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Important: This information is intended mainly for use by doctors and other health care professionals. If you have questions about this topic, you can ask your doctor, or call the Cancer Information Service at 1-800-4-CANCER (1-800-422-6237).

Childhood soft tissue sarcoma


Table of Contents

GENERAL INFORMATION
CELLULAR CLASSIFICATION
STAGE INFORMATION
TREATMENT OPTION OVERVIEW
NONMETASTATIC CHILDHOOD SOFT TISSUE SARCOMA
METASTATIC CHILDHOOD SOFT TISSUE SARCOMA
RECURRENT CHILDHOOD SOFT TISSUE SARCOMA

GENERAL INFORMATION

This treatment information summary on childhood soft tissue sarcoma is an overview of prognosis, diagnosis, classification, and treatment. The National Cancer Institute created the PDQ database to increase the availability of new treatment information and its use in treating patients. Information and references from the most recently published literature are included after review by pediatric oncology specialists.

Cancer in children and adolescents is rare. A team approach, incorporating the skills of the primary care physician, surgical specialists, radiation oncologist, pediatric hematologist/oncologist, rehabilitation specialist, pediatric nurse specialists, and social worker is imperative to ensure that patients receive treatment that will ensure optimal survival results. For advances to be made in treating these patients, therapy is best delivered in the context of a clinical trial at a major medical center with expertise in treating children. Only through entry of all eligible children into appropriate, well-designed clinical trials will progress be made against these diseases. 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]

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, account for more than one half of all cases of soft tissue sarcomas in children. (See PDQ Rhabdomyosarcoma information summary) The remaining nonrhabdomyosarcomatous soft tissue sarcomas 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] These tumors are histologically classified according to the adult tissue they most resemble, and synovial sarcomas, fibrosarcomas, and neurofibrosarcomas predominate in pediatric patients.[5,6]

Nonrhabdomyosarcomatous soft tissue sarcomas are more common in adults (approximately 5,700 new cases each year)[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. However, pediatric nonrhabdomyosarcomatous soft tissue sarcomas are often associated with a better outcome. This difference is most pronounced for infants and young children (<4 years of age) with fibrosarcoma, whose tumors are locally aggressive but not metastatic; these patients have an excellent prognosis when treated with surgery only.[3,4,7] 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, nonrhabdomyosarcomatous soft tissue sarcomas arise most commonly in the trunk and extremities.[5,6] 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 nonrhabdomyosarcomatous soft tissue sarcomas. Heritable cancer-associated changes of the p53 tumor suppressor gene can occur in families with Li-Fraumeni syndrome.[8] 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.[4,9] Some nonrhabdomyosarcomatous soft tissue sarcomas (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 acquired immune deficiency syndrome.[3,4,10]

The treatment of rhabdomyosarcoma is covered in the treatment information summary on rhabdomyosarcoma. Extraosseous Ewing's, peripheral neuroepithelioma, and Askin's tumor are discussed in the treatment information summary on Ewing's sarcoma. Because the prognosis and biology of these tumors vary greatly depending on the age of the patient, the specific histology, the primary site, and the extent of disease, and because the 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. Studies have shown that tumor size, tumor invasiveness, histologic grade, and extent of disease at diagnosis influence outcome in pediatric patients with nonrhabdomyosarcomatous soft tissue sarcomas, and these prognostic factors should be weighed before initiating therapy for these patients.[6,11-13]

References:

  1. Sanders J, Glader B, Cairo M, et al.: 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): 139-141, 1997.

  2. Pappo AS, Pratt CB: Soft tissue sarcomas in children. Cancer Treatment and Research 91: 205-222, 1997.

  3. 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. Philadelphia, PA: Lippincott-Raven, 3rd ed., 1997, pp 865-888.

  4. Enzinger FM, Weiss SW, Eds.: Soft Tissue Tumors. St. Louis, MO: Mosby, 3rd ed., 1995.

  5. Dillon P, Maurer H, Jenkins J, et al.: A prospective study of nonrhabdomyosarcoma soft tissue sarcomas in the pediatric age group. Journal of Pediatric Surgery 27(2): 241-245, 1992.

