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Childhood Soft Tissue Sarcoma Treatment (PDQ®)
Patient Version   Health Professional Version   En español   Last Modified: 12/05/2008



Purpose of This PDQ Summary






General Information






Cellular and Histopathologic Classification






Stage Information






Treatment Option Overview






Nonmetastatic Childhood Soft Tissue Sarcoma






Metastatic Childhood Soft Tissue Sarcoma






Recurrent/Progressive Childhood Soft Tissue Sarcoma






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Nonmetastatic Childhood Soft Tissue Sarcoma

Treatment Options by Soft Tissue Sarcoma Type
Current Clinical Trials



Treatment Options by Soft Tissue Sarcoma Type

For nonmetastatic pediatric nonrhabdomyosarcomatous soft tissue sarcomas (NRSTSs), treatment with surgery alone is often curative.[1-6] If the initial surgery was performed without suspicion of malignancy, re-excision by a surgeon experienced in the treatment of soft tissue sarcoma is essential, even if imaging studies do not suggest the presence of residual tumor. Postoperatively, tumor-free margins must be confirmed through pathologic evaluation, and re-excision must be performed if the margins are positive. If further resection is not feasible, postoperative radiation therapy, or if possible, brachytherapy should be used.[7,8] For patients with local recurrence, re-excision of the mass is indicated.

  1. Tumors with low potential for metastasis:

    Fibrosarcomas and hemangiopericytomas are tumors with low potential for metastasis in infants and young children; desmoid tumors, aggressive fibromatosis, dermatofibrosarcoma, and angiomatoid malignant fibrous histiocytomas typically are also clinically less aggressive, rarely metastasize, and can often be treated successfully with surgery alone.[1,9-11] In children with infantile fibrosarcoma, preoperative chemotherapy has made possible a more conservative surgical approach; agents active in this setting include vincristine, dactinomycin, cyclophosphamide, and ifosfamide.[1,12] Responses to presurgical chemotherapy with similar agents have been reported in cases of infantile hemangiopericytoma.[1]

    Desmoid tumors are well-differentiated fibrous lesions that rarely metastasize, but they have a significant potential for local invasiveness and recurrence. The treatment of choice is resection to achieve clear margins. If postoperative margins are positive, 70% of patients will have a recurrence of disease. When complete surgical excision is not feasible and the tumor poses significant potential for mortality or morbidity, preoperative strategies that include external-beam radiation therapy, postoperative interstitial iridium I 192, nonsteroidal anti-inflammatory agents, antiestrogens, vinblastine, and methotrexate should be considered.[13,14] Evaluation of the benefit of chemotherapy for treatment of desmoid tumors has been extremely difficult because desmoid tumors have a highly variable natural history. Large adult series and a single pediatric series have reported long periods of disease stabilization and even regression without systemic therapy.[15,16] A small series of mainly adult patients (n = 19) with desmoid tumors were treated with imatinib mesylate and showed infrequent objective responses.[17] A series of mainly adult familial adenomatous polyposis patients with unresectable desmoid tumors that were unresponsive to hormone therapy, showed that doxorubicin plus dacarbazine followed by meloxicam (a nonsteroidal anti-inflammatory agent) can be safely administered and can induce responses.[18] There are reports of objective responses to systemic chemotherapy in children with desmoid tumors. Combination chemotherapy using vinblastine and methotrexate has been used for the treatment of progressive desmoid tumor in children.[13] These should be interpreted cautiously in light of the variable natural history of the disease. Partially excised or recurrent lesions that do not pose a significant danger to vital organs may be monitored closely if other treatment alternatives are not available.[16,19-22] Whenever possible, however, the treatment of choice is complete resection.

    Treatment option under clinical evaluation:

    The following treatment option is currently under investigation in national and/or institutional clinical trials. For more information about clinical trials, please see the NCI Web site.

    • Imatinib mesylate used as a treatment in recurrent soft tissue sarcomas, bone sarcomas, and primary desmoid tumors.


  2. The pediatric neoplasms listed below exhibit similar biologic behavior to those lesions in adults, and a discussion of their treatment follows.
    • Fibrosarcoma in older children and adolescents.
    • Malignant peripheral nerve sheath tumor (MPNST).
    • Liposarcoma.
    • Synovial sarcoma.
    • Hemangiopericytoma in older children and young adults.
    • Extraosseous osteosarcoma.
    • Extraosseous chondrosarcoma.
    • Malignant fibrous histiocytoma.
    • Leiomyosarcoma.
    • Epithelioid sarcoma.

