Sarcoma Survivors Research Articles

Fertility, Cardiac, and Orthopedic Challenges in Survivors of Adult and Childhood Sarcoma

The combination of cisplatin, doxorubicin, and methotrexate was established as the standard backbone of contemporary osteosarcoma therapy in 1986. Since then, however, further improving the survival of patients with osteosarcoma has been challenging-30% to 40% of patients with osteosarcoma still die of this disease. In addition, these patients often experience loss of fertility at a young age, short- and long-term treatment-related cardiotoxicity, and adverse orthopedic effects from surgical resection of the tumor or endoprosthetic reconstructions. Cancer treatment often markedly increases the risk of infertility later in life, causing many patients substantial distress and regret. Sperm banking and oocyte cryopreservation are standard of care and should be available to all at-risk patients. Newer techniques, such as autologous gonadal tissue transplant for prepubertal children, are being developed, and newer systemic agents have infertility risk profiles that remain undefined and warrant further study. Cost and access remain barriers to these options. The late effects of anthracycline-induced cardiotoxicity are also increasingly a problem for these patients. These effects are often progressive and can be disabling. Adding dexrazoxane to doxorubicin therapy significantly reduces the risk for most adverse cardiac outcomes without compromising the efficacy of induction chemotherapy. Limb salvage surgery remains the standard of care for treatment in the majority of patients with extremity sarcomas. Modular metal prostheses and allograft reconstructions comprised the majority of surgical procedures for limb salvage surgery. The most common mechanism of failure of these implants is infection and mechanical failure of the implant.

Second malignancy risk among pediatric, adolescent, and young adult survivors of fusion-positive and fusion-negative sarcomas: Results from the SEER database, 1992 through 2012.

The current study builds on the hypothesis that cancer-predisposing germline mutations are less common among patients with fusion-positive (F+) sarcomas compared to those with fusion-negative (F-) sarcomas, resulting in a lower risk of developing second malignant neoplasms (SMNs) in those with F + sarcomas. Standardized incidence ratios (SIRs) for developing SMNs were evaluated in 4822 survivors of F + and 3963 survivors of F- sarcomas that were diagnosed between 1992 and 2012 in pediatric, adolescent, and young adult patients (aged birth-39 years) and reported in the Surveillance, Epidemiology, and End Results (SEER) database. Cox proportional hazards models (adjusted hazard ratio [aHR]) and competing risk methods (subhazard ratio [sHR]) were used to evaluate SMN risk in those with F- versus F + sarcomas while controlling for demographic and clinical variables. SMN risk was found to be nearly 2-fold greater among survivors of F + sarcomas (SIR, 1.86; 95% confidence interval [95% CI], 1.48-2.30) and nearly 3-fold greater among survivors of F- sarcomas (SIR, 2.89; 95% CI, 2.30-3.59) compared with the reference population. Although SMN types were noted to be similar between the fusion groups, the rate of any SMN was noted to be greater among survivors of F- sarcomas (aHR, 1.38 [95% CI, 1.01-1.89] and sHR, 1.27 [95% CI, 0.94-1.73]) when compared with survivors of F + sarcomas. The difference was most notable for solid tumor SMNs after index sarcomas were diagnosed between 2002 and 2012, for which rates of SMN were >2-fold greater among survivors of F- sarcomas (aHR, 2.31 [95% CI, 1.20-4.48] and sHR, 2.24 [95% CI, 1.13-4.43]). The findings of the current study highlight the increased SMN risk experienced by survivors of sarcoma and demonstrate higher SMN rates in survivors of F- sarcomas compared to those with a history of F + sarcomas. Cancer 2016;122:3492-3500. © 2016 American Cancer Society.

Establishing Guidelines for Safe and Effective Treatment of Pediatric Sarcoma Survivors: A Mission of the Pediatric Oncology Special Interest Group

Physical therapists have long been involved in the postoperative care of children with a diagnosis of bone and soft-tissue sarcoma. The International Classification of Functioning, Disability and Health model provides a guide to identify impairments, performance limitations, and participation restrictions in this population. The Pediatric Oncology Special Interest Group of the APTA Oncology Section is determined to use this information to facilitate improvements in the treatment of these children. Specifically, a Limb Sparing Committee has been formed to more clearly develop and define the role of physical therapy in the management of pediatric patients with sarcoma.