  6. Rao BN: Nonrhabdomyosarcoma in children: prognostic factors influencing survival. Seminars in Surgical Oncology 9(6): 524-531, 1993.

  7. 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. Journal of Pediatric Surgery 30(7): 1038-1041, 1995.

  8. Chang F, Syrjanen S, Syrjanen K: Implications of the p53 tumor-suppressor gene in clinical oncology. Journal of Clinical Oncology 13(4): 1009-1022, 1995.

  9. deCou JM, Rao BN, Parham DM, et al.: Malignant peripheral nerve sheath tumors: the St. Jude Children's Research Hospital experience. Annals of Surgical Oncology 2(6): 524-529, 1995.

  10. McClain KL, Leach CT, Jenson HB, et al.: Association of Epstein-Barr virus with leiomyosarcomas in children with AIDS. New England Journal of Medicine 332(1): 12-18, 1995.

  11. Pappo AS, Fontanesi J, Luo X, et al.: Synovial sarcoma in children and adolescents: the St. Jude Children's Research Hospital experience. Journal of Clinical Oncology 12(11): 2360-2366, 1994.

  12. Marcus KC, Grier HE, Shamberger RC, et al.: Childhood soft tissue sarcoma: a 20-year experience. Journal of Pediatrics 131(4): 603-607, 1997.

  13. 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. Journal of Clinical Oncology 17(4): 1219-1226, 1999.


CELLULAR CLASSIFICATION

Pediatric soft tissue sarcomas are classified histologically according to the soft tissue cell they resemble and include the following.[1]

tumors of fibrous tissue:

fibrohistiocytic tumors:
tumors of adipose tissue:
tumors of smooth muscle:
tumors of blood and lymph vessels:
tumors of synovial tissue:
tumors of peripheral nervous system:
tumors of bone and cartilage:
tumors of more than one tissue type:
tumors of unknown histogenesis:
Nonrhabdomyosarcomatous soft tissue tumors are fairly readily distinguished from rhabdomyosarcoma or Ewing's family of tumors. To distinguish between various nonrhabdomyosarcomatous lesions, tumor samples should be carefully evaluated by using immunocytochemical tests and light and electron microscopy.[1,2] Many nonrhabdomyosarcomatous soft tissue sarcomas are characterized by chromosomal abnormalities. Some of these chromosomal translocations lead to fusion of two disparate genes. The resulting fusion transcript can be readily detected by using polymerase chain reaction-based techniques, thus facilitating the diagnosis of those neoplasms that have translocations. Some of the most frequent aberrations seen in nonrhabdomyosarcomatous soft tissue tumors are listed in Table 1.[3-5]


Table 1.  Frequent aberrations seen in nonrhabdomyosarcomatous soft tissue
          tumors
-------------------------------------------------------------------------------
Histology                        Chromosomal aberrations        Genes involved
-------------------------------------------------------------------------------

Infantile fibrosarcoma           Trisomy 11; also +8, +17, +20
Neurofibrosarcoma                Deletion 17q11.2
Malignant fibrous histiocytoma   19p+, ring chromosome
Hemangiopericytoma               t(12;19)(q13;q13.3) and
                                   t(13;22)(q22;q13.3)
Alveolar soft part sarcoma       17q25 abnormalities
Leiomyosarcoma                   t(12;14)
Synovial sarcoma                 t(X;18)(p11.2;q11.2)           SYT/SSX
Clear cell sarcoma               t(12;22)(q13-14;q12-13)        ATF1/EWS
Myxoid liposarcoma               t(12;16)(q13;p11)              FUS/CHOP