    Standard treatment options:

    Every attempt should be made to resect the primary tumor locally with negative margins.[23,24] If the original operation failed to achieve pathologically negative tissue margins, a second surgery may be indicated.[2] Although combined surgery and radiation therapy have dramatically improved outcome in adults and children with soft tissue sarcomas over the past 20 years,[7] the morbidity of high-dose radiation therapy should be considered in infants and young children with these tumors.[25] The use of brachytherapy and intraoperative radiation therapy is under study.[8,26] Preoperative radiation therapy has been associated with excellent local control rates in adults;[27,28] this approach has not been used extensively in pediatric patients.

    The role of adjuvant (postoperative) chemotherapy remains controversial. Virtually all trials of adjuvant chemotherapy in adults with soft tissue sarcoma report the results of treatment for all patients in aggregate. This may obscure important differences in chemosensitivity among histologic subtypes of soft tissue sarcoma. A retrospective analysis of neoadjuvant chemotherapy in adults with soft tissue sarcoma suggested a benefit for patients with larger tumors.[29] The largest prospective pediatric trial failed to document any benefit of adjuvant chemotherapy with vincristine, dactinomycin, cyclophosphamide, and doxorubicin in children with grossly resected tumors.[30] This trial also reported results in aggregate for a variety of soft tissue sarcomas. In patients with unresectable or metastatic disease treated with vincristine, dactinomycin, and cyclophosphamide, the overall survival (OS) and disease-free survival rates were 31% and 10%, respectively.[31] Achieving complete responses after aggressive chemotherapy, radiation therapy, and surgery is possible in most patients with more advanced NRSTS.[32]

    Chemotherapy for extraosseous osteosarcoma has not been well studied. Treatment has previously been recommended to follow soft tissue sarcoma guidelines rather than guidelines for osteosarcoma of bone.[33] Extraosseous osteosarcoma may be more chemosensitive in young patients than in adults.[33] A retrospective analysis of the German Cooperative Osteosarcoma Study identified a favorable outcome for extraskeletal osteosarcoma treated with surgery and conventional osteosarcoma chemotherapy.[34] (Refer to the PDQ summary on Osteosarcoma/Malignant Fibrous Histiocytoma of Bone for more information.)

    Synovial sarcoma appears to be more sensitive to chemotherapy than many other NRSTSs. Children with synovial sarcoma have a higher probability for both event-free survival (EFS) and OS than children with other types of NRSTS.[35,36] A German randomized trial suggested a benefit for adjuvant chemotherapy in children with synovial sarcoma.[37] A meta-analysis also suggested that chemotherapy may improve EFS but could not confirm improvement in OS.[24] Many treatment centers advocate adjuvant chemotherapy following resection of synovial sarcoma in children and young adults; unequivocal proof of the value of this strategy from prospective, randomized clinical trials is lacking. A study of 21 patients with small (<1 cm), localized synovial sarcomas showed an excellent survival rate with no metastatic events; only one patient received chemotherapy.[38] A retrospective analysis of synovial sarcoma in children treated in Germany and Italy identified tumor size (>5 cm or <5 cm in greatest dimension) as an important predictor of EFS.[39] In this analysis, local invasiveness conferred an inferior probability of EFS, but surgical margins did not predict outcome.

    A large retrospective analysis of the German and Italian experience with MPNST identified incomplete resection, large tumor size, tumor invasiveness, nonextremity primary site, and clinical diagnosis of neurofibromatosis as unfavorable prognostic findings.[23] There was a trend toward improved outcome with adjuvant radiation therapy. While 65% of measurable tumors had objective responses to ifosfamide-containing chemotherapy regimens, the analysis did not conclusively demonstrate improved survival for chemotherapy.[23] A series of 37 young patients with MPNST and neurofibromatosis type-1 (NF-1) showed that most patients had large invasive tumors, poorly responsive to chemotherapy; progression-free survival was 19% and overall 5 year survival was 28%.[40] Another series of older patients with MPNST found that those with NF-1 had a worse prognosis than those without NF-1.[41]



