The unmet needs of patients with sarcoma.

OBJECTIVE: Sarcoma is a rare cancer that imposes a significant burden on the lives of patients. Many survivors have long-term disability as a result of treatment and the disease. Patients often experience functional issues, poorer mental health, reduced quality of life, and interpersonal issues. There is a need to explore the unmet needs of sarcoma patients as it is unclear how these issues are being addressed. The aim of this study was to explore the unmet needs of patients who have been diagnosed with sarcoma. METHODS: The participants were individuals previously diagnosed with a sarcoma (n = 22). Participants completed a semi-structured interview about their unmet needs relating to sarcoma. The transcripts were analyzed using thematic analysis. RESULTS: Five overarching themes were identified: daily living, financial needs, lack of information, need for a community, and navigating the healthcare system. Participants reported a range of practical needs, including transport, limited understanding of their treatments, and financial issues. Participants also described a need to connect with people who had gone through similar experiences. CONCLUSIONS: Sarcoma patients have fundamental needs that affect their capacity to live their life in the best possible way. Health services need to better facilitate sarcoma patients at each stage of their experience by providing individualized referrals, support, and coordination. Support interventions are needed to help patients adjust to sarcoma and to improve their quality of life as an outpatient. Connecting patients with sarcoma support groups may provide them with more relevant and intimate support.

[Physiotherapy in early rehabilitation of patients with bone sarcomas after arthroplasty of large bones and joints]. / Fizioterapiya pri rannei reabilitatsii bol'nykh s kostnymi sarkomami posle endoprotezirovaniya krupnykh kostei i sustavov.

ACTUALITY: The modern method of treating patients with tumor lesions of the bones is the replacement of large bones and joints with the inclusion of reconstructive plastic component. The main functional postoperative disorders of this method (limitation of mobility in the operated joint, pain, muscle hypotrophy and edema of the operated limb) obstruct the ability of patients to return to a full life, which makes their early rehabilitation extremely urgent. Orthopedics uses a number of physical factors for this. The literature presents data on the absence of negative effects of low-frequency electro- and magnetotherapy in the treatment of late complications of oncopedic surgery on a number of bone sarcomas, which allowed them to be included in early rehabilitation for the first time. AIM OF STUDY: Development of a complex of early rehabilitation of patients with bone tumors after arthroplasty of large bones and joints and evaluation of its effectiveness. MATERIALS AND METHODS: A prospective, open, nonrandomized, controlled study included 36 patients aged 19-67 years (Me 42 years old) with primary malignant and metastatic bone tumors who underwent endoprosthesis replacement of large bones and joints with the inclusion of reconstructive plastic component (plastic by displaced muscles, synthetic mesh) and received local low-frequency magnetotherapy, low-intensity infrared laser radiation, electroneuromyostimulation and therapeutic exercises. Physiotherapy began from the first day after the operation and lasted for 10 days. To assess the functional result, the international MSTS scale was used. RESULTS: The combined use of the reconstructive plastic component during radical surgery and physiotherapy made it possible to obtain good functional result at 63,9% of patients, satisfactory functional results in 36.1% of patients according to the MSTS scale at the time of discharge. The function of the saved limb in 10 patients after distal femoral resection with knee replacement was 80% of normal function, in 7 patients after proximal tibia resection - 72%; in 13 patients after proximal resection of the femur with hip replacement - 59%; in 5 patients after proximal resection of the humerus with endoprosthetics of the shoulder joint - 61.3%; and in 1 patient after proximal resection of the ulna with endoprosthetics of the elbow joint - 70%. CONCLUSION: The multidisciplinary approach to the early rehabilitation of patients with bone tumors made it possible to achieve functional results in a short time, reduce the time spent by patients in the surgical department without increasing the number of postoperative complications. Based on the preliminary obtained results, it is necessary to continue the study on a larger number of patients and with a longer follow-up period.

Targeted muscle reinnervation in oncologic amputees: Early experience of a novel institutional protocol.

