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Background Survivors of lower extremity (LE) malignancies experience functional deficits. Purpose The purpose of this prospective clinical trial was to determine feasibility and functional outcomes of adding pre-habilitation during the 10-12 week period prior to a planned surgery to remove the tumor in children and adolescents with a LE sarcoma. Design Pilot study. Setting St. Jude Children's Research Hospital (SJCRH). Patients Participants included 14 individuals between the ages of 8 and 20 years who were diagnosed with a LE sarcoma. Participant outcomes were compared to a control group of 35 individuals treated for osteosarcoma that obtained the same functional assessments but no pre-habilitation. Intervention The intervention group received strengthening exercises and mobility training 3 times per week for 30-60 minutes for 10-12 weeks preoperatively. Measurements Participants completed the Functional Mobility Assessment (FMA) and measures of strength and range of motion (ROM) of bilateral lower extremities (BLEs) at baseline, after 10-12 weeks of preoperative PT, and at 20-22 weeks. Results Twelve participants completed at least 50% of their schedule pre-habilitative sessions. The intervention group scored significantly better on the FMA than the control group at weeks 20-22 (35.6 vs. 25.7, p .0267). No significant difference was found in ROM or strength. Limitations Due to this study being a pilot study, the sample size was small. Therefore, we cannot infer generalizability. Conclusions Findings suggest that those diagnosed with a LE malignancy awaiting a limb sparing procedure or amputation participate in at least 50% of scheduled PT sessions and benefit from them.

Limb-salvage surgery for malignant tumors frequently involves reconstruction with an endoprosthesis anchored to bone by using third-generation cementing techniques. A 10-year-old boy with osteosarcoma had a pulmonary embolus caused by polymethylmethacrylate after having limb-salvage surgery that used high-pressure cementing techniques. He experienced transient postoperative chest pain, and a new wedge-shaped radiodense pulmonary lesion appeared on a computed tomography scan of the chest. A thoracotomy for resection of suspected metastatic osteosarcoma revealed a pulmonary infarct caused by cement embolization. Awareness of this potential complication should prompt investigation of possible pulmonary embolism and may prevent unnecessary thoracotomy.

A noninvasive expandable prosthesis for skeletally immature children after limb salvage surgery has been developed. Between 1998 and 2001, 18 Phenix prostheses were implanted in 15 pediatric patients who had been diagnosed and treated for osteosarcoma about the knee. Of the 15 original prostheses, 10 were implanted at the time of primary tumor resection and five were revisions from an endoprosthetic modular knee system. Sixty expansions of the 18 prostheses were done, with all but two done as outpatient procedures. An average 8.5 mm was obtained per lengthening (range, 1-30 mm). The average followup was 21.5 months (range, 12-33 months) and the latest Musculoskeletal Tumor Society functional scores averaged 90%. Eight revisions were required for stem fracture or loosening. There was one amputation because of a postoperative arterial thrombosis. The principle of the Phenix prosthesis involves storage of energy in a spring compressed by a locking system. Lengthening is achieved via exposure to an electromagnetic field that allows controlled release of the spring. This is a unique expandable custom prosthesis that offers many benefits in maintaining limb length equality in growing patients. Although the early experience is promising, additional data are required regarding the long-term structural integrity of the prosthesis. We are optimistic that this technology will prove beneficial, not only for patients with malignant bone tumors but in applications requiring serial limb length equalizations or for spinal deformities.

Review of magnetic resonance imaging was compared with the pathologic review of reexcision specimens from 24 pediatric patients who had initial incomplete resection for soft tissue sarcoma to determine the accuracy of magnetic resonance imaging in detecting residual tumor. The median age of patients was 12.8 years. The most common diagnosis was synovial sarcoma (eight of 24 patients; 30%). Magnetic resonance imaging detected residual tumor in eight patients and no tumor in eight patients; the images of eight patients were indeterminate. Pathologic examination showed residual tumor in 14 of 24 (58%) specimens obtained in reexcision. For two of the eight patients (25%) for whom imaging had detected no tumor, microscopic examination detected residual tumor; for one of the eight patients for whom imaging had detected tumor, microscopic examination detected no tumor. Five of the eight patients (63%) for whom the results of imaging studies were indeterminate had residual tumor on microscopic evaluation. The sensitivity of magnetic resonance imaging for detecting residual tumor was 78%, the specificity was 86%, the positive predictive value was 0.78, and the negative predictive value was 0.86. Decisions regarding the need for additional resection should not be based on magnetic resonance imaging alone.

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.

