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PubMed1997

Imaging pediatric bone sarcomas. Diagnosis and treatment-related issues.

The treatment of pediatric bone malignancies has undergone dramatic change in the past two decades. With the use of adjuvant chemotherapy, survival of patients with osteosarcoma and Ewing's sarcoma has greatly increased and most extremity lesions are now managed with an initial course of chemotherapy followed by limb-sparing surgery rather than amputation. Radiologists are called on not only to help diagnose and stage these tumors, but also to assess their extent, to determine response to preoperative chemotherapy, and to monitor patients for postoperative complications and recurrent disease. This article discusses imaging solutions to clinical issues that arise during the care of these children.

Bone Neoplasms↗
PubMed2006

Concomitant administration of vincristine, doxorubicin, cyclophosphamide, ifosfamide, and etoposide for high-risk sarcomas: the St. Jude Children's Research Hospital experience.

BACKGROUND: Intensified chemotherapy may improve the outcome of patients with high-risk pediatric sarcomas. Vincristine, doxorubicin, cyclophosphamide, ifosfamide, and etoposide are highly effective against pediatric sarcomas. The authors investigated the feasibility of administering these agents concomitantly within a defined period. METHODS: In the prospective high-risk sarcoma (HIRISA) Phase II trial HIRISA1, pediatric patients with high-risk sarcomas received 3 cycles of intensive vincristine, ifosfamide, etoposide, cyclophosphamide, and doxorubicin (VACIE) before radiotherapy and/or surgery began at Week 9 with concurrent vincristine, cyclophosphamide, and doxorubicin (Week 9) and vincristine and ifosfamide (Week 12). Three additional cycles of VACIE were then given. After delayed hematologic recovery in the first 11 patients, the protocol was modified (HIRISA2) to delay local control therapy until after 5 cycles of VACIE (to be completed within 18 weeks). Patients who responded to the protocols were eligible for myeloablative consolidation with autologous stem cell support. RESULTS: Eleven of 24 patients (median age, 14.9 years) had Ewing sarcoma family of tumors, 9 patients had rhabdomyosarcoma, and 4 patients had unresectable desmoplastic small round cell tumors. Seven of 13 patients on HIRISA2, but none of 11 patients on HIRISA1, completed therapy within the specified time. Reversible Grade 4 myelosuppression was the most common toxicity. Major nonhematologic toxic effects were mucositis, nutritional impairment, hypotension, and peripheral neuropathy. Three patients died of toxicity. The 5-year survival and 5-year event-free survival estimates both were 45.8% +/- 11.2%. CONCLUSIONS: The feasibility of administering intensive chemotherapy regimens like VACIE was dependent in part on the timing of local control therapy. This regimen was associated with significant toxicity.

Adolescent↗
PubMed2005

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.

PURPOSE: To describe the response rate and survival of children and adolescents with unresected or metastatic nonrhabdomyosarcomatous soft tissue sarcomas (NRSTS) treated with vincristine, ifosfamide, and doxorubicin. PATIENTS AND METHODS: Between September 1996 and June 2000, 39 eligible patients received vincristine (1.5 mg/m(2) weekly for 13 doses), ifosfamide (3 g/m(2) daily for 3 days every 3 weeks for seven cycles), doxorubicin (30 mg/m(2) daily for 2 days for six cycles), and mesna (750 mg/m(2) for four doses after ifosfamide). Granulocyte colony-stimulating factor was administered daily (5 mug/kg) after each cycle of chemotherapy. Radiotherapy was administered from weeks 7 through 12. RESULTS: The median patient age at diagnosis was 11.7 years; the most common primary tumor site was lower extremity (36%); and synovial sarcoma was the predominant histology. More than three fourths of all tumors were 5 cm or greater at their largest diameters. The overall objective combined partial and complete response rate was 41% (95% CI, 25.7% to 56.7%). The estimated 3-year overall survival and progression-free survival rates (+/- standard deviation) for eligible patients were 59% +/- 8.2% and 43.6% +/- 7%, respectively. Patients with clinical group III disease had significantly better 3-year and progression-free survival rates compared with patients who presented with metastatic disease. CONCLUSION: The vincristine, ifosfamide, and doxorubicin regimen was moderately active against pediatric NRSTS. Patients with synovial sarcoma had higher response rates than other patients, and patients with unresected disease had improved outcomes. Patients with metastatic disease continue to fare poorly, and newer approaches are indicated for these patients.