  3. Alveolar soft part sarcoma (ASPS) is a tumor of uncertain histogenesis characterized by an x;17 translocation.[42] Pediatric ASPS seems to have a better outcome than its adult counterpart.[43] In a series of 19 treated patients, one group reported a 5-year OS rate of 80%, a 91% OS rate for patients with localized disease, a 100% OS rate for patients with tumors 5 cm or smaller, and a 31% OS rate for patients with tumors larger than 5 cm.[6] A subset of renal tumors found in young people was previously considered to be renal cell carcinoma, but the subset now appears to be genetically related to ASPS.[44]

    Standard treatment options:

    The standard approach is complete resection of the primary lesion.[6] If complete excision is not feasible, radiation therapy should be administered. The value of adjuvant chemotherapy in completely resected ASPS remains unproven, particularly because patients with unresected or metastatic tumors failed to respond to chemotherapeutic agents frequently used to treat soft tissue sarcomas.[45] Patients with ASPS may relapse several years after a prolonged period of apparent remission.[46]

    Treatment options under clinical evaluation:

    • COG ARST0332 is a prospective study for children and young adults with soft tissue sarcomas other than rhabdomyosarcoma. Patients are stratified by tumor grade and extent. Patients with lower grade tumors and patients with small, completely resected high-grade tumors are observed after surgical resection alone. Patients with positive microscopic margins receive adjuvant radiation. Patients with high-grade tumors larger than 5 cm undergo resection and receive adjuvant chemotherapy and radiation. Patients with unresectable or metastatic disease receive neoadjuvant chemotherapy. Chemotherapy for all eligible patients is ifosfamide and doxorubicin.[47]


    • The role of adjuvant chemotherapy in children with this malignancy has not been tested. Because these tumors are rare, all children with ASPS should be enrolled in prospective clinical trials.




  4. Desmoplastic small round cell tumor is a primitive sarcoma that most frequently involves the abdomen, pelvis, or tissues around the testes.[48-50] The tumor occurs mainly in males and invades locally but may spread to the lungs and elsewhere. Cytogenetic studies of these tumors have demonstrated the recurrent translocation t(11;22)(p13;q12), which has been characterized as a fusion of the WT1 and EWS genes.[51]

    Standard treatment options:

    Complete resection of this tumor is rarely possible, thus effective treatment must rely on chemotherapy and radiation therapy. Treatment for individuals with desmoplastic small round cell tumor following surgery requires aggressive chemotherapy with the agents that are used for the treatment of sarcoma combined with appropriate radiation treatment. Prognosis is dependent on the extent and aggressiveness of the tumor and its treatment.[48,49,52] Whole abdominopelvic radiation therapy is feasible but has not significantly improved the outcome for this diagnosis.[53,54]

    Treatment options under clinical evaluation:

    • None at present.


  5. Clear cell sarcoma (malignant melanoma) of soft parts (also called clear cell sarcoma of tendons and aponeuroses) is somewhat similar to cutaneous malignant melanoma but is cytogenetically distinct; most cases have a t(12;22)(q13;q12) translocation that has not been reported in melanoma.[55] Patients who have small, localized tumors with low mitotic rate and intermediate histologic grade fare best.[56] Treatment is primarily surgical with radiation therapy for uncertain or involved margins. Antisarcoma chemotherapy is rarely effective.[57]



  6. Hemangioendotheliomas are tumors found in infants that arise within the liver or elsewhere and usually remain benign.[58] The tumors are sometimes associated with consumptive coagulopathy, also known as the Kasabach-Merritt syndrome (or phenomenon).[59-61] In older children and adults, hemangioendotheliomas may occur elsewhere in the body and can metastasize to lungs, lymph nodes, bones, and within the pleural or peritoneal cavities. The preferred pathologic designation for these lesions in older persons is epithelioid hemangioendothelioma, which connotes the possibility of distant spread. These latter lesions are considered of intermediate malignant potential, between benign hemangioma and angiosarcoma.[62,63] Treatment of the asymptomatic liver hemangioendothelioma of a child younger than 1 year may consist of close observation, because some tumors will regress. Symptomatic lesions require urgent medical or surgical management, especially if coagulopathy is present.[58-61] Epithelioid hemangioendothelioma of the liver should be managed surgically; some patients may need orthotopic liver transplantation because this disease does not respond to radiation therapy or chemotherapy.[62]



  7. Vascular tumors vary from hemangiomas, which are considered always benign, to angiosarcomas, which are highly malignant.[64] Complete surgical excision appears to be crucial for angiosarcomas and lymphangiosarcomas despite evidence of tumor shrinkage in some patients in response to local therapy.[65-67]



Current Clinical Trials

Check for U.S. clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with nonmetastatic childhood soft tissue sarcoma. The list of clinical trials can be further narrowed by location, drug, intervention, and other criteria.