Long-term functional outcomes and quality of life in adult survivors of childhood extremity sarcomas: a report from the St. Jude Lifetime Cohort Study.

This study compared measured physical performance, health-related quality of life (HRQOL), and social role attainment between extremity sarcoma survivors and controls, and evaluated associations between disease and treatment exposures, health conditions, and performance measures. Survivors of extremity sarcoma from the St. Jude Lifetime cohort and controls frequency matched by age-, sex-, and race completed physical performance testing and questionnaires. Survivors with Z-scores on outcome measures ≤ -2.0 SD (compared to controls) were categorized with severe impairment/limitation. Among 206 survivors (52.4% male median age 36years (range 19-65)), 37% had low relative lean mass, 9.7% had an ejection fraction <50%, 51.5% had diffusion capacity for carbon monoxide <75%, 27.7% had sensory and 25.2% motor neuropathy, and 78.2% had musculoskeletal complications. Severe impairments/limitations were present among ≥25% of survivors on fitness, balance, and physical HRQOL measures, and among ≥15% on strength and activity of daily living measures. Lower extremity tumor location (OR 8.23, 95% CI 2.54-26.67, P value 0.0004) and amputation (OR 8.07, 95% CI 3.06-21.27, P value <0.0001) were associated with poor fitness. Poor fitness was associated with increased odds of scoring <40 on the SF-36 physical component summary (OR 4.83, 95% CI 1.95-11.99, P value 0.001) and role-physical subscale (OR 3.34, 95% CI 1.33-8.43, P value 0.01). Survivors and controls had similar rates of marriage, independent living, employment, and college attendance. Extremity sarcoma survivors experience high rates of physical impairment and report lower than expected physical HRQOL. However, they are as likely as peers to be married, live independently, be employed, and attend college. Follow-up for extremity sarcoma survivors should include assessment of need for further orthopedic care and rehabilitation to address cardiopulmonary and musculoskeletal health.

The University of Michigan Sarcoma Survivorship Clinic: Preventing, Diagnosing, and Treating Chronic Illness for Improved Survival and Long-Term Health.

The Children's Cancer Survivorship Study reports more chronic illnesses in sarcoma survivors than other pediatric cancers. Chemotherapy and radiation put survivors at risk for developing chronic illnesses, including heart disease, diabetes, hypertension, and kidney failure. Sarcoma survivors may have a reduced life expectancy and signs of heart disease in their 30s and 40s. Since these medical problems occur much later in the general population, they often go undetected or misdiagnosed in sarcoma survivors, creating delays in intervention and treatment. The good news is that these chronic illnesses can often be prevented or minimized. The most common adverse effect of chemotherapy and radiation is coronary artery disease (CAD). CAD has a number of risk factors, including hypertension, diabetes, obesity, and dyslipidemia. These risk factors are modifiable with lifestyle changes, including diet and exercise, and/or pharmacological intervention. By identifying and managing risk factors like hypertension early, we in turn reduce the risk for CAD and prolong survival. This is well established in the general population; there is no reason a priori not to apply it to sarcoma survivors. Sarcoma survivors should be followed by physicians who understand the late effects and outcomes of sarcoma treatment. The University of Michigan Sarcoma Survivorship Clinic provides long-term care for sarcoma survivors by preventing, diagnosing, and treating the adverse long-term physical and psychological effects associated with sarcoma survivorship.

Conditional Survival and Predictors of Late Death in Patients With Ewing Sarcoma.