Adolescent↗
PubMed2001

Complications of limb-sparing procedures using endoprosthetic replacements about the knee for pediatric skeletal sarcomas.

Ninety-five percent of bone tumors are now managed with limb-sparing techniques. For pediatric patients with bone cancer, such limb reconstruction techniques often involve the placement of large endoprosthetic devices with the goal of improving survivors' quality of life. Nevertheless, few radiologic publications discuss the use of these techniques in children and adolescents. This pictorial essay describes the imaging characteristics of the complications associated with endoprosthetic devices and discusses the conditions that may simulate them.

Adolescent↗
PubMed1997

Cytogenetic and molecular genetic analysis of a pediatric pleomorphic sarcoma reveals similarities to adult malignant fibrous histiocytoma.

Cytogenetic and molecular genetic studies were performed on a pleomorphic sarcoma removed from the left atrium of a 15-year-old girl. Histologic analysis was consistent with a storiform-pleomorphic malignant fibrous histiocytoma (MFH). Although MFH is the most common soft-tissue sarcoma of late adulthood. It is extremely rare in childhood and its existence in the pediatric population remains controversial. Cytogenetic analysis revealed several alterations previously associated with adult MFH, including abnormalities of chromosomal bands 11p11 and 19p13. Moreover, the tumor demonstrated homogeneously staining regions (HSR) and double minute chromosomes (dmin) suggestive of gene amplification. We therefore screened the case for amplification of genes localized to chromosomal bands 12q13-14, including the putative protooncogenes MDM2, CDK4, SAS, CHOP, and CLI, which are frequently amplified and overexpressed in adult MFH. Southern and Northern blot analysis confirmed the coamplification of MDM2, CDK4, SAS, and CHOP. To our knowledge, such coamplification studies of the 12q13-14 amplicon have not been previously detected in pediatric MFH. Our results provide cytogenetic and molecular genetic evidence that pediatric and adult MFH are histogenetically related entities.

Adolescent↗
PubMed2003

Pediatric soft tissue sarcomas.

Many of the soft tissue sarcomas that occur in children are of the same histology as those in adults; however, the relative prevalence of these sarcomas is different between children and adults. In some cases, the biologic behavior of pediatric sarcomas is more benign than that in adults. Treatment for sarcomas in children is also different. Pediatric sarcomas are more commonly responsive to chemotherapy. Furthermore, in children who are still growing, surgery and radiation are associated with higher morbidity than in adults. This article discusses the diagnosis and treatment of rhabdomyosarcoma and undifferentiated sarcomas, with an emphasis on surgical considerations, and the diagnosis and treatment of nonrhabdomyosarcomatous soft tissue sarcomas in children.

Child↗
PubMed2003

Cyclooxygenase-2 expression does not correlate with outcome in osteosarcoma or rhabdomyosarcoma.

PURPOSE: To determine if expression of cyclooxygenase (COX)-2, an inducible enzyme with known tumor-promoting activity, correlates with outcome in pediatric sarcomas. COX-2 overexpression correlates with more aggressive disease in a variety of adult solid tumors. METHODS: Archived human osteosarcoma, rhabdomyosarcoma, and Ewing sarcoma tumors were retrospectively evaluated, blinded to outcome, for COX-2 expression by immunohistochemistry and correlated with patient characteristics and survival. RESULTS: COX-2 expression was detected in 94 of 142 (66%) tumors (osteosarcoma, 66/99 [67%]; rhabdomyosarcoma, 21/35 [60%]; Ewing sarcoma, 7/8 [88%]) and 51 of 85 (60%) diagnostic biopsies (osteosarcoma, 26/45 [58%]; rhabdomyosarcoma, 21/35 [60%]; Ewing sarcoma, 4/5 [80%]). COX-2 expression did not vary with clinicopathologic features and was not predictive of prognosis in these cases. CONCLUSIONS: This study does not support the use of COX-2 expression as an upfront prognostic variable in patients with osteosarcoma or rhabdomyosarcoma. Results from the small number of patients studied with Ewing sarcoma suggest a similar lack of predictive value for COX-2 expression. However, COX-2 inhibitors are not entirely dependent upon enzyme expression for their antitumor effects; this study does not necessarily preclude the use of COX-2 inhibitors for the treatment of pediatric sarcomas.

