacchiotti S, Mariani L, Fiore M, Casali PG. Extremity soft tissue sarcoma in a series of patients treated at a single institution: local control directly impacts survival. Ann Surg. 2010;251(3):506–11.

    Article  PubMed  Google Scholar 

  • Gustafson P, Dreinhofer KE, Rydholm A. Soft tissue sarcoma should be treated at a tumor center. A comparison of quality of surgery in 375 patients. Acta Orthop Scand. 1994;65(1):47–50.

    Article  CAS  PubMed  Google Scholar 

  • Halling AC, Wollan PC, Pritchard DJ, Vlasak R, Nascimento AG. Epithelioid sarcoma: a clinicopathologic review of 55 cases. Mayo Clin Proc. 1996;71(7):636–42.

    Article  CAS  PubMed  Google Scholar 

  • Harms D. New entities, concepts, and questions in childhood tumor pathology. Gen Diagn Pathol. 1995;141(1):1–14.

    CAS  PubMed  Google Scholar 

  • Heerema-McKenney A, Wijnaendts LC, Pulliam JF, Lopez-Terrada D, McKenney JK, Zhu S, Montgomery K, Mitchell J, Marinelli RJ, Hart AA, van de Rijn M, Linn SC. Diffuse myogenin expression by immunohistochemistry is an independent marker of poor survival in pediatric rhabdomyosarcoma: a tissue microarray study of 71 primary tumors including correlation with molecular phenotype. Am J Surg Pathol. 2008;32(10):1513–22.

    Article  PubMed  Google Scholar 

  • Herr MJ, Harmsen WS, Amadio PC, Scully SP. Epithelioid sarcoma of the hand. Clin Orthop Relat Res. 2005;431:193–200.

    Article  PubMed  Google Scholar 

  • Iwasaki H, Enjoji M. Infantile and adult fibrosarcomas of the soft tissues. Acta Pathol Jpn. 1979;29(3):377–88.

    CAS  PubMed  Google Scholar 

  • Jemal A, Siegel R, Ward E, Hao Y, Xu J, Murray T, Thun MJ. Cancer statistics, 2008. CA Cancer J Clin. 2008;58(2):71–96.

    Article  PubMed  Google Scholar 

  • Knezevich SR, Garnett MJ, Pysher TJ, Beckwith JB, Grundy PE, Sorensen PH. ETV6-NTRK3 gene fusions and trisomy 11 establish a histogenetic link between mesoblastic nephroma and congenital fibrosarcoma. Cancer Res. 1998;58(22):5046–8.

    CAS  PubMed  Google Scholar 

  • Kransdorf MJ. Malignant soft-tissue tumors in a large referral population: distribution of diagnoses by age, sex, and location. AJR Am J Roentgenol. 1995;164(1):129–34.

    Article  CAS  PubMed  Google Scholar 

  • Lewis JJ, Leung D, Heslin M, Woodruff JM, Brennan MF. Association of local recurrence with subsequent survival in extremity soft tissue sarcoma. J Clin Oncol. 1997;15(2):646–52.

    CAS  PubMed  Google Scholar 

  • Lewis JJ, Antonescu CR, Leung DH, Blumberg D, Healey JH, Woodruff JM, Brennan MF. Synovial sarcoma: a multivariate analysis of prognostic factors in 112 patients with primary localized tumors of the extremity. J Clin Oncol. 2000;18(10):2087–94.

    CAS  PubMed  Google Scholar 

  • McCarville MB, Spunt SL, Skapek SX, Pappo AS. Synovial sarcoma in pediatric patients. AJR Am J Roentgenol. 2002;179(3):797–801.

    Article  PubMed  Google Scholar 

  • McKee PH, Fletcher CD. Dermatofibrosarcoma protuberans presenting in infancy and childhood. J Cutan Pathol. 1991;18(4):241–6.

    Article  CAS  PubMed  Google Scholar 

  • McPhee M, McGrath BE, Zhang P, Driscoll D, Gibbs J, Peimer C. Soft tissue sarcoma of the hand. J Hand Surg Am. 1999;24(5):1001–7.