Long-term survivors of Ewing sarcoma (EWS) are at considerable risk for future complications, including late relapse and death. Data on prognostic factors for late death in those who have survived beyond 5 years are lacking. We conducted a retrospective cohort study using the Surveillance, Epidemiology, and End Results database. We obtained clinical features and outcome data on 1,351 patients with EWS who had survived 60 months or more. From these data, we performed univariate and multivariable analyses of overall survival (OS) using log-rank tests and Cox proportional hazard models. Of 1,351 patients in the cohort, there were 209 deaths, 144 (69%) of which were reported to be due to EWS. The OS for 5-year survivors at 10 years was 87.5% (95% confidence interval 85.4-89.3%). Univariate adverse prognostic factors for late death in 5-year survivors included age ≥ 18 years at initial diagnosis, male sex, and axial/pelvic primary site. Initial stage was not prognostic. Independent adverse prognostic factors for late death included black race (hazard ratio [HR] 2.16, P = 0.01), age ≥ 18 years at diagnosis (HR 2.02, P < 0.001), male sex (HR 1.43, P = 0.01), and axial/pelvic primary site (HR 1.43, P = 0.02). The majority of late deaths in 5-year survivors are due to EWS. Black race, age ≥18 at diagnosis, male sex, and axial/pelvic primary site (but not stage at diagnosis) are independently associated with increased risk of late death.

Do Patients After Chondrosarcoma Treatment Have Age-appropriate Bone Mineral Density in the Long Term?

BackgroundIn long-term survivors of osteosarcoma and Ewing sarcoma treated with the addition of radio- and chemotherapy, low bone mineral density (BMD) and fractures have been observed, presumably resulting from these adjuvants. Because patients with chondrosarcoma usually are not treated with conventional adjuvant treatment, observation of low BMD in patients with chondrosarcoma presumably would be the result of other mechanisms. However, BMD in patients with a history of chondrosarcoma has not been well characterized.Questions/PurposesThe aim of our study was to address the following questions: (1) Do long-term survivors of chondrosarcoma have normal BMD and, if not, which factors contribute to low BMD? (2) Is there a greater risk of fracture and does the Fracture Risk Assessment Tool (FRAX®) score reflect fracture likelihood?MethodsAll known patients with a history of chondrosarcoma treated at our institution before 2006 were identified. Of 127 patients believed to be alive at the time of this study, 30 agreed to participate in this study (11 females, 19 males; mean age at surgery, 39 ± 12 years; mean followup, 12 ± 5 years). With the data available, the 30 participants were not different from the 97 nonparticipants in terms of age, sex, BMI, tumor grade, tumor location (axial versus appendicular, lower extremity versus elsewhere), and use of any treatment known to influence osteopenia (chemotherapy, lower extremity surgery). BMD was measured and history of fractures was assessed using a questionnaire. The patients´ BMD measurements in this study were sex- and age-matched with a normative sex- and age-categorized reference population reported by Kudlacek et al. Associations were tested by univariate regressions and ANOVAs of all measures of BMD and eligible oncologic and demographic factors.ResultsEighteen of 30 (60%) patients had a pathologic BMD according to the WHO dual-energy x-ray absorptiometry definition, 15 (50%) had osteopenia, and three (10%) had osteoporosis. T-scores in the study cohort were lower than reference values for the femur neck (mean difference, 0.64; 95% CI, 0.27–1.01; p < 0.0015), but not for the spine (mean difference, 0.39; 95% CI, −0.06 to 0.84; p = 0.09). Thirteen patients (45%) reported a history of fractures not distinguishing between low and high impact. The incidence of fractures was 2.8 greater than expected from a comparison with a published microcensus survey of the Austrian population. No effect of the FRAX® score on fracture risk could be identified (p = 0.057).ConclusionsLong-term survivors of chondrosarcoma appear to be at greater risk for having low BMD develop than the healthy population. Although these results are preliminary and based on a very small sampling of patients, if they can be confirmed in larger studies, BMD assessment by dual-energy x-ray absorptiometry might be considered as these patients are followed posttreatment by sarcoma care units. The reasons for low BMD still must be elucidated.Level of EvidenceLevel IV, prognostic study.

Marriage and fertility in long-term survivors of childhood, adolescent and young adult (AYA) high-grade sarcoma.