BACKGROUND: We describe a multidisciplinary approach for comprehensive care of amputees with concurrent targeted muscle reinnervation (TMR) at the time of amputation. METHODS: Our TMR cohort was compared to a cross-sectional sample of unselected oncologic amputees not treated at our institution (N = 58). Patient-Reported Outcomes Measurement Information System (NRS, PROMIS) were used to assess postamputation pain. RESULTS: Thirty-one patients underwent amputation with concurrent TMR during the study; 27 patients completed pain surveys; 15 had greater than 1 year follow-up (mean follow-up 14.7 months). Neuroma symptoms occurred significantly less frequently and with less intensity among the TMR cohort. Mean differences for PROMIS pain intensity, behavior, and interference for phantom limb pain (PLP) were 5.855 (95%CI 1.159-10.55; P = .015), 5.896 (95%CI 0.492-11.30; P = .033), and 7.435 (95%CI 1.797-13.07; P = .011) respectively, with lower scores for TMR cohort. For residual limb pain, PROMIS pain intensity, behavior, and interference mean differences were 5.477 (95%CI 0.528-10.42; P = .031), 6.195 (95%CI 0.705-11.69; P = .028), and 6.816 (95%CI 1.438-12.2; P = .014), respectively. Fifty-six percent took opioids before amputation compared to 22% at 1 year postoperatively. CONCLUSIONS: Multidisciplinary care of amputees including concurrent amputation and TMR, multimodal postoperative pain management, amputee-centered rehabilitation, and peer support demonstrates reduced incidence and severity of neuroma and PLP.

Gait analysis in tumor patients after distal femoral resection and implantation of a megaprosthesis.

We analysed nine patients who had had a megaprosthesis implanted into the distal femur and knee joint for treatment of sarcomas. Data obtained from the leg operated on were compared with those from the contralateral side and healthy volunteers. Gait data, kinematics, ground reaction forces and the EMG from five muscles around the knee joint were analysed by means of a video-based analysis system recording data from reflection markers ; a Kistler-plate recorded the GRF and a ten channel surface EMG the muscle activity. Muscle around the knee showed a cocontraction between the extensor and flexor muscles in the thigh and the calf in the operated leg as well as contralaterally. Gait characteristics exhibited a reduced speed, -cadence, and a shorter step. This correlated with a reduced flexion in the hip and knee joint. The GRF exhibited significant changes in the data representing the reduced gait dynamic.

Rehabilitation of the sarcoma patient-enhancing the recovery and functioning of patients undergoing management for extremity soft tissue sarcomas.

Although relatively rare, soft tissue sarcomas cause significant morbidity and mortality due to their advanced stage at initial diagnosis. Rehabilitation and surgical outcomes have traditionally focused on physical parameters to assess function and recovery, emphasizing return to ambulation, activities of daily living (ADLs) and community re-integration. Assessments of functional impairment and other quality-of-life parameters are necessary to better understand the experience of the patient with extremity soft tissue sarcoma and thereby improve outcomes.

Development of reference values for the Functional Mobility Assessment.

PURPOSE: To obtain reference values for healthy participants performing the Functional Mobility Assessment (FMA). The FMA is a reliable and valid tool used to examine functional abilities subjectively and objectively in patients with lower-extremity sarcoma in all components of the International Classification of Functioning, Disability, and Health model (body function, activity, and participation). METHODS: Children, adolescents, and young adults who were healthy, representing a sample of convenience (n = 503; 260 females, age 10-21 years), participated in this study. RESULTS: Means and standard deviations for all participants: Timed Up and Down Stairs 6.18 ± 0.8 seconds, Timed Up and Go 3.78 ± 0.6 seconds, 9-minute run/walk 4161 ± 893 feet, and the FMA total 59 ± 3. CONCLUSION: The reference values provided in this study will allow health care professionals to compare the functional abilities of children, adolescents, and young adults with lower extremity sarcoma to age- and gender-matched healthy peers when using the FMA.

Sports activity after soft tissue sarcoma of the lower extremity.