General information about clinical trials is also available from the NCI Web site.

References

  1. Miser JS, Pappo AS, Triche TJ: Other soft tissue sarcomas of childhood. In: Pizzo PA, Poplack DG, eds.: Principles and Practice of Pediatric Oncology. 4th ed. Philadelphia, Pa: Lippincott, Williams and Wilkins, 2002, pp 1017-1050. 

  2. Rao BN: Nonrhabdomyosarcoma in children: prognostic factors influencing survival. Semin Surg Oncol 9 (6): 524-31, 1993 Nov-Dec.  [PUBMED Abstract]

  3. Dillon PW, Whalen TV, Azizkhan RG, et al.: Neonatal soft tissue sarcomas: the influence of pathology on treatment and survival. Children's Cancer Group Surgical Committee. J Pediatr Surg 30 (7): 1038-41, 1995.  [PUBMED Abstract]

  4. deCou JM, Rao BN, Parham DM, et al.: Malignant peripheral nerve sheath tumors: the St. Jude Children's Research Hospital experience. Ann Surg Oncol 2 (6): 524-9, 1995.  [PUBMED Abstract]

  5. Pappo AS, Fontanesi J, Luo X, et al.: Synovial sarcoma in children and adolescents: the St Jude Children's Research Hospital experience. J Clin Oncol 12 (11): 2360-6, 1994.  [PUBMED Abstract]

  6. Casanova M, Ferrari A, Bisogno G, et al.: Alveolar soft part sarcoma in children and adolescents: A report from the Soft-Tissue Sarcoma Italian Cooperative Group. Ann Oncol 11 (11): 1445-9, 2000.  [PUBMED Abstract]

  7. Marcus KC, Grier HE, Shamberger RC, et al.: Childhood soft tissue sarcoma: a 20-year experience. J Pediatr 131 (4): 603-7, 1997.  [PUBMED Abstract]

  8. Merchant TE, Parsh N, del Valle PL, et al.: Brachytherapy for pediatric soft-tissue sarcoma. Int J Radiat Oncol Biol Phys 46 (2): 427-32, 2000.  [PUBMED Abstract]

  9. Ferrari A, Casanova M, Bisogno G, et al.: Hemangiopericytoma in pediatric ages: a report from the Italian and German Soft Tissue Sarcoma Cooperative Group. Cancer 92 (10): 2692-8, 2001.  [PUBMED Abstract]

  10. Cecchetto G, Carli M, Alaggio R, et al.: Fibrosarcoma in pediatric patients: results of the Italian Cooperative Group studies (1979-1995). J Surg Oncol 78 (4): 225-31, 2001.  [PUBMED Abstract]

  11. Buitendijk S, van de Ven CP, Dumans TG, et al.: Pediatric aggressive fibromatosis: a retrospective analysis of 13 patients and review of literature. Cancer 104 (5): 1090-9, 2005.  [PUBMED Abstract]

  12. 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 24 (9): 722-6, 2002.  [PUBMED Abstract]

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  14. Gandhi MM, Nathan PC, Weitzman S, et al.: Successful treatment of life-threatening generalized infantile myofibromatosis using low-dose chemotherapy. J Pediatr Hematol Oncol 25 (9): 750-4, 2003.  [PUBMED Abstract]

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  16. Faulkner LB, Hajdu SI, Kher U, et al.: Pediatric desmoid tumor: retrospective analysis of 63 cases. J Clin Oncol 13 (11): 2813-8, 1995.  [PUBMED Abstract]

  17. Heinrich MC, McArthur GA, Demetri GD, et al.: Clinical and molecular studies of the effect of imatinib on advanced aggressive fibromatosis (desmoid tumor). J Clin Oncol 24 (7): 1195-203, 2006.  [PUBMED Abstract]

  18. Gega M, Yanagi H, Yoshikawa R, et al.: Successful chemotherapeutic modality of doxorubicin plus dacarbazine for the treatment of desmoid tumors in association with familial adenomatous polyposis. J Clin Oncol 24 (1): 102-5, 2006.  [PUBMED Abstract]

