Racial and ethnic disparities in treatment and survival of pediatric sarcoma.

BACKGROUND: Childhood sarcomas are rare and require complex interdisciplinary care including surgery, chemotherapy, and radiation. The goal of this study was to determine if racial or ethnic disparities exist for pediatric sarcoma patients in the United States. METHODS: The United States' National Cancer Institute's Surveillance, Epidemiology, and End Results database was used to identify patients aged 0-21 diagnosed with primary sarcomas from 1973 to 2012. Patients were considered by race and ethnicity. Survival curves were computed using the Kaplan-Meier method and the log-rank test. RESULTS: A total of 11,502 patients were included in this study. When stratified by race, non-Hispanic black and Hispanic patients were significantly more likely to present with advanced stage disease than white patients. White patients were more likely to receive radiation therapy than black and Hispanic patients (P = 0.01). There was no significant difference between patients who underwent surgery (P = 0.21). Overall survival was better for white patients than black or Hispanic ones. Despite the overall 5-year survival improvement during the study period (56.2%-70.3%), survival disparities between race and ethnicity have grown. CONCLUSIONS: Racial and ethnic disparities do exist with respect to stage, treatment, and survival of these rare tumors. Black and Hispanic patients are presenting at more advanced stage and have overall worse survival. This survival disparity has widened over the past 4 decades.

Cross-cultural adaptation and validation of the Japanese version of the Toronto Extremity Salvage Score (TESS) for patients with malignant musculoskeletal tumors in the upper extremities.

BACKGROUND: The Toronto Extremity Salvage Score (TESS) is a widely used disease-specific patient-completed questionnaire for the assessment of physical function in patients with musculoskeletal tumors; however, there had not been the validated Japanese version of the TESS. The aim of this study was to validate the Japanese version of the TESS in patients with musculoskeletal tumors in the upper extremity. METHODS: After developing a Japanese version of the TESS, the questionnaire was administered to 53 patients to examine its reliability and validity in comparison with the Musculoskeletal Tumor Society (MSTS) scoring system and Short Form-36 (SF-36). RESULTS: Test-retest reliability with intraclass correlation coefficient (0.93) and internal consistency with Cronbach's alpha (0.90) were excellent. Factor analysis showed that the construct structure consisted of 3-item clusters, and the Akaike Information Criterion network also demonstrated that the items could be divided into 3 domains according to their content. The TESS strongly correlated with the MSTS rating scale (r = 0.750; P < 0.001) and the SF-36 physical functioning scale (r = 0.684; P < 0.001). However, as expected, the TESS had low correlations with the SF-36 mental health and role-emotional subscales and the MSTS scoring system manual dexterity domain. CONCLUSIONS: Our study suggests that the TESS is a reliable and valid instrument to measure patient-reported physical functioning in patients with upper extremity sarcoma.

Giant cell tumours in fingers among the Inuit population in Greenland.

OBJECTIVE: Giant cell tumours (GCTs) of the tendon sheets in fingers are rare. We therefore find it of interest to report on 5 cases identified in the Inuit population in Greenland within 16 months prior to this study. MATERIAL AND METHODS: The Inuit account for 56,000 people of the total population in Greenland. From November 2010 to 16 months prior to this study, we diagnosed 5 cases (0.6% of all orthopaedic operations) with a GCT of the flexor tendon sheet of a finger. The patients were aged between 10 and 54 years, and 4 were women. All of them had noticed slow-growing tumours over 3 or more years and were referred for a suspected ganglion. RESULTS: In two cases, the tumour was located at the distal interphalangeal (DIP) joint in the thumb and in one case at the third finger. Two other patients had tumours at the metacarpophalangeal (MCP) joint of the third finger and the thumb, respectively; one of these two had a communicating tumour to the DIP joint. The last patient had two tumours on the same finger, one at the MCP joint and the other at the DIP joint. In one case, the tumour had also eroded the cortex of the first phalanx of the thumb, and the largest tumour measured 5 cm. CONCLUSION: GCTs of the flexor tendon sheets in fingers are rare. It could be a coincidence that we have seen 5 cases within a short period of time. It is not possible to identify past cases through a register. A tumour in a finger is not the most common location for a ganglion, especially not at the DIP level. Therefore, a large tumour at this location is more likely to be a GCT.

Socio-economic factors affect the outcome of soft tissue sarcoma: an analysis of SEER data.