Introduction: The aim of this case series was to assess sports activity levels in long-term survivors of soft-tissue sarcomas after multimodal treatment including limb salvaging surgical resection and radio-chemotherapy.Methods: Thirty-two patients (17 f/15 m) with a mean age of 29 (range 10-44) years at the time of diagnosis and a mean follow-up time of 9 (range 3-21) years following sarcoma were included. Ten patients had been diagnosed with liposarcoma, seven with synovial sarcoma, four with fibrosarcoma, three with undifferentiated pleomorphic sarcomas and the remaining eight patients with different soft-tissue sarcoma entities. Sarcoma location of twenty-four (75%) patients was in the thigh, eight (25%) in the lower leg. Eleven (34%) tumors were located epifascial, 21 (66%) in deeper tissue layers. Sports activity was measured by the University of California, Los Angeles (UCLA) Activity Score.Results: One year before treatment 30 patients (94%) and >3 years post-treatment 29 patients (91%)had been pursuing athletic activity regularly. They were performing 5.5 h/week prior and 4.2 h/week >3 years post surgery. The mean UCLA score in case of subfascial sarcomas was 8.0 and thereby lower (p < 0.05) than those of epifascial sarcomas, which were 9.3. Furthermore, the mean UCLA score after subfascial sarcoma resection 3 years postoperative was still below preoperative UCLA levels (p < 0.05), whereas patients after epifascial tumor resection showed no loss of sports activity. No relation between complications and postoperative sports activity level was found.Conclusions: Healthy long-term survivors can achieve high levels of sports activity following limb salvage after soft-tissue sarcomas. The concerned muscle groups may influence the functional and sports outcome and draw attention to this topic in future rehabilitation. This knowledge may be of high value for patients counseling, physicians and orthopedists treating patients, as well as for patients inquiring information regarding post-treatment activity levels.Implication for rehabilitationSport is possible after soft tissue sarcoma treatment and longer-term rehabilitation which is usually three yearsRehabilitation is best undertaken in specialized centersIt is important to maintain contact with surgeons in order to optimize mobilizationHigh impact sports may be affected by deep muscle resections.

Functional outcomes after limb-salvage surgery and endoprosthetic reconstruction with an expandable prosthesis: a report of 4 cases.

OBJECTIVE: To determine the functional outcomes of skeletally immature patients after replacement of the femur and tibia performed by using noninvasive expandable endoprostheses. DESIGN: Case series. SETTING: A hospital-based ambulatory care center. PARTICIPANTS: Pediatric patients (N=4) with primary bone tumors of the distal femur and proximal tibia who underwent surgical replacement performed by using the Repiphysis noninvasive expandable endoprosthesis (Wright Medical Technology, Memphis, TN). INTERVENTIONS: Wide resection of bone sarcoma and placement of expandable endoprosthesis. MAIN OUTCOME MEASURES: Musculoskeletal Tumor Society (MSTS) scores were assessed at the beginning of the study and at each follow-up visit. Medical Outcomes Study 36-Item Short-Form Health Survey, Version 2 (SF-36); gait; sit-to-stand transition; and range of motion (ROM) were assessed at an average follow-up of 31.5 months. RESULTS: At an average of 31.5 months postoperative, the SF-36 physical component summary scores lagged behind the national mean, whereas the mental component summary scores were satisfactory. MSTS scores indicated low levels of pain and supports use with high emotional acceptance and walking ability but persisting difficulties with function and gait. Patients also showed altered patterns of sit-to-stand transition including decreased peak vertical force in the operated limb and increased center of mass momentum in a shorter amount of time. Parts of gait functioning were found to be decreased, including gait velocity, stride length, and cadence. Some patients displayed alternate weight-bearing strategies that accompanied increased double-limb support and stance phase during walking. ROM and strength were diminished at both the hip and knee joints in the operated limb and in the nonoperated limb. CONCLUSIONS: Reconstruction with a noninvasive expandable endoprosthesis produces satisfactory functional outcomes in pediatric patients with primary tumors of the bone. Patients in our study displayed some persisting physical difficulties including decreased ROM and strength and altered gait and sit-to-stand patterns, yet they maintained high levels of emotional acceptance and coping.