Purpose To evaluate the long-term sequelae of treatment for malignant germ cell tumors (GCT) during childhood and adolescence. Patients and methods Of 128 patients treated for GCT at St. Jude Children's Research Hospital between 1962 and 1988, 73 are long-term survivors (continuously disease-free for > or =5 years after diagnosis), with a median follow-up of 11.3 years). Survivors' ages at diagnosis ranged from birth to 18.3 years (median, 9.2 years); 64% (47 patients) were female. Initial surgical resection was followed by observation for stage I germinomas (n = 2), testicular tumors (n = 13), and selected cases of ovarian or sacrococcygeal tumors (n = 2), and by radiation therapy (RT) for patients with stage II to III germinoma (n = 8). The remaining 48 patients received postoperative chemotherapy (vincristine, dactinomycin, and cyclophosphamide [VAC] +/- doxorubicin, 1962 to 1978; VAC and/or cisplatin, vinblastine, and bleomycin [PVB], 1979 to 1988). RT was added to the chemotherapy for 21 patients. Late complications involving various organ systems and their relationship to treatment were evaluated. Results More than two-thirds of long-term survivors (n = 50) had at least 1 complication, and half (n = 38) had > 1 organ system affected. The systems most often involved included the musculoskeletal (41% of survivors), endocrine (42%), cardiovascular (16% excluding those who had only abnormal chest radiograph), gastrointestinal (25%), genitourinary tract (23%), pulmonary (19%), and neurologic (16%) systems. High-frequency hearing loss occurred in 58% (11 of 19) of patients treated with cisplatin. Musculoskeletal, gastrointestinal, and urinary tract abnormalities were most frequent in patients whose treatment included RT. Conclusions A high frequency of late effects after treatment for pediatric GCT, particularly in patients who received RT, was demonstrated. Treatment sequelae could be anticipated from the intensity and type of therapeutic modalities. Treatment-directed screening evaluations may improve quality of life in long-term survivors of pediatric GCT through timely identification of sequelae that can be prevented or ameliorated.

Purpose: We report the treatment and outcome of patients with peripheral primitive neuroectodermal tumor (PNET) and extraosseous Ewing's tumor (EOE) using Ewing's-directed therapy, including an ifosfamide and etoposide window. Methods: Seventeen pediatric patients with peripheral PNET (n = 14) or EOE (n = 3) were enrolled between 1988 and 1992 on our institutional Ewing's protocol. Induction therapy comprised a 9-week “window” of ifosfamide and etoposide, followed by 9 weeks of therapy with cyclophosphamide and Adriamycin (Adria Laboratories, Columbus, OH). Response assessment after 17 weeks was followed by surgery and/or radiotherapy (doses based on tumor size and response to induction), repeat evaluation, and maintenance chemotherapy with alternating courses of vincristine/dactinomycin, ifosfamide/etoposide, and cyclophosphamide/Adriamycin for a total of 45 weeks. Results: At diagnosis, 8 patients had large lesions (>8 cm) and 3 had pulmonary metastases (1 with large tumor). Surgical resection was performed at diagnosis for 9 patients and after induction therapy for 5. During window therapy, all of the 9 evaluable patients responded (8 partial, 1 objective), and no patient without measurable disease developed disease progression. Responses were maintained or improved during subsequent induction in six of the patients with residual disease. Fourteen patients received local radiotherapy. At 49 to 94 months after diagnosis, 12 patients are disease-free (1 in second remission), 4 have died, and 1 is alive with disease. The five-year overall and progression-free survival rates are 77 ± 13% and 62 ± 16%, respectively. Conclusion: The use of consistent Ewing's-directed combined-modality therapy for patients with soft tissue peripheral PNET/EOE results in survival similar to that of patients with osseous Ewing's tumor. The combination of ifosfamide and etoposide appears active and should be incorporated in future treatment protocols.