In the majority of cases, accurate histopathologic classification of soft tissue sarcomas does not yield optimal information about their clinical behavior. Therefore, several histologic parameters including degree of cellularity, cellular pleomorphism, mitotic activity, degree of necrosis, and invasive growth are evaluated in the process known as "grading." This process is used to improve the correlation between histologic findings and clinical outcome.[6] In children, grading of soft tissue sarcomas is compromised by the good prognosis of certain tumors such as infantile fibrosarcoma. In addition, testing of a grading system within the pediatric population is difficult due to the rarity of these neoplasms. In March 1986, the Pediatric Oncology Group (POG) conducted a prospective study for pediatric soft tissue sarcomas other than rhabdomyosarcoma and devised the grading system that is shown below. Analysis of outcome for patients with localized soft tissue sarcomas other than rhabdomyosarcoma demonstrated that patients with grade 3 tumors fared significantly worse than did those with grade 1 or grade 2 lesions. This finding suggests that this system can accurately predict the clinical behavior of nonrhabdomyosarcomatous soft tissue tumors in children.[2,6]

Grade 1 lesions:

Grade 2 lesions:
Soft tissue sarcomas not included in grades 1 and 3. In grade 2
lesions, less than 15% of the surface area shows necrosis and there are
less than
five mitotic figures per 10 high-power fields (40X objective). As
secondary criteria of grade 2 tumors, nuclear atypia is not marked, and
the tumor is not markedly cellular.

Grade 3 lesions:
References:

  1. Enzinger FM, Weiss SW, Eds.: Soft Tissue Tumors. St. Louis, MO: Mosby, 3rd ed., 1995.

  2. Association of Directors of Anatomic and Surgical Pathology: Recommendations for the reporting of soft tissue sarcomas. Modern Pathology 11(12): 1257-1261, 1998.

  3. Sreekantaiah C, Ladanyi M, Rodriguez E, et al.: Chromosomal aberrations in soft tissue tumors. Relevance to diagnosis, classification, and molecular mechanisms. American Journal of Pathology 144(6): 1121-1134, 1994.

  4. Rabbitts TH: Chromosomal translocations in human cancer. Nature 372(6502): 145-149, 1994.

  5. Shipley J, Crew J, Gusterson B: The molecular biology of soft tissue sarcomas. European Journal of Cancer 29A(14): 2054-2058, 1993.

  6. Parham DM, Webber BL, Jenkins JJ 3rd, et al.: Nonrhabdomyosarcomatous soft tissue sarcomas of childhood: formulation of a simplified system for grading. Modern Pathology 8(7): 705-710, 1995.


STAGE INFORMATION

Clinical staging has an important role in predicting the clinical outcome and determining the most effective therapy for pediatric soft tissue sarcomas. As yet, there is no well-accepted staging system that is applicable to all childhood sarcomas; the system from the American Joint Commission for Cancer that is used for adults has not been validated in pediatric studies.[1] Two systems are currently in use for staging pediatric nonrhabdomyosarcomatous soft tissue tumors. The surgicopathologic staging system used by the Intergroup Rhabdomyosarcoma Study (see below) is based on the amount of tumor that remains after initial surgery and on whether the disease has metastasized.[2]

Nonmetastatic disease

Metastatic disease
Recurrent disease
The other schema typically used to stage pediatric soft tissue tumors is the tumor-node-metastases system of the International Union Against Cancer.[3] In this staging system, T1 lesions are those that are confined to the organ of origin; T2 lesions invade adjacent organs. These categories can be subclassified to reflect the maximum tumor diameter (a: </=5 cm; b: >5 cm). Nodal involvement is indicated by N1 (N0: no nodal involvement), and the presence of distant metastases at the time of diagnosis is indicated by the M1 (versus M0) designation. Several adult and pediatric series have shown that patients with large or invasive tumors have a significantly worse prognosis than do those with small, noninvasive tumors.