We investigated the marital status and the presence or absence of children in survivors of childhood, adolescent and young adult (AYA) high-grade sarcoma and examined the influence of these factors on the quality of life (QOL) of these survivors. Thirty-eight survivors of childhood and AYA high-grade sarcoma (18 males, 20 females) participated in a questionnaire survey on marital status and presence or absence of children, as well as on the health-related QOL (HR-QOL), using the Short Form 36 Health Survey. Diagnoses among these survivors were osteosarcoma (28 participants), Ewing's sarcoma (4 participants), synovial sarcoma (4 participants) and others (2 participants). Of the 18 males who participated in the survey, eight (44.4%) were married, of whom five (62.5%) had children. Fifteen (75.0%) of the 20 females were married, of whom 14 (93.3%) had children. The proportions of surviving male patients who were married and who had children, respectively, were lower than those of surviving female patients. The proportion of ifosfamide-treated men with children was significantly lower than that of non-ifosfamide-treated men (p=0.018). With respect to the relationship between marital status and HR-QOL, the scores for the vitality and mental health domains of the SF-36 of survivors who were married were significantly higher than those of unmarried survivors. The results of our questionnaire survey reveal that among the male survivors of high-grade sarcoma, the proportions of those who were married and of those with children were lower than those of female survivors, suggesting that strategies providing support for marriage and child-rearing may be necessary for the male survivor group. In the married group, mental QOL was high.

Changes in the body image of bone sarcoma survivors following surgical treatment--A qualitative study.

Background and ObjectivesAmong several long‐term effects, people treated for cancer may experience an altered appearance. Our study aims to identify how visible body changes following surgical treatment affect the life and identity of primary bone sarcoma survivors 3–10 years after diagnosis. A qualitative, phenomenological, and hermeneutic design was applied.MethodsSarcoma survivors (n = 18) who were previously treated at Norwegian Radium Hospital, Oslo University Hospital, participated in the study. In‐depth and semi‐structured interviews were conducted and analyzed using inductive thematic analysis.ResultsThe main finding of this study concerned how altered appearance after bone cancer treatment in the hip/pelvis or lower extremities affected the participants’ self‐esteem. Half of the participants expressed concerns about their visible differences, particularly those with functional impairment. They felt that it is important to hide the bodily signs of changes to appear as normal as possible, as well as attractive and healthy. They describe, with specific examples, how these changes influence their self‐realization, especially their social life.ConclusionsHealthcare providers who guide bone sarcoma survivors during follow‐up should develop a comprehensive understanding of what it means to cope with a changed and challenging body. J. Surg. Oncol. 2016;113:229–234. © 2015 The Authors. Journal of Surgical Oncology Published by Wiley Periodicals, Inc.

Aspects of mental health dysfunction among survivors of childhood cancer.

Background:Some previous studies have reported that survivors of childhood cancer are at an increased risk of developing long-term mental health morbidity, whilst others have reported that this is not the case. Therefore, we analysed 5-year survivors of childhood cancer using the British Childhood Cancer Survivor Study (BCCSS) to determine the risks of aspects of long-term mental health dysfunction.Procedure:Within the BCCSS, 10 488 survivors completed a questionnaire that ascertained mental health-related information via 10 questions from the Short Form-36 survey. Internal analyses were conducted using multivariable logistic regression to determine risk factors for mental health dysfunction. External analyses were undertaken using direct standardisation to compare mental health dysfunction in survivors with UK norms.Results:This study has shown that overall, childhood cancer survivors had a significantly higher prevalence of mental health dysfunction for 6/10 questions analysed compared to UK norms. Central nervous system (CNS) and bone sarcoma survivors reported the greatest dysfunction, compared to expected, with significant excess dysfunction in 10 and 6 questions, respectively; the excess ranged from 4.4–22.3% in CNS survivors and 6.9–15.9% in bone sarcoma survivors. Compared to expected, excess mental health dysfunction increased with attained age; this increase was greatest for reporting ‘limitations in social activities due to health', where the excess rose from 4.5% to 12.8% in those aged 16–24 and 45+, respectively. Within the internal analyses, higher levels of educational attainment and socio-economic classification were protective against mental health dysfunction.Conclusions:Based upon the findings of this large population-based study, childhood cancer survivors report significantly higher levels of mental health dysfunction than those in the general population, where deficits were observed particularly among CNS and bone sarcoma survivors. Limitations were also observed to increase with age, and thus it is important to emphasise the need for mental health evaluation and services across the entire lifespan. There is evidence that low educational attainment and being unemployed or having never worked adversely impacts long-term mental health. These findings provide an evidence base for risk stratification and planning interventions.