  19. Merchant TE, Nguyen D, Walter AW, et al.: Long-term results with radiation therapy for pediatric desmoid tumors. Int J Radiat Oncol Biol Phys 47 (5): 1267-71, 2000.  [PUBMED Abstract]

  20. Zelefsky MJ, Harrison LB, Shiu MH, et al.: Combined surgical resection and iridium 192 implantation for locally advanced and recurrent desmoid tumors. Cancer 67 (2): 380-4, 1991.  [PUBMED Abstract]

  21. Weiss AJ, Lackman RD: Low-dose chemotherapy of desmoid tumors. Cancer 64 (6): 1192-4, 1989.  [PUBMED Abstract]

  22. Klein WA, Miller HH, Anderson M, et al.: The use of indomethacin, sulindac, and tamoxifen for the treatment of desmoid tumors associated with familial polyposis. Cancer 60 (12): 2863-8, 1987.  [PUBMED Abstract]

  23. Carli M, Ferrari A, Mattke A, et al.: Pediatric malignant peripheral nerve sheath tumor: the Italian and German soft tissue sarcoma cooperative group. J Clin Oncol 23 (33): 8422-30, 2005.  [PUBMED Abstract]

  24. Okcu MF, Munsell M, Treuner J, et al.: Synovial sarcoma of childhood and adolescence: a multicenter, multivariate analysis of outcome. J Clin Oncol 21 (8): 1602-11, 2003.  [PUBMED Abstract]

  25. Suit H, Spiro I: Radiation as a therapeutic modality in sarcomas of the soft tissue. Hematol Oncol Clin North Am 9 (4): 733-46, 1995.  [PUBMED Abstract]

  26. Schomberg PJ, Gunderson LL, Moir CR, et al.: Intraoperative electron irradiation in the management of pediatric malignancies. Cancer 79 (11): 2251-6, 1997.  [PUBMED Abstract]

  27. Sadoski C, Suit HD, Rosenberg A, et al.: Preoperative radiation, surgical margins, and local control of extremity sarcomas of soft tissues. J Surg Oncol 52 (4): 223-30, 1993.  [PUBMED Abstract]

  28. Cannon CP, Ballo MT, Zagars GK, et al.: Complications of combined modality treatment of primary lower extremity soft-tissue sarcomas. Cancer 107 (10): 2455-61, 2006.  [PUBMED Abstract]

  29. Grobmyer SR, Maki RG, Demetri GD, et al.: Neo-adjuvant chemotherapy for primary high-grade extremity soft tissue sarcoma. Ann Oncol 15 (11): 1667-72, 2004.  [PUBMED Abstract]

  30. Pratt CB, Pappo AS, Gieser P, et al.: Role of adjuvant chemotherapy in the treatment of surgically resected pediatric nonrhabdomyosarcomatous soft tissue sarcomas: A Pediatric Oncology Group Study. J Clin Oncol 17 (4): 1219, 1999.  [PUBMED Abstract]

  31. Pratt CB, Maurer HM, Gieser P, et al.: Treatment of unresectable or metastatic pediatric soft tissue sarcomas with surgery, irradiation, and chemotherapy: a Pediatric Oncology Group study. Med Pediatr Oncol 30 (4): 201-9, 1998.  [PUBMED Abstract]

  32. Nathan PC, Tsokos M, Long L, et al.: Adjuvant chemotherapy for the treatment of advanced pediatric nonrhabdomyosarcoma soft tissue sarcoma: the National Cancer Institute experience. Pediatr Blood Cancer 44 (5): 449-54, 2005.  [PUBMED Abstract]

  33. Wodowski K, Hill DA, Pappo AS, et al.: A chemosensitive pediatric extraosseous osteosarcoma: case report and review of the literature. J Pediatr Hematol Oncol 25 (1): 73-7, 2003.  [PUBMED Abstract]

  34. Goldstein-Jackson SY, Gosheger G, Delling G, et al.: Extraskeletal osteosarcoma has a favourable prognosis when treated like conventional osteosarcoma. J Cancer Res Clin Oncol 131 (8): 520-6, 2005.  [PUBMED Abstract]

  35. McGrory JE, Pritchard DJ, Arndt CA, et al.: Nonrhabdomyosarcoma soft tissue sarcomas in children. The Mayo Clinic experience. Clin Orthop (374): 247-58, 2000.  [PUBMED Abstract]