    Article  CAS  PubMed  Google Scholar 

  • Neville HL, Andrassy RJ, Lobe TE, Bagwell CE, Anderson JR, Womer RB, Crist WM, Wiener ES. Preoperative staging, prognostic factors, and outcome for extremity rhabdomyosarcoma: a preliminary report from the Intergroup Rhabdomyosarcoma Study IV (1991–1997). J Pediatr Surg. 2000;35(2):317–21.

    Article  CAS  PubMed  Google Scholar 

  • Ognjanovic S, Linabery AM, Charbonneau B, Ross JA. Trends in childhood rhabdomyosarcoma incidence and survival in the United States, 1975–2005. Cancer. 2009;115(18):4218–26.

    Article  PubMed Central  PubMed  Google Scholar 

  • Okcu MF, Munsell M, Treuner J, Mattke A, Pappo A, Cain A, Ferrari A, Casanova M, Ozkan A, Raney B. Synovial sarcoma of childhood and adolescence: a multicenter, multivariate analysis of outcome. J Clin Oncol. 2003;21(8):1602–11.

    Article  PubMed  Google Scholar 

  • Orbach D, Rey A, Cecchetto G, Oberlin O, Casanova M, Thebaud E, Scopinaro M, Bisogno G, Carli M, Ferrari A. Infantile fibrosarcoma: management based on the European experience. J Clin Oncol. 2010;28(2):318–23.

    Article  PubMed  Google Scholar 

  • Pappo AS, Fontanesi J, Luo X, Rao BN, Parham DM, Hurwitz C, Avery L, Pratt CB. Synovial sarcoma in children and adolescents: the St Jude Children’s Research Hospital experience. J Clin Oncol. 1994;12(11):2360–6.

    CAS  PubMed  Google Scholar 

  • Pisters PW, Harrison LB, Leung DH, Woodruff JM, Casper ES, Brennan MF. Long-term results of a prospective randomized trial of adjuvant brachytherapy in soft tissue sarcoma. J Clin Oncol. 1996;14(3):859–68.

    CAS  PubMed  Google Scholar 

  • Rabinowitz LG, Luchetti ME, Segura AD, Esterly NB. Acrally occurring dermatofibrosarcoma protuberans in children and adults. J Dermatol Surg Oncol. 1994;20(10):655–9.

    Article  CAS  PubMed  Google Scholar 

  • Rakheja D, Wilson KS, Meehan J, Schultz RA, Gomez AM. “Proximal-type” and classic epithelioid sarcomas represent a clinicopathologic continuum: case report. Pediatr Dev Pathol. 2005;8(1):105–14.

    Article  PubMed  Google Scholar 

  • Raney RB, Anderson JR, Barr FG, Donaldson SS, Pappo AS, Qualman SJ, Wiener ES, Maurer HM, Crist WM. Rhabdomyosarcoma and undifferentiated sarcoma in the first two decades of life: a selective review of intergroup rhabdomyosarcoma study group experience and rationale for Intergroup Rhabdomyosarcoma Study V. J Pediatr Hematol Oncol. 2001;23(4):215–20.

    Article  CAS  PubMed  Google Scholar 

  • Rikhof B, de Jong S, Suurmeijer AJ, Meijer C, van der Graaf WT. The insulin-like growth factor system and sarcomas. J Pathol. 2009;217(4):469–82.

    Article  CAS  PubMed  Google Scholar 

  • Rock MG, Wood MB, Fleegler EJ. Reconstruction for tumors of the upper limb. In: Fleegler C, Bogumill GP, editors. Tumors of the hand and upper extremity. Edinburgh: Churchill and Livingstone; 1993. p. 413–34.

    Google Scholar 

  • Rosenberg SA, Tepper J, Glatstein E, Costa J, Baker A, Brennan M, DeMoss EV, Seipp C, Sindelar WF, Sugarbaker P, Wesley R. The treatment of soft-tissue sarcomas of the extremities: prospective randomized evaluations of (1) limb-sparing surgery plus radiation therapy compared with amputation and (2) the role of adjuvant chemotherapy. Ann Surg. 1982;196(3):305–15.