These two staging systems have proven to be of prognostic significance in pediatric and adult nonrhabdomyosarcomatous soft tissue sarcomas.[4-7] In a review of a large adult series of non-rhabdo sarcomas, superficial extremity sarcomas have a better prognosis than deep tumors. Thus, in addition to grade and size, the depth of invasion of the tumor should be considered.[8]

References:

  1. Enzinger FM, Weiss SW, Eds.: Soft Tissue Tumors. St. Louis, MO: Mosby, 3rd ed., 1995.

  2. Maurer HM, Beltangady M, Gehan EA, et al.: The Intergroup Rhabdomyosarcoma Study-I: a final report. Cancer 61(2): 209-220, 1988.

  3. Harmer MH, Ed.: TNM Classification of Pediatric Tumors. Geneva: UICC, 1982.

  4. Rao BN: Nonrhabdomyosarcoma in children: prognostic factors influencing survival. Seminars in Surgical Oncology 9(6): 524-531, 1993.

  5. Pisters PW, Leung DH, Woodruff J, et al.: Analysis of prognostic factors in 1,041 patients with localized soft tissue sarcomas of the extremities. Journal of Clinical Oncology 14(5): 1679-1689, 1996.

  6. Coindre JM, Terrier P, Bui NB, et al.: Prognostic factors in adult patients with locally controlled soft tissue sarcoma. A study of 546 patients from the French Federation of Cancer Centers Sarcoma Group. Journal of Clinical Oncology 14(3): 869-877, 1996.

  7. Pappo AS, Fontanesi J, Luo X, et al.: Synovial sarcoma in children and adolescents: the St. Jude Children's Research Hospital experience. Journal of Clinical Oncology 12(11): 2360-2366, 1994.

  8. Brooks AD, Heslin MJ, Leung DH, et al.: Superficial extremity soft tissue sarcoma: an analysis of prognostic factors. Annals of Surgical Oncology 5(1): 41-47, 1998.


TREATMENT OPTION OVERVIEW

Because of the rarity of pediatric nonrhabdomyosarcomatous soft tissue sarcomas, all children, adolescents, and young adults with these tumors should have their treatment planned by a multidisciplinary team composed of pediatric oncologists, surgeons, and radiotherapists. To better define the natural history and response to therapy of these tumors, children with these rare neoplasms should be considered for entry into national or institutional treatment protocols.

Wide surgical resection of the primary tumor to yield negative margins remains the mainstay of therapy for pediatric soft tissue sarcomas other than rhabdomyosarcoma.[1,2] When there is concern about the adequacy of the surgical margin, radiation therapy is indicated [3] particularly in high grade tumors with tumor margins less than 1 cm.[4] By using these two treatment modalities, local control of the primary tumor can be achieved in more than 80% of patients.[5]

Therapeutic strategies for children and adolescents with soft tissue tumors are similar to those for adult patients, although there are important differences. For example, the biology of the pediatric form of the neoplasm may differ dramatically from that of the adult lesion. In addition, the morbidity of radiation therapy in young children may be much greater than that observed in adults, and limb-sparing procedures are more difficult to perform in pediatric patients. Further, the concern regarding potential long-term side effects of combined modality therapy (radiation, surgery, and chemotherapy) is greater for children, whose survival may be much longer than that of adults. Therefore, to maximize tumor control and minimize long-term morbidity, treatment must be individualized for children and adolescents with nonrhabdomyosarcomatous soft tissue tumors. These patients should be enrolled in prospective studies that accurately assess any potential complications.[1]

References:

  1. 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. Philadelphia, PA: Lippincott-Raven, 3rd ed., 1997, pp 865-888.

  2. Rao BN: Nonrhabdomyosarcoma in children: prognostic factors influencing survival. Seminars in Surgical Oncology 9(6): 524-531, 1993.

  3. Marcus KC, Grier HE, Shamberger RC, et al.: Childhood soft tissue sarcoma: a 20-year experience. Journal of Pediatrics 131(4): 603-607, 1997.

  4. Blakely ML, Spurbeck WW, Pappo AS, et al.: The impact of margin of resection on outcome in pediatric nonrhabdomyosarcoma soft tissue sarcoma. Journal of Pediatric Surgery 34(5): 672-675, 1999.