Salivary gland second cancer after bone sarcoma treatment.

Second malignant neoplasms (SMN) or second cancers may occur in 2-5 % of childhood cancer survivors within 5-25 years after diagnosis and treatment of a bone sarcoma. The most common are leukemia and breast cancer; salivary gland SMN are exceptional. To enhance the literature, we reviewed our patients with bone sarcomas and evaluated the incidence and outcome of salivary gland SMN. We retrospectively studied all patients with osteosarcoma and Ewing's sarcoma treated at the Istituto Ortopedico Rizzoli with chemotherapy from January 1983 to December 2012. There were 883 osteosarcoma and 543 Ewing's sarcoma patients. We evaluated the date of diagnosis and histology of bone sarcoma, chemotherapy administered, date of diagnosis and histology of SMN, and survival of patients. The 10-year incidence of SMN was 3.6 %; the most common were breast cancer, leukemia, sarcomas, and salivary gland neoplasms. The incidence of salivary gland SMN was 0.5 %; there were five male and two female patients with a mean age of 19 years (range 13-28 years) who experienced a salivary gland SMN within a mean interval of 79 months (range 51-97 months). The most common salivary gland involved was the parotid followed by the submandibular gland. One of the seven patients with salivary gland SMN died from his SMN. Treating physicians should be aware of the risk of salivary gland SMN after chemotherapy for bone sarcomas in children and adolescents. Close follow-up of childhood bone sarcoma survivors for SMN is important.

Long-term risks for chronic medical conditions and premature mortality in survivors of pediatric soft tissue sarcoma: A report from the Childhood Cancer Survivor Study (CCSS).

10013 Background: Approximately 67% of children with soft tissue sarcoma (STS) survive 5+ years. Late mortality, subsequent malignant neoplasms (SMN), and frequency or severity of chronic medical c...

Long-term adverse outcomes in survivors of childhood bone sarcoma: the British Childhood Cancer Survivor Study.

Background:With improved survival, more bone sarcoma survivors are approaching middle age making it crucial to investigate the late effects of their cancer and its treatment. We investigated the long-term risks of adverse outcomes among 5-year bone sarcoma survivors within the British Childhood Cancer Survivor Study.Methods:Cause-specific mortality and risk of subsequent primary neoplasms (SPNs) were investigated for 664 bone sarcoma survivors. Use of health services, health and marital status, alcohol and smoking habits, and educational qualifications were investigated for survivors who completed a questionnaire.Results:Survivors were seven times more likely to experience all-cause mortality than expected, and there were substantial differences in risk depending on tumour type. Beyond 25 years follow-up the risk of dying from all-causes was comparable to the general population. This is in contrast to dying before 25 years where the risk was 12.7-fold that expected. Survivors were also four times more likely to develop a SPN than expected, where the excess was restricted to 5–24 years post diagnosis. Increased health-care usage and poor health status were also found. Nonetheless, for some psychosocial outcomes survivors were better off than expected.Conclusions:Up to 25 years after 5-year survival, bone sarcoma survivors are at substantial risk of death and SPNs, but this is greatly reduced thereafter. As 95% of all excess deaths before 25 years follow-up were due to recurrences and SPNs, increased monitoring of survivors could prevent mortality. Furthermore, bone and breast SPNs should be a particular concern. Since there are variations in the magnitude of excess risk depending on the specific adverse outcome under investigation and whether the survivors were initially diagnosed with osteosarcoma or Ewing sarcoma, risks need to be assessed in relation to these factors. These findings should provide useful evidence for risk stratification and updating clinical follow-up guidelines.

Systematic review and meta-analysis of objective and subjective quality of life among pediatric, adolescent, and young adult bone tumor survivors.