    Article  CAS  PubMed Central  PubMed  Google Scholar 

  • Sandberg AA, Bridge JA. Updates on the cytogenetics and molecular genetics of bone and soft tissue tumors. Synovial sarcoma. Cancer Genet Cytogenet. 2002;133(1):1–23.

    Article  CAS  PubMed  Google Scholar 

  • Siegel R, Naishadham D, Jemal A. Cancer statistics, 2013. CA Cancer J Clin. 2013;63(1):11–30.

    Article  PubMed  Google Scholar 

  • Sorensen PH, Lynch JC, Qualman SJ, Tirabosco R, Lim JF, Maurer HM, Bridge JA, Crist WM, Triche TJ, Barr FG. PAX3-FKHR and PAX7-FKHR gene fusions are prognostic indicators in alveolar rhabdomyosarcoma: a report from the children’s oncology group. J Clin Oncol. 2002;20(11):2672–9.

    Article  CAS  PubMed  Google Scholar 

  • Stanelle EJ, Christison-Lagay ER, Healey JH, Singer S, Meyers PA, La Quaglia MP. Pediatric and adolescent synovial sarcoma: multivariate analysis of prognostic factors and survival outcomes. Ann Surg Oncol. 2013;20(1):73–9.

    Article  PubMed  Google Scholar 

  • Stasik CJ, Tawfik O. Malignant peripheral nerve sheath tumor with rhabdomyosarcomatous differentiation (malignant triton tumor). Arch Pathol Lab Med. 2006;130(12):1878–81.

    PubMed  Google Scholar 

  • Toomayan GA, Robertson F, Major NM, Brigman BE. Upper extremity compartmental anatomy: clinical relevance to radiologists. Skeletal Radiol. 2006;35(4):195–201.

    Article  PubMed  Google Scholar 

  • Tucker T, Wolkenstein P, Revuz J, Zeller J, Friedman JM. Association between benign and malignant peripheral nerve sheath tumors in NF1. Neurology. 2005;65(2):205–11.

    Article  CAS  PubMed  Google Scholar 

  • Williard WC, Hajdu SI, Casper ES, Brennan MF. Comparison of amputation with limb-sparing operations for adult soft tissue sarcoma of the extremity. Ann Surg. 1992;215(3):269–75.

    Article  CAS  PubMed Central  PubMed  Google Scholar 

  • Wirman JA, Sherman S, Sullivan MR. Dermatofibrosarcoma protuberans arising on the hand. Hand. 1981;13(2):187–91.

    Article  CAS  PubMed  Google Scholar 

  • Yang JC, Chang AE, Baker AR, Sindelar WF, Danforth DN, Topalian SL, DeLaney T, Glatstein E, Steinberg SM, Merino MJ, Rosenberg SA. Randomized prospective study of the benefit of adjuvant radiation therapy in the treatment of soft tissue sarcomas of the extremity. J Clin Oncol. 1998;16(1):197–203.

    CAS  PubMed  Google Scholar 

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Authors and Affiliations

  1. Orthopaedic Oncology Service, Montefiore Medical Center, Albert Einstein College of Medicine, Bronx, NY, 10467, USA

    David S. Geller

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  1. David S. Geller
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Correspondence to David S. Geller .

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Editors and Affiliations

  1. Dept. of Orthopedics, University of Maryland School of Medicine, Baltimore, Maryland, USA

    Joshua M. Abzug

  2. Shriners Hospitals for Children, Philadelphia, Pennsylvania, USA

    Scott H. Kozin

  3. Shriners Hospitals for Children, Philadelphia, Pennsylvania, USA

    Dan A. Zlotolow

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Geller, D.S. (2015). Malignant Soft Tissue Lesions. In: Abzug, J., Kozin, S., Zlotolow, D. (eds) The Pediatric Upper Extremity. Springer, New York, NY. https://doi.org/10.1007/978-1-4614-8515-5_86

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  • DOI: https://doi.org/10.1007/978-1-4614-8515-5_86

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