Purpose The outcome for children with advanced-stage rhabdomyosarcoma remains poor with contemporary treatment regimens. Evaluation of new drugs is important to improve clinical outcome. Because methotrexate has shown promising activity in the treatment of patients with recurrent rhabdomyosarcoma, we conducted a phase II trial in untreated children with advanced-stage disease to evaluate the efficacy and safety of this agent. Purpose Patients and Methods: Fifteen patients received 1 to 4 courses of high-dose methotrexate (HDMTX, 12 g/m2). Patients then received standard multiagent chemotherapy (vincristine, dactinomycin, cyclophosphamide, ifosfamide, mesna) with cytokine support and local radiotherapy. Patients who responded to HDMTX received four additional courses of this drug during continuation therapy. Purpose Results: Twelve patients were evaluable for response after 2 or more courses of HDMTX; 4 achieved a partial response (33.3%). After administration of standard chemotherapy and radiation, the estimated 2-year progression-free survival for all patients was 56% (SD 15%). The drug was well-tolerated and the most common side effects included mucositis, transient elevation of transaminases, and neutropenia. The four patients who received additional courses of HDMTX during continuation therapy had limited toxicity which included mucositis, anemia, and thrombocytopenia. Purpose Conclusions: About one-third of children with previously untreated advanced-stage rhabdomyosarcoma responded to HDMTX. Its different mechanism of action and non-overlapping toxicity with other agents make HDMTX an attractive candidate for incorporation into front-line treatment regimens for rhabdomyosarcoma.

The objective of this study was to assess the complications and efficacy of gastrostomy (GT) feedings in pediatric cancer patients.We reviewed the medical records of 33 pediatric cancer patients who received enteral nutrition via a GT.Median age was 9.4 years (range, 1-19.8 years), and 28 of the 33 patients had solid tumors. Seventeen patients had a significant weight loss (median, 8.5%) and therapy-related weight loss was anticipated in 16 patients. The GT device was placed an average of 5.5 months after diagnosis. Twenty-five patients were fed via a tube and eight via a button device. The tube was placed surgically in 21 cases (including all eight button types) and endoscopically in 12. Nutritional support lasted a median of 9.5 months. One or more complications occurred in 30 patients and were categorized as (a) insertion site reactions (inflammation, 23; infection/colonization, 14; exuberant granulation tissue, 6); (b) mechanical problems (leaking, 3; obstruction, 2; breakage, 1; accidental dislodgement, 2); (c) insertion site bleeding, 8; and (d) feeding intolerance, 12. Only one insertion site cellulitis progressed to a systemic infection. All eight patients with a button GT experienced insertion site complications, with local infection occurring significantly more often in patients with the button than in those with the tube GT. There were no significant associations between insertion technique and type of complication. Twenty-seven patients (82%) achieved or maintained ideal body weight with this intervention.GT feeding was associated with minor complications, but permitted effective nutritional support for pediatric cancer patients.

The increasing survival of children treated for osteosarcoma has led to an increase in limb sparing surgery. Little published information is available about the postoperative imaging appearance of this technique. Thus, information gleaned from medical records and imaging reviews was correlated with clinical outcome of 19 consecutive children (median age at diagnosis, 12.3 years) treated for distal femoral osteosarcoma with a cemented rotating hinged knee endoprosthesis with porous coated collar. An extracortical bridging bony callus was identified in 16 patients who were followed up after surgery for a median of 2.8 years (range, 1.4-6 years). Extracortical bridging bony callus formation was circumferential in eight and preferentially posterior in nine, and ranged from 0.5 to 0.7 cm in thickness; 14 patients had lucent lines subjacent to the bridging bone. Thirteen patients had good functional use of the surgically treated leg. Five patients had progressive metastatic disease develop. One patient had local disease recurrence 8 months after surgery. Extracortical bridging bone was not identified in three patients, two of whom had infection develop 7 months and 17 months after surgery; both infections required amputation. Extracortical bridging bone preferentially develops posteriorly, along the compression side of the femur, often is associated with subjacent lucency, and seems to reflect increased prosthetic stability.

With the development of combined chemotherapy and radiation therapy for embryonal rhabdomyosarcoma, the role and extent of surgical resection of these pelvic tumors need to be defined. Thirty-six children with pelvic genitourinary rhabdomyosarcoma seen at St. Jude Children's were managed on protocols combining surgical resection and radiation, and chemotherapy. Ten children presented with cervical-vaginal tumors, which were managed with combined therapy; the surgical resection was histovaginectomy in eight and pelvic exenteration in one. Eight of the ten are free of disease from 1 to 14 years. Twelve children presented with bladder and prostate tumors, which were resected with segmental cystectomy in four cases, biopsy in five, and pelvic exenteration in three. All received combination therapy and six of the twelve are surviving free of disease from 6 months to 16 years. Fourteen children presented with paratesticular rhabdomyosarcoma. Chemotherapy was combined with radical orchiectomy in all cases. Retroperitoneal node dissection was done in nine and five had inguinal node dissection. Nine of the 14 are surviving NED from 2 to 16 years. One patient died, free of disease, with complications of combination therapy. The results of this review supports the approach of combining chemotherapy, radiation, and complete surgical resection.