General Information
The National Cancer Institute (NCI) provides the PDQ pediatric cancer treatment information summaries as a public service to increase the availability of evidence-based cancer information to health professionals, patients, and the public.
Cancer in children and adolescents is rare. Children and adolescents with
cancer should be referred to medical centers that have a multidisciplinary team
of cancer specialists with experience treating the cancers that occur during
childhood and adolescence. This multidisciplinary team approach incorporates the skills
of the primary care physician, pediatric surgical subspecialists, radiation
oncologist, pediatric hematologist/oncologist, rehabilitation specialist,
pediatric nurse specialists, social workers, and others to ensure that
children receive treatment, supportive care, and rehabilitation that will
achieve optimal survival and quality of life. Refer to the PDQ Supportive Care summaries for specific information about supportive care for children and adolescents with cancer.
Guidelines for pediatric cancer
centers and their role in the treatment of pediatric patients with cancer have
been outlined by the American Academy of Pediatrics.[1] At these pediatric
cancer centers, clinical trials are available for most types of cancer
that occur in children and adolescents, and the opportunity to participate in
these trials is offered to most patients/families. Clinical trials for
children and adolescents with cancer are generally designed to compare
potentially better therapy with therapy that is currently accepted as standard.
Most of the progress made in identifying curative therapies for
childhood cancers has been achieved through clinical trials. Information about
ongoing clinical trials is available from the NCI Web site.
In recent decades, dramatic improvements in survival have been achieved for children and adolescents with cancer. Childhood and adolescent cancer survivors require close follow-up because cancer therapy side effects may persist or develop months or years after treatment. Refer to the PDQ summary on Late Effects of Treatment for Childhood Cancer for specific information about the incidence, type, and monitoring of late effects in childhood and adolescent cancer survivors.
Pediatric soft tissue sarcomas are a group of malignant tumors that originate
from primitive mesenchymal tissue and account for 7% of all childhood
tumors.[2] Rhabdomyosarcomas, tumors of striated muscle, and undifferentiated
sarcomas account for more than half of all cases of soft tissue sarcomas in
children. (Refer to the PDQ summary on Childhood Rhabdomyosarcoma Treatment for more
information.) The remaining nonrhabdomyosarcomatous soft tissue sarcomas
(NRSTSs) account for approximately 3% of all childhood tumors.[3] This heterogeneous
group of tumors includes neoplasms of smooth muscle (leiomyosarcoma),
connective tissue (fibrous and adipose), vascular tissue (blood and lymphatic
vessels), and the peripheral nervous system.[4] Synovial sarcomas,
fibrosarcomas, and malignant peripheral nerve sheath tumors predominate in pediatric patients.[5-9]
NRSTSs are more common in adults [4] than
in children; therefore, much of the information regarding the treatment and
natural history of children with these lesions has been on the basis of
findings from adult studies. Pediatric NRSTSs, however, are often associated with a better outcome. This
difference is most pronounced for infants and children younger than 4 years
with fibrosarcoma that is locally aggressive but not metastatic. These patients have an excellent prognosis when chemosensitive and treated with surgery
only.[3,4,10,11] Soft tissue sarcomas in older children and adolescents often
behave similarly to those in adult patients.[3,4]
Although they can develop in any part of the body, NRSTSs arise most commonly in the trunk and extremities.[5,6,12] These
neoplasms can present initially as an asymptomatic solid mass, or they may be
symptomatic because of local invasion of adjacent anatomical structures.
Systemic symptoms (e.g., fever, weight loss, and night sweats) are rare.
Hypoglycemia and hypophosphatemic rickets have been reported in cases of
hemangiopericytoma, whereas hyperglycemia has been noted in patients with
fibrosarcoma of the lung.[4]
Genetic and environmental factors influence the development of
NRSTS. Heritable cancer-associated
changes of the p53 tumor suppressor gene can occur in families with Li-Fraumeni
syndrome.[13] Members of these families have an increased risk of developing
soft tissue tumors, bone sarcomas, breast cancer, brain tumors, and acute
leukemia.[3] Approximately 4% of patients with neurofibromatosis type 1
develop malignant peripheral nerve sheath tumors, which usually develop after a
long latency; some patients develop multiple lesions.[4,14,15] Some
NRSTSs (particularly malignant fibrous
histiocytoma) can develop within a previously irradiated site; others (e.g.,
leiomyosarcoma) have been linked to Epstein-Barr virus infection in patients
with AIDS.[3,4,16]
Synovial sarcomas are the most common NRSTS reported in children. The most common location is the lower extremity
followed by upper extremity, trunk, abdomen, and head and neck. Approximately
30% of patients with synovial sarcoma are younger than 20 years. The
most common site of metastasis is the lung.[17] Factors such as International Union Against Cancer/American Joint Committee on Cancer stage III/stage IVA, tumor
necrosis, truncal locations, elevated mitotic rate, age, and histologic grade have been associated with
a worse prognosis in adults.[18-20]
(Refer to the PDQ summary on Childhood Rhabdomyosarcoma Treatment for more information.
