Compliance and satisfaction with intensive physiotherapy treatment during chemotherapy in patients with bone tumours and evaluation of related prognostic factors: An observational study.

The purpose of this study was to evaluate compliance and satisfaction of adult patients to intensive rehabilitation treatment during chemotherapy cycles after surgery for bone-musculoskeletal tumours, as well as to identify possible predictive factors. An observational, prognostic, prospective study was conducted. The study enrolled 27 patients who previously had undergone modular knee prosthesis surgery in the period between October 2014 and October 2015. The outcome was compliance to intensive rehabilitation treatment during hospitalisations in the chemotherapy unit and patient satisfaction 6 months' post-surgery. The variables taken into account were linked to the patient's characteristics, to the oncological pathology and to the chemotherapy treatment administered. Patients' compliance was 100% (range, 61-100). The presence of surgery complications (29.6%) produced 5% loss in compliance to treatment; likewise, chemotherapy treatment with prevalent use of ifosfamide reduced compliance to rehabilitation by 6%. The mean patient satisfaction score was 7.9 in the Likert scale from 0 to 10. Intensive physiotherapy starting during chemotherapy administration is a feasible treatment for bone tumour patients that have shown to be able to positively adhere to it. Rehabilitation treatments, within chemotherapy wards, should therefore be promoted according to satisfaction level as reported by patient.

Sarcoma de Ewing axial. A propósito de un caso / Axial Ewing's sarcoma. A case report

El sarcoma de Ewing (SE) axial es un tumor óseo y de tejidos blandos maligno, raro, con una tríada clínica característica de dolor, déficit neurológico y masa palpable. Típicamente presenta la translocación cromosómica t(11;22)(q24;q12). El tratamiento habitual del SE combina cirugía, radioterapia y quimioterapia. Se presenta el caso de una paciente de 40 años con clínica de lumbalgia aguda y paraparesia progresiva, que en el estudio radiológico realizado se objetiva una tumoración con morfología de reloj de arena, de localización primaria vertebral y sin metástasis, por lo que fue intervenida quirúrgicamente, y posteriormente realizó tratamiento quimioterápico y rehabilitador. Destacamos la importancia del caso clínico por la rareza de su edad de presentación y la localización vertebral primaria (AU)

Axial Ewing's sarcoma (ES) is a rare and malignant bone and soft tissue tumor, with a characteristic clinical triad of pain, neurological deficit and palpable mass. It typically presents the chromosome translocation t(11;22)(q24;q12). Its usual treatment combines surgery, radiotherapy and chemotherapy. A case report is presented of a 40-year old female patient with symptoms of acute low back pain and progressive paraparesis. The MRI study showed lumbar tumor with hourglass morphology, having a primary spinal location without metastases. The patient was operated on, after which chemotherapy and rehabilitation treatment were performed. We emphasize the importance of the clinical case due to its rarity of age of presentation and the primary spinal location (AU)

Calidad de vida y funcionalidad en pacientes pediátricos intervenidos de tumores óseos en miembros inferiores: cirugía reconstructiva versus amputación / Quality of life and functionality in pediatric patients operated on for lower limb bone tumors: limb salvage surgery versus amputation

Objetivo. Analizar las diferencias en cuanto a funcionalidad y calidad de vida relacionadas con la salud en pacientes intervenidos de un tumor óseo maligno en edad infantil según el tipo de cirugía practicada. Material y método. Estudio descriptivo transversal de pacientes mayores de 14 años tratados por osteosarcoma o sarcoma de Ewing en miembro inferior y que recibieron tratamiento quirúrgico en edad infantil tras una evolución mínima de 2 años. Se realizó una evaluación de funcionalidad y calidad de vida utilizando las escalas de Enneking y Medical Outcomes Study Short-Form 36. Comparamos los resultados funcionales según la técnica quirúrgica. Resultados. Nuestra muestra quedó formada por 17 pacientes (8 hombres y 9 mujeres) con una edad media actual de 22 años. El tumor más frecuente fue el osteosarcoma, y la localización, el tercio distal del fémur y proximal de la tibia. En relación a la técnica quirúrgica, en 8 pacientes se colocó endoprótesis, 4 se trataron mediante injerto y/u osteosíntesis y 5 fueron amputados en cirugía inicial. Cinco pacientes tuvieron que ser amputados en un segundo momento debido a complicaciones. La puntuación expresada en mediana y percentiles obtenidos en la escala de Enneking sobre la totalidad de la muestra fue del 83,33% (66,66-88,33%). En pacientes no amputados fue del 83,33% (73,33-86,66%), frente al 90% (80-93,33%) en pacientes amputados inicialmente. En los que se realizó amputación posterior tras complicación, la puntuación fue de 56,66% (51,66-73,33%). Conclusiones. En nuestra muestra no encontramos diferencias significativas en cuanto a funcionalidad y calidad de vida de los pacientes tratados mediante cirugía reconstructiva en comparación con amputación inicial, aunque sí peores resultados en los pacientes que sufren una amputación tardía debido a una complicación tras cirugía de salvamento inicial (AU)