  5. 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. Journal of Clinical Oncology 17(4): 1219-1226, 1999.


NONMETASTATIC CHILDHOOD SOFT TISSUE SARCOMA

The outcome for selected nonmetastatic pediatric soft tissue sarcomas is shown in Table 2.


Table 2.  Outcome for selected nonmetastatic pediatric soft tissue sarcomas
-------------------------------------------------------------------------------
Histologic Subtype              Outcome
-------------------------------------------------------------------------------

Infantile fibrosarcoma          Excellent with surgery alone

Adult-type fibrosarcoma         Similar to that in adults (60%)

Neurofibrosarcoma               Stage-related: very good with complete
                                excision; poor with incomplete excision

Malignant fibrous histiocytoma  Approximate survival 50%

   Angiomatoid variant          Excellent with surgery alone

Leiomyosarcoma                  Prognosis good for tumors arising outside the
                                gastrointestinal tract but poor for those in
                                the gastrointestinal tract

Liposarcoma                     Good with complete resection

Synovial sarcoma                80% survival for localized grossly or
                                completely resected tumors

Hemangiopericytoma              Excellent in young children; 30% to 70% in
                                older patients

Alveolar soft part sarcoma      50% survival; late relapses are common

Desmoid tumors                  Excellent survival; rarely metastasizes

A. Fibrosarcomas and hemangiopericytomas in infants and young children, desmoid tumors, and angiomatoid malignant fibrous histiocytomas typically are clinically less aggressive, rarely metastasize, and can be treated with surgery alone.[1]

Treatment options:

Standard:

For nonmetastatic pediatric nonrhabdomyosarcomatous soft tissue sarcomas,
treatment with surgery alone is often curative.[1-5] Postoperatively,
tumor-free margins must be confirmed through pathologic evaluation, and re-
excision must be performed if the margins are positive. For patients with
local recurrence, re-excision of the mass is indicated in an attempt to
avoid radiation therapy. If further resection is not feasible, postoperative
radiation therapy should be used.[6] 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] 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 have a significant potential for local invasiveness and
recurrence. A surgical approach to clear margins is the treatment of
choice. When 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-192, nonsteroidal antiinflammatory agents,
antiestrogens, vinblastine, and methotrexate should be considered.
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.[7-10]

Under clinical evaluation:
Vinblastine and methotrexate in recurrent or unresectable desmoid tumors.

B. The following pediatric neoplasms exhibit similar biologic behavior to those lesions in adults. Treatment for these tumors will be discussed together. Treatment options:

Standard:

Every attempt should be made to resect the primary tumor locally with
negative margins. If the original surgery failed to achieve pathologically
negative tissue margins, a second surgery may be indicated.[2] Although
combined surgery and radiation therapy has dramatically improved outcome in
adults and children with soft tissue sarcomas over the past 20 years,[6] the
morbidity of high-dose radiation therapy should be taken into consideration
in infants and young children with these tumors.[11] The use of
brachytherapy and intraoperative electron irradiation is under study.[12,13]
Preoperative radiation therapy has been associated with excellent local
control rates in adults;[14] this approach has not been used extensively in
pediatric patients. The role of adjuvant (postoperative) chemotherapy
remains controversial. 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.[15] However, a role for chemotherapy has been suggested with
synovial cell sarcomas based on the German prospective study.[16] In
patients with unresectable or metastatic disease treated with vincristine,
dactinomycin, and cyclophosphamide, the overall and disease-free survival
rates were 31% and 10%, respectively.[17]

Under clinical evaluation:
None at present.