Prognostic factors for adult sarcomas of head and neck.

The optimal management of adult soft-tissue sarcomas is not clearly established. To assess prognostic factors and survival, the experience of 45 recent successive cases was reviewed. Data were collected from a retrospective database (1993-2005) and statistically analyzed. Rhabdomyosarcomas were excluded. The mean age was 50.1 years; there were 24 men and 21 women. The main histological subtypes were undifferentiated sarcoma (14) and angiosarcoma (10); 21 tumours were grade 3 (46%). The most frequent primary sites were neck muscles (15, 33%) and scalp (11, 24%). At presentation, 5 (20%) cases with lymph-node involvement and another 11 cases (24%) with distant metastasis were observed. The treatment was with curative intent in 33 cases (73%). This entailed surgery, with adjuvant radiotherapy in 15 cases and adjuvant chemotherapy in 5 cases. The 5-year overall survival was 52% (+/-8%). In univariate analysis, the poor prognostic factors were high grade, initial metastasis or lymph nodes, absence of surgery, and number of surgical procedures. In multivariate analysis, two factors remained significant: grade (P=0.006) and absence of surgery (P=0.005). After taking into account grade and metastasis at presentation, quality of surgery has prognostic value. The primary aim of a multidisciplinary approach to these tumours must favour complete resection.

Construct validity of the Enneking score for measuring function in patients with malignant or aggressive benign tumours of the upper limb.

We evaluated the construct validity of the Musculoskeletal Tumour Society rating scale (Enneking score) as a functional measure for patients with sarcoma involving the upper limb. We compared the Enneking score by examining the correlation between two patient-derived outcome measures, the Disability of the Arm, Shoulder, and Hand (DASH) questionnaire and the Medical Outcomes Study Short Form-36 (SF-36) as indicators of functional status in 40 patients with malignant or aggressive benign bone and soft-tissue tumours of the upper limb who had undergone surgical treatment. The frequency distributions were similar among the three scoring systems. As for the validity, Spearman's rank correlation coefficient of the Enneking score to the DASH questionnaire was -0.79 and that of the Enneking to the SF-36 subscales ranged from 0.38 to 0.60. Despite being a measure from the surgeon's perspective, the Enneking score was shown to be a valid indicator of physical disability in patients with malignant or aggressive benign tumours of the upper limb and reflected their opinion.

Issues of Survivorship and Rehabilitation in Soft Tissue Sarcoma.

As the number of survivors of extremity soft tissue sarcoma increases, so does the need to understand the experience of survivors and develop measures, systems and services that support rehabilitation into normal life roles. Survivorship includes considerations of the physical, psychological and social domains, of which the physical sequelae of treatment are the best characterised in the literature. The survivorship experience may include disability, pain, lymphoedema, psychological problems, as well as difficulty with employment, relationships and lower quality of life. Rehabilitation strategies for extremity sarcoma patients must be personalised, holistic and begin early in the pathway, ideally before the first treatment intervention. The International Classification of Functioning, Disability and Health model is a useful framework for combining assessments, including objective outcome measures, which can be combined into a rehabilitation prescription. Research is needed to develop an evidence base for rehabilitation interventions to support patients with extremity soft tissue sarcoma.

Rehabilitation Strategies and Outcomes of the Sarcoma Patient.

Sarcomas are a relatively rare cancer that, depending on the location, can cause significant neuromusculoskeletal dysfunction and require rehabilitation interventions to reduce pain, restore function, and improve quality of life. This review focuses on sarcoma subtypes that frequently cause these complications: bony and soft tissue sarcomas leading to limb salvage or amputation, desmoid tumors, and malignant peripheral nerve sheath tumors. Rehabilitation approaches and outcomes are discussed, as well as considerations for childhood sarcoma survivors transitioning to adulthood.

Reconstruction after musculoskeletal sarcomas: how to avoid a surgical trap.