  36. Ferrari A, Gronchi A, Casanova M, et al.: Synovial sarcoma: a retrospective analysis of 271 patients of all ages treated at a single institution. Cancer 101 (3): 627-34, 2004.  [PUBMED Abstract]

  37. Ladenstein R, Treuner J, Koscielniak E, et al.: Synovial sarcoma of childhood and adolescence. Report of the German CWS-81 study. Cancer 71 (11): 3647-55, 1993.  [PUBMED Abstract]

  38. Michal M, Fanburg-Smith JC, Lasota J, et al.: Minute synovial sarcomas of the hands and feet: a clinicopathologic study of 21 tumors less than 1 cm. Am J Surg Pathol 30 (6): 721-6, 2006.  [PUBMED Abstract]

  39. 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. Pediatr Blood Cancer 46 (1): 11-7, 2006.  [PUBMED Abstract]

  40. Ferrari A, Bisogno G, Macaluso A, et al.: Soft-tissue sarcomas in children and adolescents with neurofibromatosis type 1. Cancer 109 (7): 1406-12, 2007.  [PUBMED Abstract]

  41. Hagel C, Zils U, Peiper M, et al.: Histopathology and clinical outcome of NF1-associated vs. sporadic malignant peripheral nerve sheath tumors. J Neurooncol 82 (2): 187-92, 2007.  [PUBMED Abstract]

  42. Ladanyi M, Lui MY, Antonescu CR, et al.: The der(17)t(X;17)(p11;q25) of human alveolar soft part sarcoma fuses the TFE3 transcription factor gene to ASPL, a novel gene at 17q25. Oncogene 20 (1): 48-57, 2001.  [PUBMED Abstract]

  43. Fanburg-Smith JC, Miettinen M, Folpe AL, et al.: Lingual alveolar soft part sarcoma; 14 cases: novel clinical and morphological observations. Histopathology 45 (5): 526-37, 2004.  [PUBMED Abstract]

  44. Argani P, Antonescu CR, Illei PB, et al.: Primary renal neoplasms with the ASPL-TFE3 gene fusion of alveolar soft part sarcoma: a distinctive tumor entity previously included among renal cell carcinomas of children and adolescents. Am J Pathol 159 (1): 179-92, 2001.  [PUBMED Abstract]

  45. Ogose A, Yazawa Y, Ueda T, et al.: Alveolar soft part sarcoma in Japan: multi-institutional study of 57 patients from the Japanese Musculoskeletal Oncology Group. Oncology 65 (1): 7-13, 2003.  [PUBMED Abstract]

  46. Lieberman PH, Brennan MF, Kimmel M, et al.: Alveolar soft-part sarcoma. A clinico-pathologic study of half a century. Cancer 63 (1): 1-13, 1989.  [PUBMED Abstract]

  47. Pappo AS, Devidas M, Jenkins J, et al.: Phase II trial of neoadjuvant vincristine, ifosfamide, and doxorubicin with granulocyte colony-stimulating factor support in children and adolescents with advanced-stage nonrhabdomyosarcomatous soft tissue sarcomas: a Pediatric Oncology Group Study. J Clin Oncol 23 (18): 4031-8, 2005.  [PUBMED Abstract]

  48. Leuschner I, Radig K, Harms D: Desmoplastic small round cell tumor. Semin Diagn Pathol 13 (3): 204-12, 1996.  [PUBMED Abstract]

  49. Kushner BH, LaQuaglia MP, Wollner N, et al.: Desmoplastic small round-cell tumor: prolonged progression-free survival with aggressive multimodality therapy. J Clin Oncol 14 (5): 1526-31, 1996.  [PUBMED Abstract]

  50. Saab R, Khoury JD, Krasin M, et al.: Desmoplastic small round cell tumor in childhood: the St. Jude Children's Research Hospital experience. Pediatr Blood Cancer 49 (3): 274-9, 2007.  [PUBMED Abstract]

  51. Gerald WL, Ladanyi M, de Alava E, et al.: Clinical, pathologic, and molecular spectrum of tumors associated with t(11;22)(p13;q12): desmoplastic small round-cell tumor and its variants. J Clin Oncol 16 (9): 3028-36, 1998.  [PUBMED Abstract]