BACKGROUND: This study analyzed whether socio-economic factors affect the cause specific survival of soft tissue sarcoma (STS). METHODS: Surveillance, Epidemiology and End Results (SEER) soft tissue sarcoma (STS) data were used to identify potential socio-economic disparities in outcome. Time to cause specific death was computed with Kaplan-Meier analysis. Kolmogorov-Smirnov tests and Cox proportional hazard analysis were used for univariate and multivariate tests, respectively. The areas under the receiver operating curve were computed for predictors for comparison. RESULTS: There were 42,016 patients diagnosed STS from 1973 to 2009. The mean follow up time (S.D.) was 66.6 (81.3) months. Stage, site, grade were significant predictors by univariate tests. Race and rural-urban residence were also important predictors of outcome. These five factors were all statistically significant with Cox analysis. Rural and African-American patients had a 3-4% disadvantage in cause specific survival. CONCLUSIONS: Socio-economic factors influence cause specific survival of soft tissue sarcoma. Ensuring access to cancer care may eliminate the outcome disparities.

Predictors of acute chemotherapy-associated toxicity in patients with Ewing sarcoma.

BACKGROUND: Ewing sarcoma (ES) is a malignant tumor of bone and soft tissue of children and young adults. Patients with ES are treated with intensive chemotherapy regimens. We describe predictors of acute chemotherapy-associated toxicity in this population. PROCEDURE: In this retrospective cohort study, records of ES patients treated at two academic medical centers between 1980 and 2010 were reviewed. Grade 3 and 4 non-hematologic chemotherapy-associated toxicities during frontline therapy were recorded for each patient, along with potential clinical and demographic predictors of toxicity. Bivariate analyses were performed using the Fisher exact test. Multivariate analysis was performed using logistic regression. RESULTS: The cohort included 142 patients with ES and toxicity data. In bivariate analyses, age <12 years at diagnosis, Latino ethnicity, low family income, and treatment on a clinical trial were associated with higher incidence of toxicity (P < 0.01). Tumor size, site, stage, mode of local control, body mass index, overall chemotherapy exposure and dose-intensity were not associated with toxicity. In multivariate analysis, low income (odds ratio (OR) 4.97, 95% confidence interval (CI) 1.9-13.1), clinical trial enrollment (OR 3.67, 95% CI 1.2-10.9), pelvic tumor site (OR 3.88, 95% CI 1.17-12.88), and age <12 years (OR 2.8, 95% CI 1.0-7.5) were independent predictors of toxicity. CONCLUSION: ES patients who are younger, of Latino ethnicity, have pelvic tumors or low income have higher rates of toxicity that may require increased supportive care. Treatment on a clinical trial was also associated with higher rates of toxicity, though this finding may reflect better reporting in these patients.

The natural history and prognosticative factors of adult extremity soft tissue sarcomas: an Asian perspective.

INTRODUCTION: We describe the natural history of Asian adult soft tissue sarcomas (STSs) in the extremities and predict prognosticative factors for local recurrence, metastasis and tumour-related death. MATERIALS AND METHODS: Between January 1999 and May 2009, 67 adult patients with first presentation STSs of extremity sites underwent surgical treatment at a single institution. The associations between patient demographics and pathological features with local recurrence, metastasis and mortality were studied using univariate and multivariate analysis. RESULTS: The mean age of our patients was 52.4 years with most presentations occurring in the thigh. Majority of Asian STSs were high grade (61.3%) and large tumours with 81.0% being >5 cm. Stages Ia, Ib, IIa, IIb, IIc, III and IV accounted for 6.6%, 6.6%, 26.2%, 11.5%, 3.3%, 42.6% and 3.3% of presentations, respectively. Patients were followed-up for a mean period of 45.9 months. On univariate analysis, high tumour grade and advanced stage (IIc to IV) were predictive of local recurrence and metastasis. Deep lesions were more likely to recur but not metastasise or cause death. Age, sex, size, and margin positivity were not predictive for all end-points. On multivariate testing, only pathological high grade was associated adversely with local recurrence [odds ratio (OR) = 10.0, 95% CI, 1.2 to 84.9, P = 0.035], metastasis (OR = 12.7, 95% CI, 2.46 to 65.2, P = 0.002) and mortality (OR = 16.2, 95% CI, 1.95 to 135.0, P = 0.010). CONCLUSIONS: Asian adult extremity soft tissue sarcomas present late and are most commonly found in the thigh. High pathological grade is a consistent independent predictor for local failure, distant spread and tumour-related death. Our results reaffirm the current thinking that tumour biology is of primary importance in determining patient outcomes.

PAX--FKHR fusion genes and AChR-gamma in Chinese patients with rhabdomyosarcoma: diagnosis using formalin-fixed archival tissues.