C. Alveolar soft part sarcoma is a tumor of uncertain histogenesis that has some unique biological features. This neoplasm is characterized by an indolent clinical course, unresponsiveness to chemotherapy, and usually a fatal outcome in nearly 50% of cases.[18]

Treatment options:

Standard:

The standard approach is complete surgical resection of the primary lesion.
If complete surgical excision is not feasible, radiation therapy should be
administered. The value of adjuvant chemotherapy in completely resected
alveolar soft part sarcomas remains unproven, particularly because patients
with unresected or metastatic tumors failed to respond to chemotherapeutic
agents frequently used to treat soft tissue sarcomas. Patients with
alveolar soft part sarcomas may relapse several years after a prolonged
period of apparent remission.[19]

Under clinical evaluation:
The role of adjuvant chemotherapy in children with this malignancy has not
been tested. Because these tumors are rare, all children with alveolar soft
part sarcoma should be enrolled in prospective clinical trials.

References:

  1. 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. Philadelphia, PA: Lippincott-Raven, 3rd ed., 1997, pp 865-888.

  2. Rao BN: Nonrhabdomyosarcoma in children: prognostic factors influencing survival. Seminars in Surgical Oncology 9(6): 524-531, 1993.

  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. Journal of Pediatric Surgery 30(7): 1038-1041, 1995.

  4. deCou JM, Rao BN, Parham DM, et al.: Malignant peripheral nerve sheath tumors: the St. Jude Children's Research Hospital experience. Annals of Surgical Oncology 2(6): 524-529, 1995.

  5. Pappo AS, Fontanesi J, Luo X, et al.: Synovial sarcoma in children and adolescents: the St. Jude Children's Research Hospital experience. Journal of Clinical Oncology 12(11): 2360-2366, 1994.

  6. Marcus KC, Grier HE, Shamberger RC, et al.: Childhood soft tissue sarcoma: a 20-year experience. Journal of Pediatrics 131(4): 603-607, 1997.

  7. Faulkner LB, Hajdu SI, Kher U, et al.: Pediatric desmoid tumor: retrospective analysis of 63 cases. Journal of Clinical Oncology 13(11): 2813-2818, 1995.

  8. 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): 380-384, 1991.

  9. Weiss AJ, Lackman RD: Low-dose chemotherapy of desmoid tumors. Cancer 64(6): 1192-1194, 1989.

  10. 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): 2863-2868, 1987.

  11. Suit H, Spiro I: Radiation as a therapeutic modality in sarcomas of the soft tissue. Hematology Oncology Clinics of North America 9(4): 733-746, 1995.

  12. Fontanesi J, Rao BN, Fleming ID, et al.: Pediatric brachytherapy. The St. Jude Children's Research Hospital experience. Cancer 74(2): 733-739, 1994.

  13. Schomberg PJ, Gunderson LL, Moir CR, et al.: Intraoperative electron irradiation in the management of pediatric malignancies. Cancer 79(11): 2251-2256, 1997.

  14. Sadoski C, Suit HD, Rosenberg A, et al.: Preoperative radiation, surgical margins, and local control of extremity sarcomas of soft tissues. Journal of Surgical Oncology 52(4): 223-230, 1993.

  15. 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. Journal of Clinical Oncology 17(4): 1219-1226, 1999.

  16. Ladenstein R, Treuner J, Koscielniak E, et al.: Synovial sarcoma of childhood and adolescence: report of the German CWS-81 study. Cancer 71(11): 3647-3655, 1993.

  17. 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. Medical and Pediatric Oncology 30: 201-209, 1998.

  18. Pappo AS, Parham DM, Cain A, et al.: Alveolar soft part sarcoma in children and adolescents: clinical features and outcome of 11 patients. Medical and Pediatric Oncology 26(2): 81-84, 1996.

  19. Lieberman PH, Brennan MF, Kimmel M, et al.: Alveolar soft-part sarcoma--a clinicopathologic study of half a century. Cancer 63(1): 1-13, 1989.