  52. Schwarz RE, Gerald WL, Kushner BH, et al.: Desmoplastic small round cell tumors: prognostic indicators and results of surgical management. Ann Surg Oncol 5 (5): 416-22, 1998 Jul-Aug.  [PUBMED Abstract]

  53. Goodman KA, Wolden SL, La Quaglia MP, et al.: Whole abdominopelvic radiotherapy for desmoplastic small round-cell tumor. Int J Radiat Oncol Biol Phys 54 (1): 170-6, 2002.  [PUBMED Abstract]

  54. Lal DR, Su WT, Wolden SL, et al.: Results of multimodal treatment for desmoplastic small round cell tumors. J Pediatr Surg 40 (1): 251-5, 2005.  [PUBMED Abstract]

  55. Speleman F, Delattre O, Peter M, et al.: Malignant melanoma of the soft parts (clear-cell sarcoma): confirmation of EWS and ATF-1 gene fusion caused by a t(12;22) translocation. Mod Pathol 10 (5): 496-9, 1997.  [PUBMED Abstract]

  56. Coindre JM, Hostein I, Terrier P, et al.: Diagnosis of clear cell sarcoma by real-time reverse transcriptase-polymerase chain reaction analysis of paraffin embedded tissues: clinicopathologic and molecular analysis of 44 patients from the French sarcoma group. Cancer 107 (5): 1055-64, 2006.  [PUBMED Abstract]

  57. Ferrari A, Casanova M, Bisogno G, et al.: Clear cell sarcoma of tendons and aponeuroses in pediatric patients: a report from the Italian and German Soft Tissue Sarcoma Cooperative Group. Cancer 94 (12): 3269-76, 2002.  [PUBMED Abstract]

  58. Daller JA, Bueno J, Gutierrez J, et al.: Hepatic hemangioendothelioma: clinical experience and management strategy. J Pediatr Surg 34 (1): 98-105; discussion 105-6, 1999.  [PUBMED Abstract]

  59. Lyons LL, North PE, Mac-Moune Lai F, et al.: Kaposiform hemangioendothelioma: a study of 33 cases emphasizing its pathologic, immunophenotypic, and biologic uniqueness from juvenile hemangioma. Am J Surg Pathol 28 (5): 559-68, 2004.  [PUBMED Abstract]

  60. Hu B, Lachman R, Phillips J, et al.: Kasabach-Merritt syndrome-associated kaposiform hemangioendothelioma successfully treated with cyclophosphamide, vincristine, and actinomycin D. J Pediatr Hematol Oncol 20 (6): 567-9, 1998 Nov-Dec.  [PUBMED Abstract]

  61. Deb G, Jenkner A, De Sio L, et al.: Spindle cell (Kaposiform) hemangioendothelioma with Kasabach-Merritt syndrome in an infant: successful treatment with alpha-2A interferon. Med Pediatr Oncol 28 (5): 358-61, 1997.  [PUBMED Abstract]

  62. Makhlouf HR, Ishak KG, Goodman ZD: Epithelioid hemangioendothelioma of the liver: a clinicopathologic study of 137 cases. Cancer 85 (3): 562-82, 1999.  [PUBMED Abstract]

  63. Pinet C, Magnan A, Garbe L, et al.: Aggressive form of pleural epithelioid haemangioendothelioma: complete response after chemotherapy. Eur Respir J 14 (1): 237-8, 1999.  [PUBMED Abstract]

  64. Coffin CM, Dehner LP: Vascular tumors in children and adolescents: a clinicopathologic study of 228 tumors in 222 patients. Pathol Annu 28 Pt 1: 97-120, 1993.  [PUBMED Abstract]

  65. Lezama-del Valle P, Gerald WL, Tsai J, et al.: Malignant vascular tumors in young patients. Cancer 83 (8): 1634-9, 1998.  [PUBMED Abstract]

  66. Fata F, O'Reilly E, Ilson D, et al.: Paclitaxel in the treatment of patients with angiosarcoma of the scalp or face. Cancer 86 (10): 2034-7, 1999.  [PUBMED Abstract]

  67. Ferrari A, Casanova M, Bisogno G, et al.: Malignant vascular tumors in children and adolescents: a report from the Italian and German Soft Tissue Sarcoma Cooperative Group. Med Pediatr Oncol 39 (2): 109-14, 2002.  [PUBMED Abstract]

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