Adult↗
PubMed2001

Functional and clinical outcomes of limb-sparing therapy for pediatric extremity sarcomas.

PURPOSE: To determine the clinical and functional outcomes of children undergoing limb-sparing therapy for extremity sarcomas. METHODS AND MATERIALS: We retrospectively reviewed 30 patients, age < or = 21 years, who were treated between l979 and l998 with external beam radiotherapy as a component of limb-sparing therapy for primary sarcomas of the extremity at UCSF. Included were patients for whom complete follow-up and functional outcome assessments were available. We assessed the patterns of failure, overall survival, disease-free survival, local control, and limb function. RESULTS: At a median follow-up of 3 years, 12 of the 30 patients recurred: 3 locally, 8 distantly, and 1 with synchronous local and distant disease as site of first progression. Eighteen patients were alive with no evidence of disease. The median overall survival was 10 years, with a median disease-free survival of 8 years. Functional outcome assessment revealed 15 patients retained excellent, 12 good, 1 fair, and 2 poor limb function. CONCLUSION: In pediatric patients receiving limb-sparing therapy, 90% maintained excellent or good limb function without compromising survival, demonstrating the validity of limb preservation in children with extremity sarcomas.

Adolescent↗
PubMed2001

Acquisition of secondary structural chromosomal changes in pediatric ewing sarcoma is a probable prognostic factor for tumor response and clinical outcome.

BACKGROUND: The Ewing sarcoma (ES) group of tumors commonly have the t(11;22)(q24;q12) or other rearrangements involving 22q12. In addition to these consistent aberrations, both numeric and structural aberrations have been reported: namely gains of chromosomes 8 and 12, the unbalanced translocation t(1;16), and deletions at the short arm of chromosome 1. METHODS: To evaluate the frequency and to study the prognostic implications of some of these aberrations in children, the authors performed a pilot study of 26 ES pediatric patients by classic cytogenetics and/or interphase fluorescence in situ hybridization (FISH) and compared these data with clinical parameters. RESULTS: Gains of chromosomes 8 and 12 were detected, by interphase FISH, in 48% (10 of 21) and 38% (6 of 16) of the tumors, respectively, and this was not significant with respect to treatment response. Statistical analysis revealed that the presence of additional secondary structural chromosomal aberrations was associated with an unfavorable outcome (P = 0.0034 as an independent prognostic value as an unfavorable marker). Presence of metastasis at diagnosis also was found to be associated with poor outcome (P = 0.0131). Spectral karyotyping analysis was shown to facilitate the detection of more complex structural chromosomal aberrations in a representative ES tumor. CONCLUSIONS: It is important to determine whether additional structural chromosomal aberrations are present in ES tumors because it appears that a more complex karyotype with multiple chromosomal aberrations is associated with poor outcome in ES.

Adolescent↗
PubMed1978

Computed tomography of pediatric craniofacial sarcoma.

Review of the clinical and roentgenographic findings in 16 children with craniofacial sarcomas has revealed that CT is the most accurate method for delineation of the sarcomas and is indispensable for proper therapy planning and post therapy evaluation. The patients were examined and treated according to a protocol which required a CT examination before any type of therapy and after each mode of therapy, chemotherapy and radiotherapy. Chemotherapy alone was noted to produce marked reduction in tumor volume. The orbital sarcomas, which are most frequent, are small tumors without bone destruction. On the other hand, the nasopharyngeal, paranasal sinus and middle ear sarcomas are large tumors frequently associated with extensive bone destruction.