BACKGROUND: Musculoskeletal sarcomas comprise 1% of all malignancies in adults. Unfortunately, sometimes they are addressed in non-appropriate way requiring a more invasive procedure to achieve radical surgery at a later date. Due to incomplete predictability of their extension, scheduled reconstruction cannot be performed at times, forcing plans to change or clogging up immediate reconstruction. In this paper, the authors provide an insight in the treatment of musculoskeletal sarcomas, particularly focusing on the preoperative planning of reconstructive strategies, which is crucial in order to prevent unpleasant surprises during reconstruction. METHODS: Fifty-six consecutive patients requiring reconstructive procedures following the extirpation of tumors were recruited. All data collected during the diagnostic phase were analyzed collectively during a multi-disciplinary meeting where the surgical procedure was planned. A score system was created and results were then classified into "excellent", "good", "sufficient" and "poor". RESULTS: After a minimum follow up of 12 months, we recorded the following results: excellent in 10 patients (17.9%), good in 28 patients (50%), sufficient in 12 cases (21.4%) and poor in 6 cases (10.7%). CONCLUSIONS: The improvement of treatment and the long-lasting survival in musculoskeletal sarcoma have shifted the goal of therapeutic protocol to obtaining radical tumor removal and maximum functional restoration. When facing unpredictable extension of the resections, reconstruction may be a challenging or even impossible task to fulfil. Only meticulous preoperative planning can prevent surgeons from falling into all sorts of surgical traps following wide resections.

Resection of the ilium in patients with a sarcoma: should the pelvic ring be reconstructed?

AIMS: The aim of the study was to investigate the controversial issue of whether the pelvic ring should be reconstructed following resection of the sarcomas of the ilium. PATIENTS AND METHODS: From our database, we identified 64 patients who underwent excision of a tumour involving the ilium between 1976 and 2015. A total of 35 underwent complete resection, of whom 24 were reconstructed with a non-vascularised fibula graft, and four with extracorporeal irradiation and reimplantation. A total of 29 patients had a partial resection. The mean follow-up was 9.2 years (1.1 to 25.6). Functional outcomes were assessed using the Toronto Extremity Salvation Score (TESS) at final follow-up. In all, 32 patients (50%) had a chondrosarcoma. RESULTS: The mean TESS for all patients was 71.6% (17% to 100%). The mean TESS for those who underwent total resection with reconstruction was 72.0% (17% to 100%) and without reconstruction it was 53.3% (20% to 90%) and for those who underwent partial resection it was 76.3% (31.3% to 100%). The rate of local recurrence was 42.2% and this was more common in those treated by partial resection (p = 0.048). The risk of local recurrence was related to the margin achieved at resection. CONCLUSIONS: Given the high rate of local recurrence following excision of a tumour from the ilium, obtaining wide surgical margins should be a priority even if this requires more aggressive surgery. In young patients, where late recurrence may occur, more radical complete resection should be considered. When total resection of the ilium is considered, reconstruction should also be considered as it confers a higher functional outcome than total resection without reconstruction. Cite this article: Bone Joint J 2017;99-B:538-43.

The introduction of early patient rehabilitation in surgery of soft tissue sarcoma and its impact on post-operative outcome.

BACKGROUND: Enhanced recovery after surgery (ERAS) programs are implemented in multiple fields of surgery, but not yet in soft-tissue sarcoma (STS) surgery. We wondered whether its introduction into STS surgery might have impacted postoperative outcome. METHODS: Two hundred and fifty seven adult patients with primary limb or trunk wall STS received ERAS from 2008 to 2012 as a part of the intra-operative management. We evaluated, in retrospect, the intra-operative management, post-operative outcomes, functional and oncological results of these patients and compared them with 459 prior patients treated under a standard recovery after surgery (SRAS) program from 1989 to 2007. RESULTS: The most visible change from SRAS to ERAS in the perioperative management was decrease of wound drainage (72% vs. 15%, p < 0.001) and increase of wound bandaging (16% vs. 66%; p < 0.001), underlining the appliance of the ERAS protocol. Post-operatively, hospital stay dropped from nine (0-74) to three (0-22) days (p < 0.001) without affecting major morbidity (8% vs. 5%, NS) or readmission to the hospital (5% vs. 4%, NS). Functional outcome improved (p = 0.009) but whether this change was due to ERAS remains to be proved because complementary treatments changed over time. Tumour control remained unaffected, with an estimated risk of local recurrence at 5 years of 12% in both groups. CONCLUSION: Introducing a rapid recovery program was associated with a shorter hospitalization stay without compromising surgical or oncological outcomes. The program appears to be safe and reliable to use in patients undergoing STS surgery.