Objective. To analyze the differences in functionality and quality of life related with outcome of a malignant bone tumor intervention in childhood age according to type of surgery performed. Material and methods. A descriptive, cross-sectional study of patients over 14 years treated for osteosarcoma or Ewing's sarcoma in lower limb during childhood after a minimum of two years evolution. Functionality and quality of life were evaluated with the Enneking and Medical Outcomes Study Short-Form 36 scales. The functional results were compared according to surgical technique. Results. Our sample consisted of 8 men and 9 women, with a current average age of 22 years. The most common type of tumor was osteosarcoma and most common location was distal third of the femur and proximal tibia. In relation to the surgical technique, stent was placed in 8 patients, 4 were treated by graft and/or osteosynthesis and 5 patients underwent amputation as initial surgery. Five more patients had to under amputation at a second time due to complications. The score, expressed as median and percentiles obtained in the scale of Enneking on the entire sample, was 83.33% (66.66%-88.33%). In non-amputees, it was 83.33% (73.33%-86.66%), compared with 90% (80%-93.33%) in patients who were initially amputees. In those in whom the amputation was performed later after a complication, the median Enneking score was 56.66% (51.66%-73.33%). Conclusions. In our sample, we did not find significant differences in terms of functionality and quality of life of patients treated by reconstructive surgery compared with initial amputation although there were worse results in those patients who underwent a later amputation due to a complication after the initial life-saving surgery (AU)

Quality of life in young patients after bone tumor surgery around the knee joint and comparison with healthy controls.

BACKGROUND: This study aimed to compare the health related quality of life (HRQoL) of children and adolescents after malignant bone tumor surgery of the leg with healthy controls. PROCEDURE: Patients between 8 and 25 years old were cross-sectional recruited. Patients under 16 years of age received the TNO (Netherlands Organization for Applied Scientific Research) and AZL (Leiden University Medical Center) Children's Quality of Life Questionnaire (TACQOL), patients aged 16 years and older received the TNO-AZL Questionnaire for Adult's Quality of Life (TAAQOL) and the Short Form-36 (SF-36). Three age- and sex-matched normative random samples, drawn from large, nationwide studies, were used for the comparison with healthy controls. Patients were interviewed regarding their most important problems related to the disease and its treatment. RESULTS: Eighty-one patients with a mean age of 16.9 years (SD 4.2) were included (41 female). Limb sparing surgery was executed in 38 patients, ablative surgery in 43 patients. In comparison with healthy controls, patients had significantly poorer HRQoL within the domains autonomy and motor function of the TACQOL, gross motor function, cognitive functioning, daily functioning and sexuality of the TAAQOL, and physical functioning, role physical, general health, and the physical and mental component summary scales of the SF-36. Patients reported limitations in physical activities, participation in sports, and cosmetic aspects as the most detrimental consequences of their disease and its treatment. CONCLUSION: In children and adolescents who underwent surgery for a malignant tumor of the leg physical, functioning was significantly impaired as compared to healthy controls.

Assessment of health-related quality of life after bone cancer in young people: easier said than done.

BACKGROUND: Improved survival rates coupled with awareness of physical and psychological late-effects have resulted in calls to consider the health-related quality of life (HRQOL) of survivors of childhood cancer. Survivors of bone tumours (osteosarcoma and Ewing's sarcoma) may be more vulnerable to compromised HRQOL than survivors of other cancers given their poor physical functioning. METHOD: Current research is reviewed in relation to (i) HRQOL following a bone tumour compared with the healthy population and other child cancers and (ii) between those treated by amputation or limb salvage. RESULTS: Limitations of current research include (i) measurement of HRQOL; (ii) reliance on single informants, usually mothers and (iii) research design. In the process of adjustment to disease, patients reassess the meaning, value and importance of different domains, so that decisions about HRQOL are based on changing standards over time. These 'response shifts' challenge the validity of both cross-sectional and longitudinal research designs. CONCLUSIONS: We conclude that methodological difficulties underlying previous work account for some of the discrepancies apparent in the current literature and challenge understanding of the complex processes of adjustment following a bone tumour.