The majority of alveolar RMSs have t(2;13)(q35;q14) or (1:13)(p36;q14),which generate PAX3/7 -FKHR fusion genes. Here, the authors detected the PAX3/7-FKHR fusion transcripts in 17 formalin-fixed, paraffin-embedded RMSs and 26 other SRCTs using one-step RT-PCR. PAX3 -FKHR and PAX7-FKHR transcripts were positive in 4/8 and 2/8 cases of ARMS, respectively. 9 ERMSs and 26 other SRCTs were negative for PAX3/7-FKHR. In addition, AChR-gamma and AChR-alpha mRNA were detected by semiquantitative duplex PCR in above cases and 3 normal muscles. 17 RMSs were found to have overexpression of AChR-gamma, with an AChR-gamma/-alpha ratio of > or =1; 3 cases of normal muscle had very weak AChR-gamma expression, with an AChR- gamma/-alpha ratio of <1. AChR-gamma transcripts were not detectable in all 26 other SCRTs. The results demonstrated that detection of PAX3/7-FKHR fusion gene by one-step RT-PCR is useful in the diagnosis of RMS and that AChR-gamma is overexpressed in Chinese RMS patients.

H-ras and K-ras mutations in soft tissue sarcoma: comparative studies of sarcomas from Korean and American patients.

BACKGROUND: The authors' recent investigation of Korean patients with sarcoma has suggested that ras gene activation may play a role in oncogenesis. The authors attempted to extend the mutation analysis to sarcomas in American patients to determine whether there were racial or geographic factors relevant to the initiation or progression of sarcoma. METHODS: H-ras and K-ras genes were examined in sarcomas obtained from patients in the midwestern U. S. using the polymerase chain reaction technique and direct automated sequencing analysis. Tumors studied included 29 malignant fibrous histiocytomas, 7 liposarcomas, 5 rhabdomyosarcomas, and 9 leiomyosarcomas. RESULTS: Of the 50 sarcomas evaluated, only 1 (2%) definable mutation was found; a GGC to AGC transition at codon 12 of H-ras was found in a rhabdomyosarcoma. None of the patients had a K-ras mutation. The rates of incidence of ras point mutations in these samples were much lower (H-ras: 2%; 95% confidence interval [95% CI], 0-11.5% and K-ras: 0%) than described for both genes in Korean studies (H-ras: 16%; 95% CI, 5.2-26.8% and K-ras: 44%; 95% CI, 29.5-58.5%). CONCLUSIONS: Although the reason for this discrepancy is not clear, there were no major differences found in histology or clinical stages. Based on this study of 50 sarcoma samples from American patients and the authors' previous study of 45 Korean tumor samples, the authors conclude that differing genetic and/or environmental mechanisms can affect sarcoma development or progression. Mutation of the H-ras and K-ras genes appears to be uncommon in sarcomas occurring in American patients, suggesting that the activation by point mutations of the H-ras and K-ras genes does not play a significant role in the pathogenesis or progression of sarcoma in these patients.

Soft tissue sarcomas in whites and blacks living in the United States of America.

Five hundred four hospitals volunteered reports on 2,355 patients in a long-term study and 645 institutes reported on 3,457 in a short-term study. Out of 5,623 cases of soft tissue sarcoma (STS) reported in white and blacks living in the United States of America, 574 cases (10.2%) were reported in blacks. No striking differences were found between blacks and whites concerning anatomic sites, histologic types, histologic grades, or clinical stages of STS. A higher percentage of patients with recurrences were reported in whites with liposarcoma (37.7% compared to 28.9% in blacks), and leiomyosarcoma (45% in whites compared to 39.1% in blacks). On the other hand recurrences were more frequent in fibrosarcoma and rhabdomyosarcoma in black patients. No significant differences in survival was found between white and black patients with STS.

The incidence and epidemiologic characteristics of neuroblastoma in the United States.

The incidence of neuroblastoma in the United States is described in relation to age, sex, race, and anatomic site, as well as population-derived indicators of socioeconomic levels, degree of urbanization, and farming activity. Incidence data were obtained for the years 1973-1978 from the Surveillance, Epidemiology and End Results Program of the National Cancer Institute. Based upon 265 cases, the overall incidence of neuroblastoma was 2.26 per million person-years. Approximately 60% of the cases were diagnosed under age two years, 75% under age five years and 84% under age 10 years. The incidence among males was 1.3 times that among females, but the male predominance was observed only among persons diagnosed under age five years. Although no difference in overall incidence was observed by race, the rate among whites was 1.6 times that among blacks and 1.5 times that among other nonwhites under age five years. Approximately 50% of all cases were diagnosed with tumors arising from the adrenals or soft tissues. No clear pattern of area-to-area variation in incidence was identified. Neuroblastoma incidence was inversely related to socioeconomic level as measured by per capita income (p = 0.05), as well as the proportion of county land devoted to farming (p = 0.034). No association was observed in relation to urbanization or population density.