Functional outcome in extremity soft tissue sarcoma.

The studies reporting functional outcome for patients undergoing limb preservation surgery for extremity soft tissue sarcoma (STS) have evaluated mainly impairments, that is, deficits at an anatomic structure level, such as joint range of motion and strength. Disability, activities of daily living, self-care, and mobility have been less frequently evaluated. Review of the literature suggests that approximately 50% of patients treated for STS have significant impairments, whereas the frequency of disability is less. Synthesis of the results is difficult because of the heterogeneity of patient samples, treatment, and the outcomes used to evaluate function. Future studies require the use of standardized definitions and reliable and valid functional outcome measures. Improved patient outcomes can be achieved only by understanding the determinants of these outcomes and by introducing interventions to improve patient functional outcome.

Limb function following conservation treatment of adult soft tissue sarcoma.

Quality of life and limb function were studied in 54 patients who were disease-free 2 or more years after limb-conserving treatment for soft tissue sarcoma of the leg or pelvic girdle. Tumours of the thigh predominated (25 patients) and the mean tumour size was 9.9 cm. 41 patients had been treated with a combination of surgery and radiotherapy (29 with conventional and 12 with high dose), 12 with surgery alone and one with irradiation and intra-arterial doxorubicin. Only 15 patients had a normal range of movement in all lower limb joints and only 12 had normal power in all muscle groups; tumours of the lower leg were particularly unfavourable in this respect. Gait was normal in 42 patients but 8 required a walking aid and 4 a joint support. 16 had detectable lymphoedema but only 2 needed to wear compression hosiery. 35 patients still experienced pain at some time but only 6 required analgesia. However, when assessed by questionnaire for locomotion, grooming and home/leisure/vocational activities, 37 patients (68%) reported excellent function, and only 2 had moderate impairment. Function loss was most marked in leisure (25 patients) and vocational (8) activities, but was mild in 66% of cases. Multivariate analysis was carried out to determine the prognostic factors for poor limb function. The results suggested that overall functional score was predominantly determined by gait (P less than 0.001), muscle power or range of movement (P less than 0.001), with increasing age, female sex and the use of radiotherapy poor prognostic factors. Reduced muscle power or range of movement were the major factors determining gait (P less than 0.02) with the use of radiotherapy the significant prognostic factor for both in the conventionally treated group. Doses in excess of 60 Gy resulted in increased fibrosis and a worse functional outcome. Extent of surgery was not an independent prognostic factor for limb function, although univariate analysis suggested an association with range of movement in the conventionally treated group (P less than 0.025). Despite significant objective loss of range of movement and muscle power patients retain excellent limb function and quality of life following limb conserving treatment. For optimal function, radiotherapy should be given with small fractions to a dose not exceeding 60 Gy.

Microvascular approach to function and appearance of large orbital maxillary defects.

Forty-three patients with major three-dimensional orbitomaxillary defects underwent 48 free-tissue transfers for defects arising from resection of a neoplasm or trauma. Defects were complex, involving the malar skin, mandible, lateral nasal wall, orbit, palate, and brain. A three-dimensional approach attempting to recreate the midface by folding the flaps was the usual method, with subsequent revisions if necessary. Ten patients had simultaneous free bone grafts and 6 had vascularized bone grafts. There were three flap failures and four perioperative deaths. Excluding the 4 deaths, the results were assessed as excellent in 25 patients, good in 12, and poor in 2. Free-tissue transfer is a reliable method of providing enough tissue at one initial operation to satisfy complex three-dimensional defects of the orbitomaxillary region. Followed by minor revision, it provides a means of restoring function and appearance without multiple staged procedures with a high risk of failure.