Self-reported global function among adult survivors of childhood lower-extremity bone tumors: a report from the Childhood Cancer Survivor Study (CCSS).

INTRODUCTION: Adult survivors of childhood lower-extremity bone tumors may experience physical and psychosocial late effects that impact physical performance, global function and quality of life. The identification of survivors at greatest risk for poor outcomes will inform potential intervention targets. METHODS: Study participants were selected from the Childhood Cancer Survivor Study (CCSS), a multi-institutional study of childhood cancer survivors. Adult survivors (n = 629) of either childhood onset osteosarcoma or Ewing's sarcoma, with a primary tumor location in the lower-extremity were identified and contacted via mail to complete an additional questionnaire. Participants completed the Reintegration into Normal Living Index (RNL) to evaluate global function (maximum score of 22), daily function (maximum score of 16) and self perception (maximum score of 6). RESULTS: Survivors reported high levels of global function with an adjusted mean overall RNL index score of 20.6 (SE 0.14), mean daily function score of 15.0 (SD 0.10) and mean self perception score of 5.6 (SE 0.05). While female gender and increasing age were associated with lower RNL scores, the magnitude of difference is of questionable clinical significance. Global function was only moderately correlated with physical performance (r = 0.56) and QOL (r = 0.59). DISCUSSION: Based upon the RNL index, the vast majority of long-term survivors of childhood lower extremity bone tumors adapt well to their environment. IMPLICATIONS FOR CANCER SURVIVORS: While some long-term survivors of lower-extremity bone tumors may report measurable limitations in physical performance and quality of life, the majority do not report moderate or severe difficulties with social integration.

Avaliação funcional dos pacientes portadores de sarcomas ósseos submetidos à tratamento cirúrgico utilizando a endoprótese total ou parcial, na substituição da extremidade distal do fêmur / Functional assessment of patients with bone sarcomas submitted to surgical treatment using total or partial prosthesis in replacement of the distal femoral end

OBJETIVO: O Osteosarcoma e o Sarcoma de Ewing são as principais neoplasias malignas primárias ósseas, que acometem indivíduos menores de 15 anos. O objetivo deste estudo é comparar, retrospectivamente, os resultados funcionais dos pacientes submetidos à ressecção da extremidade distal do fêmur e à reconstrução com endoprótese não convencional, total ou parcial, do joelho. MÉTODOS: Foram analisados 26 pacientes portadores de sarcomas ósseos da extremidade distal do fêmur, acompanhados no Centro Infantil Boldrini, no período de 1990 a 2003. Vinte e quatro eram portadores de Osteossarcoma e 2 de Sarcoma de Ewing. O sistema de avaliação foi o proposto por Enneking (1987), preconizado pela Musculoskeletal Tumor Society. Para a comparação das médias entre cada critério e também entre os escores finais, utilizou-se o teste de Wilcoxon, com erro alfa de 5 por cento. RESULTADOS: A idade variou de 5 a 17 anos, média=11,9 anos. A predominância foi no sexo feminino (61,5 por cento). Na avaliação funcional, a comparação entre as médias de cada critério, foi encontrada diferença estatisticamente significativa somente relacionada ao item estabilidade (p=0,0037). Nos demais critérios, não foi observado diferença estatisticamente significativa: movimento (p=0,7546), dor (p=0,4848), deformidade (p=0,8695), força (p=1,0000), atividade funcional (p=0,9127) e resultado funcional (p=0,5866). CONCLUSÕES: O escore final global da avaliação funcional não apresentou diferença estatisticamente significativa (p=0,6027). O tipo de endoprótese utilizado para reconstrução do fêmur não interferiu nos resultados funcionais dos pacientes.

OBJECTIVES: Osteosarcoma and Ewing's sarcoma are the most common malignant primary bone tumors in individuals under the age of 15 years. The purpose of the study is to retrospectively compare functional outcomes of patients submitted to resection of the distal femoral end and to reconstruction with total or partial non-conventional prosthesis of the knee. METHODS: We assessed 26 patients with bone sarcomas in the distal femoral end treated at the Boldrini's Children Center between 1990 and 2003. Twenty-four presented with Osteosarcoma and two had Ewing's sarcoma. The assessment system employed was the one proposed by Enneking (1987) as recognized by the Musculoskeletal Tumor Society. For the statistical analysis between the criteria and final scores, we have used the Wilcoxon test, with an alpha error of 5 percent. RESULTS: Ages ranged from 5 to 17 years, (mean: 11 years and 9 months); with prevalence being higher in females (61.5 percent). The only statistically significant difference found in this study was concerned to stability (p=0.0037). No statistical significance was found on any other criteria such as movement (p=0.7546), pain (p=0.4848), deformity (p=0.8695), strength (p=1.0000), functional activities (p=0.9127) and final functional outcome (p=0.5866). CONCLUSIONS: The overall functional end score did not show statistically significant differences (p=0.6027). The type of prosthesis for femoral reconstruction did not affect patients' functional outcomes.