Refer to the PDQ summary on Ewings Family of Tumors Treatment for more
information on extraosseous Ewing, peripheral neuroepithelioma, and Askin
tumor.)
The prognosis and biology of NRSTS tumors vary greatly depending on the age of
the patient, the primary site, tumor size, tumor invasiveness, histologic
grade, depth of invasion, and extent of disease at diagnosis. Because
long-term related morbidity must be minimized while disease-free survival is maximized, the ideal therapy for each patient must be
carefully and individually determined utilizing these prognostic factors before
initiating therapy for these patients.[6,10,17,21-23]
References
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Guidelines for the pediatric cancer center and role of such centers in diagnosis and treatment. American Academy of Pediatrics Section Statement Section on Hematology/Oncology. Pediatrics 99 (1): 139-41, 1997.
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Pappo AS, Pratt CB: Soft tissue sarcomas in children. Cancer Treat Res 91: 205-22, 1997.
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Miser JS, Triche TJ, Kinsella TJ, et al.: Other soft tissue sarcomas of childhood. In: Pizzo PA, Poplack DG, eds.: Principles and Practice of Pediatric Oncology. 3rd ed. Philadelphia, Pa: Lippincott-Raven, 1997, pp 865-888.
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Weiss SW, Goldblum JR: Enzinger and Weiss's Soft Tissue Tumors. 4th ed. St. Louis, Mo: Mosby, 2001.
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Dillon P, Maurer H, Jenkins J, et al.: A prospective study of nonrhabdomyosarcoma soft tissue sarcomas in the pediatric age group. J Pediatr Surg 27 (2): 241-4; discussion 244-5, 1992.
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Rao BN: Nonrhabdomyosarcoma in children: prognostic factors influencing survival. Semin Surg Oncol 9 (6): 524-31, 1993 Nov-Dec.
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Fletcher CD, Dal Cin P, de Wever I, et al.: Correlation between clinicopathological features and karyotype in spindle cell sarcomas. A report of 130 cases from the CHAMP study group. Am J Pathol 154 (6): 1841-7, 1999.
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Skytting BT, Bauer HC, Perfekt R, et al.: Clinical course in synovial sarcoma: a Scandinavian sarcoma group study of 104 patients. Acta Orthop Scand 70 (6): 536-42, 1999.
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Herzog CE: Overview of sarcomas in the adolescent and young adult population. J Pediatr Hematol Oncol 27 (4): 215-8, 2005.
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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.
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Neville H, Corpron C, Blakely ML, et al.: Pediatric neurofibrosarcoma. J Pediatr Surg 38 (3): 343-6; discussion 343-6, 2003.
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Zeytoonjian T, Mankin HJ, Gebhardt MC, et al.: Distal lower extremity sarcomas: frequency of occurrence and patient survival rate. Foot Ankle Int 25 (5): 325-30, 2004.
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Chang F, Syrjänen S, Syrjänen K: Implications of the p53 tumor-suppressor gene in clinical oncology. J Clin Oncol 13 (4): 1009-22, 1995.
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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.
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Stark AM, Buhl R, Hugo HH, et al.: Malignant peripheral nerve sheath tumours--report of 8 cases and review of the literature. Acta Neurochir (Wien) 143 (4): 357-63; discussion 363-4, 2001.
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McClain KL, Leach CT, Jenson HB, et al.: Association of Epstein-Barr virus with leiomyosarcomas in children with AIDS. N Engl J Med 332 (1): 12-8, 1995.
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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.
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Trassard M, Le Doussal V, Hacène K, et al.: Prognostic factors in localized primary synovial sarcoma: a multicenter study of 128 adult patients. J Clin Oncol 19 (2): 525-34, 2001.
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Guillou L, Benhattar J, Bonichon F, et al.: Histologic grade, but not SYT-SSX fusion type, is an important prognostic factor in patients with synovial sarcoma: a multicenter, retrospective analysis. J Clin Oncol 22 (20): 4040-50, 2004.
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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.
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Marcus KC, Grier HE, Shamberger RC, et al.: Childhood soft tissue sarcoma: a 20-year experience. J Pediatr 131 (4): 603-7, 1997.
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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.
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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.
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