Pediatric, adolescent, and young adult survivors of bone sarcomas are at risk for poor quality of life (QOL). We conducted a systematic review and meta-analysis to summarize the literature describing QOL in this population and differences in QOL based on local control procedures. Included studies described ≥5 patients <25 years old who had completed local control treatment for bone sarcoma, defined QOL as a main outcome, and measured it with a validated instrument. Data extraction and quality assessments were conducted with standardized tools. Meta-analyses compared QOL based on surgical procedure (limb-sparing vs. amputation) and were stratified by assessment type (objective physical function, clinician-assessed disability, patient-reported disability, and patient-reported QOL). Effect sizes were reported as the standard mean difference when multiple instruments were used within a comparison and weighted mean difference otherwise. All were weighted by inverse variance and modeled with random effects. Twenty-two of 452 unique manuscripts were included in qualitative syntheses, eight of which were included in meta-analyses. Manuscripts were heterogeneous with respect to included patient populations (age, tumor type, time since treatment) and QOL instruments. Prospective studies suggested that QOL improves over time, and that female sex and older age at diagnosis are associated with poor QOL. Meta-analyses showed no differences in outcomes between patients who underwent limb-sparing versus amputation for local control. QOL studies among children and AYAs with bone sarcoma are remarkably diverse, making it difficult to detect trends in patient outcomes. Future research should focus on standardized QOL instruments and interpretations.

Ovarian reserve after treatment with alkylating agents during childhood.

What is the effect of different alkylating agents used without pelvic radiation to treat childhood cancer in girls on the ovarian reserve in survivors? Ovarian reserve seems to be particularly reduced in survivors who received procarbazine (in most cases for Hodgkin lymphoma) or high-dose chemotherapy; procarbazine but not cyclophosphamide dose is associated with diminished ovarian reserve. A few studies have demonstrated diminished ovarian reserve in survivors after various combination therapies, but the individual role of each treatment is difficult to assess. Prospective cross-sectional study, involving 105 survivors and 20 controls. One hundred and five survivors aged 17-40 years and 20 controls investigated on Days 2-5 of a menstrual cycle or Day 7 of an oral contraceptive pill-free interval. ovarian surface area (OS), total number of antral follicles (AFC), serum levels of follicle-stimulating hormone (FSH), luteinizing hormone (LH), estradiol and anti-Müllerian hormone (AMH). Survivors had a lower OS than controls: 3.5 versus 4.4 cm(2) per ovary (P = 0.0004), and lower AMH levels: 10.7 versus 22 pmol/l (P = 0.003). Ovarian markers (OS, AMH, AFC) were worse in patients who received high-dose compared with conventional-dose alkylating agents (P = 0.01 for OS, P = 0.002 for AMH, P < 0.0001 for AFC). Hodgkin lymphoma survivors seemed to have a greater reduction in ovarian reserve than survivors of leukaemia (P = 0.04 for AMH, P = 0.01 for AFC), sarcoma (P = 0.04 for AMH, P = 0.04 for AFC) and other lymphomas (P = 0.04 for AFC). A multiple linear regression analysis showed that procarbazine but not cyclophosphamide nor ifosfamide dose was associated with reduced OS (P = 0.0003), AFC (P = 0.0007), AMH (P < 0.0001) and higher FSH levels (P < 0.0001). The small percentage of participating survivors (28%) from the total cohort does not allow conclusion on fertility issues because of possible response bias. The association between procarbazine and HL makes it impossible to dissociate their individual impacts on ovarian reserve. The number of controls is small, but ovarian volume and AMH levels in survivors were compared with published normal values and results were unchanged. Early detection and follow-up of compromised ovarian function after cancer therapy should help physicians to counsel young survivors about their fertility window. However, longitudinal follow-up is required to determine the rate of progression from low ovarian reserve to premature ovarian failure. La Ligue contre le Cancer (grant no., PRAYN7497). The authors have no competing interests to disclose.

Survival and Quality of Life Following Treatment for Sarcoma

Advances in multimodality therapy have improved the survival rates of pediatric sarcomas over the last 40 years, with current rates over 65 %. However, the advances have not been equal, and patients with metastatic and relapsed disease continue to have a poor prognosis. This review focuses on the epidemiology, prognostic factors, treatment, and survival of the three most common pediatric sarcomas: osteosarcoma, Ewing sarcoma, and rhabdomyosarcoma. It highlights a new collaboration to study osteosarcoma, recent advances in Ewing sarcoma treatment with increased intensity of chemotherapy timing, and advances in low-risk and high-risk rhabdomyosarcoma treatment. Additionally, it discusses the common treatment-related sequelae encountered in survivors. Sarcoma survivors have increased functional impairment and activity limitations compared with healthy controls and other cancer survivors, emphasizing the need to increase research in these areas in order to improve future physical functioning and quality of life.