METASTATIC CHILDHOOD SOFT TISSUE SARCOMA

The prognosis for children with metastatic soft tissue sarcomas is poor,[1-6] and these children should receive combined treatment with chemotherapy, radiation therapy, and surgical resection of pulmonary metastases. In a prospective randomized trial, chemotherapy with vincristine, dactinomycin, doxorubicin, and cyclophosphamide with or without dacarbazine led to tumor responses in one third of patients with unresectable or metastatic disease. However, the estimated 4-year survival rate was poor, with less than one third of children surviving.[7]

Treatment options:

Standard:

Children with isolated pulmonary metastases should undergo exploratory
thoracotomy in an attempt to resect all gross disease. The estimated 5-year
survival rate after thoracotomy for pulmonary metastasectomy has ranged from
10% to 58% in adult studies. Formal segmentectomy, lobectomy, and
mediastinal lymph node dissection are unnecessary.[8]

Under clinical evaluation:
Vincristine, doxorubicin, and ifosfamide with granulocyte colony-stimulating
factor in patients with unresected or metastatic tumors. The Pediatric
Oncology Group is prospectively evaluating the combination of doxorubicin
and ifosfamide in children with unresected or metastatic soft tissue
sarcomas because several adult trials have suggested that ifosfamide-based
regimens may be superior to other chemotherapeutic regimens for soft tissue
sarcomas.

References:

  1. Demetri GD, Elias AD: Results of single-agent and combination chemotherapy for advanced soft tissue sarcomas. Implications for decision making in the clinic. Hematology Oncology Clinics of North America 9(4): 765-785, 1995.

  2. Elias A, Ryan L, Sulkes A, et al.: Response to mesna, doxorubicin, ifosfamide, and dacarbazine in 108 patients with metastatic or unresectable sarcoma and no prior chemotherapy. Journal of Clinical Oncology 7(9): 1208-1216, 1989.

  3. Edmonson JH, Ryan LM, Blum RH, et al.: Randomized comparison of doxorubicin alone versus ifosfamide plus doxorubicin or mitomycin, doxorubicin, and cisplatin against advanced soft tissue sarcomas. Journal of Clinical Oncology 11(7): 1269-1275, 1993.

  4. Rao BN: Nonrhabdomyosarcoma in children: prognostic factors influencing survival. Seminars in Surgical Oncology 9(6): 524-531, 1993.

  5. deCou JM, Rao BN, Parham DM, et al.: Malignant peripheral nerve sheath tumors: the St. Jude Children's Research Hospital experience. Annals of Surgical Oncology 2(6): 524-529, 1995.

  6. Pappo AS, Fontanesi J, Luo X, et al.: Synovial sarcoma in children and adolescents: the St. Jude Children's Research Hospital experience. Journal of Clinical Oncology 12(11): 2360-2366, 1994.

  7. 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. Journal of Clinical Oncology 17(4): 1219-1226, 1999.

  8. Putnam JB Jr, Roth JA: Surgical treatment for pulmonary metastases from sarcoma. Hematology Oncology Clinics of North America 9(4): 869-887, 1995.


RECURRENT CHILDHOOD SOFT TISSUE SARCOMA

With the possible exception of infants with congenital fibrosarcoma, the prognosis for patients with recurrent or progressive disease is poor. The selection of further treatment depends on many factors, including the site of recurrence, prior therapy, and individual patient considerations. Local recurrence or isolated pulmonary recurrence should be treated with complete surgical excision. All patients with recurrent tumors should be offered enrollment in current drug studies.

Treatment options:

Standard:

Surgical excision is the standard treatment for recurrent pediatric
nonrhabdomyosarcomatous soft tissue sarcomas. If the patient has not yet
received radiation therapy, adjuvant radiation should be considered after
local excision of the recurrent tumor. Limb-sparing procedures with adjuvant
brachytherapy has been evaluated in adults but has not been studied
extensively in children. For some children with extremity sarcomas who have
received previous radiation therapy, amputation may be the only therapeutic
option. No prospective trial has been able to prove that enhanced local
control of pediatric soft tissue sarcomas will ultimately improve survival.
Therefore, treatment should be individualized in light of the site of
recurrence and biologic characteristics (e.g., grade, invasiveness, and
size) of the tumor. All patients should be considered for enrollment in
clinical trials.

Under clinical evaluation:
Phase I and II trials of chemotherapy.

Date Last Modified: 10/1999



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