Adolescent↗
PubMed2001

Fractures in pediatric Ewing sarcoma.

PURPOSE: To determine the incidence, timing, and clinical significance of long-bone fractures in children with Ewing sarcoma family of tumors (ESFT). PATIENTS AND METHODS: We retrospectively reviewed 93 consecutive cases of ESFT of the long bones seen at a single institution over the course of a 37-year period. RESULTS: Fracture occurred in 14 (15%) of 93 patients with long-bone ESFT, most commonly in the femur. Approximately 30% of patients with tumors of the femur had fractures at some point in the course of their disease. The incidence of fracture was highest among patients with tumors of the proximal third of the femur (50%); these fractures were usually present at the time of initial diagnosis. Nine (64%) of the 14 fractures occurred after the start of radiotherapy, and three of these were associated with either local recurrence or second malignancy. CONCLUSIONS: Patients with femoral ESFT are at high-risk for fracture. If fractures occur after the completion of therapy, recurrence or second malignancy should be suspected.

Adolescent↗
PubMed2006

Is PET useful in detecting occult nonpulmonary metastases in pediatric bone sarcomas?

UNLABELLED: Orthopaedic oncologists are increasingly utilizing positron emission tomography (PET) technology in the initial workup and staging of sarcomas and for monitoring treatment response. We evaluated the use of PET with fluorine-18-fluoro-2-deoxy D-glucose (FDG) to detect occult nonpulmonary metastases in patients < age 30 newly diagnosed with either Ewing's or osteosarcoma, and the impact of this information upon therapeutic decision making. We retrospectively reviewed prospectively collected data (1994-2004) on 55 patients age < 30 years old over a 10 year span. PET detected metastases in 12/55 (22%) of these patients, eight of whom (67%) harbored disease outside the lung; however, only 4/55 (7%) were upstaged to Stage IV specifically due to findings determined by PET alone. Three of 17 (18%) Ewing's sarcoma patients, but only one of 38 (3%) osteosarcoma patients, were upstaged by PET alone. The most important alteration in treatment decisions was the substitution of irradiation in lieu of surgery for local control in Ewing's sarcoma patients. LEVEL OF EVIDENCE: Diagnostic study, level II.

Adolescent↗
PubMedDate not supplied

Technetium-99m tetrofosmin scintigraphy in pediatric osteogenic sarcoma.

Technetium-99m tetrofosmin (TF) is a myocardial perfusion agent that has recently proved useful also as tumor-seeking tracer. The aim of this study was to make a preliminary assessment of TF scintigraphy in osteosarcoma, with regard to tumor detection and monitoring of response to preoperative chemotherapy (PC). Current clinical investigations have demonstrated that the curability of osteogenic sarcoma mainly depends on the response to PC, and the percentage of tumor necrosis is the most powerful prognostic factor. However, this is difficult to assess radiologically, and it can only been obtained by histologic examination after surgery. Fourteen patients (7 males, age range: 1-17 years) with recently discovered osteosarcoma of the extremities or the pelvis were injected with TF ( approximately 7.4 MBq/kg i.v., never in the affected limb): a dynamic acquisition (20 min) was performed immediately post-injection, followed by static planar views of the involved area. Six patients were studied twice: before and after PC. Images were evaluated visually and quantitatively using regions of interest over lesion (L) and controlateral normal tissue (N). All initial TF scans demonstrated very rapid tumoral uptake (1-2 min post-injection); the mean L/N ratio at 20 min was 2.76+/-1.05. The post-PC scans showed reduced uptake in 4 patients, unchanged in one and increased accumulation in one case, with a strong correlation between the percentage change in L/N and the percentage of tumor necrosis. Our preliminary data indicate that TF is able to detect osteosarcoma; further studies including larger population is need to confirm its usefulness in non-invasively predicting response to PC.

Adolescent↗