Endoprosthetic reconstruction for malignant bone and soft-tissue tumors.

BACKGROUND: Nowadays, the results of the management of malignant bone and soft-tissue tumors have been dramatically improved because of the advance in imaging, chemotherapy, radiation therapy, and surgical techniques. Patients can have longer survival times with limb-salvage surgery. Several techniques of reconstruction have been advocated and gained more popularity following malignant tumor resection by using allograft, tumor prostheses, composite allograft prosthesis, or arthrodesis. OBJECTIVE: To report the preliminary results of 32 endoprosthetic reconstructions following malignant bone and soft-tissue tumor resection. The oncologic results, functional outcomes, and complications from the surgery were assessed in the present study. MATERIAL AND METHOD: Since September 1988, the authors have performed 188 limb-salvage surgical operations for the treatment of musculoskeletal tumors at Siriraj Hospital. From March 1994 to July 2006, 32 endoprosthetic reconstructions were performed on 30 patients following malignant bone or soft-tissue tumor removal. There were 16 males and 14 females with a mean age of 28 years (range 10-73). The diagnosis was conventional osteosarcoma in 16 patients, parosteal osteosarcoma in two patients, chondrosarcoma in two patients, leiomyosarcoma in two patients, failed allograft in two patients and one patient each of periosteal osteosarcoma, Ewing's sarcoma, Gorham's disease, synovial sarcoma, malignant fibrous histiocytoma, metastatic renal cell carcinoma, and prosthetic loosening. Wide excision was performed with a mean length of 18.5 cm (range 10-41). Five proximal femurs, 17 distal femurs, 1 total femur 3 proximal tibias, 1 intercalary tibia, 4 proximal humerus and 1 distal humerus were used for reconstruction. Modular replacement systems (MRS, Stryker/Howmedica/Osteonics) were the most common prostheses used in the present series. RESULTS: The mean follow-up time was 26 months (range 6-128.7). Sixteen patients are continuously free of the disease, two are alive with the disease, two had no evidence of the disease, nine died of the disease, and one patient died from complication of hypertension. The mean Musculoskeletal Tumor Society functional analysis for upper extremity reconstruction was 93% (range 86.7-100) and for lower extremity was 89% (range 63.3-100). Two patients (6.7%) were determined to be a failure. Revision due to aseptic loosening was performed in one patient (3.3%) and one hip disarticulation was done related to local recurrence (3.3%). One patient with sciatic nerve palsy and two seromas was found and successfully treated in the present study. CONCLUSION: Endoprosthetic reconstruction could yield satisfactory results as a wide excision and limb-salvage for patients with malignant bone and soft-tissue tumors. Most patients in the present report had good to excellent functions following surgery and few complications occurred in the present report.

Ewing's sarcoma of the pelvis: combined surgery and radiotherapy treatment.

BACKGROUND AND OBJECTIVES: Local therapy of pelvic Ewing's sarcoma remains a challenge because of the complex anatomy and large tumor volumes. Wide resections often end up contaminated with marginal margins in one part of the specimen. Pelvic tumor surgery causes considerable disability, because of the loss of hip function. This may cause a low quality of life for tumor survivors. The aim of the study was to evaluate the oncologic results of combined surgery and radiotherapy of pelvic Ewing's sarcoma. Functional and psychosocial outcome of the tumor survivors was assessed. METHODS: Thirty-six pelvic Ewing's sarcoma patients were treated surgically for local tumor control between 1986 and 1996. Chemotherapy was administered according to current European Intergroup/Cooperative Ewing's Sarcoma Study (EI/CESS) protocols. Radiotherapy was also performed on all patients. All patients were intended to get wide tumor excision and a limb salvage procedure. Complications necessitating operative revision were analyzed and functional results were scored according to the criteria of the Musculoskeletal Tumor Society (MSTS). Quality of life and psychosocial functioning was assessed by the QLQ-C 30 of the European Organization for Research and Treatment of Cancer (EORTC). RESULTS: The overall 5- and 10-year survival rate was 45%. The prognosis of patients with primary metastases was 33%. There were two local recurrences after marginal resection. Every second patient needed at least one surgical revision. A secondary hindquarter amputation was avoided in all patients. MSTS functional evaluation instruments reached 59% (+/-21.0%). Global quality of life of the QLQ-C 30 showed good results (70 +/- 16%) compared to the general population (75 +/- 24%). CONCLUSIONS: The advantages of surgical local control of pelvic Ewing's sarcoma should be offered to the patient. Quality of life measurement after internal hemipelvectomy shows that the life of these patients is more than worth living and that they are able to compensate the functional impairment.