Cured of primary bone cancer, but at what cost: a qualitative study of functional impairment and lost opportunities.

Purpose. Our study aims to explore how former cancer patients experience physical and psychosocial late effects 3–7 years after they underwent treatment for primary bone sarcoma in the hip/pelvic region. A qualitative, phenomenological, and hermeneutic design was applied. Methods. Sarcoma survivors (n = 10) previously treated at Oslo University Hospital, Norwegian Radium Hospital were selected to participate. In-depth and semistructured interviews were conducted. The interviews were analysed using inductive thematic analysis. Results. The participants reported that the late effects had three core spheres of impact: “their current daily life,” “their future opportunities,” and “their identity.” They expressed negative changes in activity, increased dependence on others, and exclusion from participation in different areas. Their daily life, work, sports activities, and social life were all affected. Several of their experiences are similar to those described by people with functional impairment or disability. Conclusion. Patients cured of bone cancer in the hip/pelvic region pay a significant price in terms of functional impairment, practical challenges, exclusion from important aspects of life, and loss of previous identity. It is important to appreciate this in order to help bone cancer survivors who struggle to reorient their life and build a secure new identity.

Endoprosthetic Reconstruction in the Limb Salvage Surgery for Treatment of Malignant Bone Tumors

Endoprosthetic replacement is the most frequently used surgical method for the skeletal reconstruction in the limb salvage surgery for malignant bone tumors. Modular prostheses are generally used for typical locations such as distal and proximal femur, proximal tibia and proximal humerus. Expandable prosthesis is used to overcome leg length discrepancy in growing children. Aseptic loosening, infection and mechanical failure are major complications resulting in revision surgery. Many efforts should be made to develop more durable and functional implants to improve the quality of life of sarcoma survivors.

Self-Reported Functional Outcomes and Quality of Life Assessments in Long-Term Survivors of Ewing Sarcoma

Self-Reported Functional Outcomes and Quality of Life Assessments in Long-Term Survivors of Ewing Sarcoma

Anti-Müllerian hormone screening to assess ovarian reserve among female survivors of childhood cancer

Anti-Müllerian hormone (AMH) is an indicator of oocyte reserve in healthy females. The role of AMH testing in oncology remains investigational, although its sensitivity and stability over the menstrual cycle make it an attractive screening test for fertility assessment among female cancer survivors. We measured AMH level in survivors of childhood cancer and evaluated its association with treatment and patient factors. Participants were adult female survivors of childhood malignancy treated with chemotherapy. Serum AMH was measured at a random day of the menstrual cycle. Multivariate analysis was used to evaluate the association between AMH level, alkylating agent exposure using the cyclophosphamide equivalent dose (CED), and other covariates. Sixty-six females with a median attained age of 23.3 years were eligible for analysis. Median AMH was 25.5 pM (range 0.5-108.0), at a median time of 11.5 years (range 1.4-25.1) since cancer diagnosis. Twenty-three patients (34.8%) had low AMH, including a significant proportion that reported normal menstrual cycles. Compared to ALL survivors, sarcoma survivors had significantly lower AMH levels. Among alkylating agents evaluated, procarbazine had the greatest adverse effect on AMH. In multivariate analysis, higher CED (p = 0.001), older age at diagnosis (p < 0.001), and use of oral contraceptive pills (p = 0.04) remained significantly associated with lower AMH. Random AMH can reveal evidence of oocyte depletion among female survivors reporting normal cycles, although low AMH should be interpreted cautiously among those taking oral contraception. Age at exposure and CED can aid identification of those more likely to have low AMH, although CED may underestimate the effect of procarbazine on oocyte reserve. Measurement of AMH can reveal apparent depletion of ovarian reserve in female childhood cancer survivors reporting normal menstrual cycles. Sarcoma survivors and those exposed to procarbazine may benefit from targeted AMH evaluation in an outpatient setting, and thereby allow appropriate fertility counseling before the onset of premature ovarian failure. The cyclophosphamide equivalent dose may facilitate comparison of the potential effect of different regimens on fertility.