Vertical distraction osteogenesis of fibular free flap in mandibular prosthetic rehabilitation: a case report.

A 17-year-old boy underwent three cycles of chemotherapy and a subsequent mandibular resection for a Ewing's sarcoma of the left body and ramus. The mandible was immediately reconstructed with a microvascular osteomuscular fibular flap. One year after the mandibular reconstruction, distraction osteogenesis of the anterior portion of the fibula was performed using a Martin distractor according to the Hoffmeister technique. Bone lengthening was achieved at a rate of 1 mm/day by turning the device twice each day for 12 days. Subsequently, we waited for 70 days for bone consolidation to occur. After 6 additional weeks, five Maestro implants were placed into the distracted fibula. Bone specimens were retrieved with a trephine bur during implant placement. Mature bone was present after 70 days and after 6 months. The bone height increase was 12 mm.

Gait analysis in patients after Van Nes rotationplasty.

Gait was analyzed through kinetic, kinematic, and temporal-distance parameters assessment to evaluate the functional performance of ten children treated by the classical Van Nes rotationplasty (VNR). Van Nes rotationplasty is a surgical procedure, which permits the conversion of the ankle joint into a "pseudo-knee" joint. The data were compared with measurements of a normal population of ten healthy subjects. Significant differences with respect to the normal were found in stride duration, stride length, cadence, velocity, and stance-swing ratio, and in ground-reaction forces parameters, which define the propulsive phase in the prosthetic side and the acceptance phase in the sound side. There were differences in some kinematic parameters as well. Van Nes rotationplasty is acceptable from a biomechanical-functional point of view, particularly with respect to the active control of the pseudo-knee, which allows a smooth and coordinate gait pattern.

Assessment of patient function after limb-sparing surgery.

Cancer rehabilitation is becoming more of a focus for the field of physiatry due to increased longevity and the side effects of treatment. In order to investigate the rehabilitation needs of patients undergoing limb-sparing procedures, chart analysis was conducted on 17 children treated for primary bone tumors by resection and an expandable endoprosthetic replacement. Each patient underwent a course of postoperative inpatient and outpatient physical therapy and was followed over an average of 2.5 years. Gait training was relatively straightforward and in seven patients required neither orthosis nor ambulatory aid. The other ten patients walked with a knee orthosis, axillary crutches, or both. Until the time came for reoperation to lengthen the implant, a shoe lift of 1in maximum was added to compensate for the limb length discrepancy. These findings compare favorably with the more complex requirements of high proximal amputees with external prostheses, including more difficult gait training and the need for frequent adjustments, as well as prosthetic replacement as the children grow. It is clear that children undergoing limb-sparing surgery have special needs that should be addressed, including early mobilization, gait training, adjustment to repeated brief hospitalizations for lengthening, and continued follow-up to monitor their activity restriction.

[Orthopedic rehabilitation of children after combined treatment of malignant bone tumors]. / Ortopedicheskaia reabilitatsiia detei posle kombinirovannogo lecheniia zlokachestvennykh opukholei kostei.

The report is based on the data concerning 27 children who were given radiotherapy and chemotherapy for osteogenic sarcomas (14), Ewing's sarcomas and reticulosarcoma (10) and malignant giant cell tumours (2). The reconstructive conservative treatment (7 patients) were referred for prevention of contractures and fractures. The orthopaedic surgical correction of the appearing false joints, deformations and defects of the bones (20 patients) was performed 4 to 5 years after the termination of the primary treatment. During the reconstructive period 3 patients with malignant giant cell tumours and osteogenic sarcoma died because of metastases. The authors have made a conclusion about the advantage of extrafocal compression and distraction osteosynthesis over other methods of fixation and correction of deformations in children after the treatment of malignant bone tumours.