Summary Delayed diagnosis of paediatric cancers is reported regularly and is a source of remorse for physicians and parents and a leading cause of malpractice claims. We did a systematic review of information about the distribution, determinants, and consequences of time to diagnosis of paediatric malignancies and compared these findings with those of court-appointed expert witnesses in malpractice claims in Canada and France. Time to diagnosis varied widely between tumour types in the 98 relevant studies (medians ranged from 2–260 weeks) without any significant decrease with time. Determinants of a long delay in diagnosis included older age, qualification of the first physician contacted, non-specific symptoms, histological type, and tumour localisation. Delayed diagnosis was associated with poor outcome for retinoblastoma and possibly for leukaemia, nephroblastoma, and rhabdomyosarcoma (data were insufficient for definitive conclusions). It was not associated with an adverse outcome for most CNS tumours, osteosarcoma or Ewing's sarcoma, and, paradoxically, was frequently associated with better outcomes than was short time to diagnosis in these cancers. A third of the court-appointed experts provided testimony concordant with the medical literature. The relations between delay in diagnosis and outcome are complex and probably depend more on tumour biology than on parental or medical factors.

https://www.thelancet.com/pdfs/journals/lanonc/PIIS1470-2045(12)70361-3.pdf

Delays in diagnosis of paediatric cancers: a systematic review and comparison with expert testimony in lawsuits.

Purpose The time to diagnosis (TtD) of Ewing tumors is one of the longest among pediatric tumors. Its precise consequences, however, have not been studied well. We analyzed the distribution of TtD for Ewing tumors in children and adolescents and its association with clinical features, tumor characteristics, surgical outcome, and long-term survival. Patients and Methods We analyzed prospectively collected data from two multicenter clinical trials of patients younger than 21 years old who had Ewing bone tumors treated in France between 1988 and 2000. Clinical and tumoral features, TtD, and outcome associations were studied by univariable and multivariable analyses. Results The median TtD for the 436 patients was 70 days (interquartile range, 27 to 146 days), with no significant decrease during the study period (P > .2). The factors associated with long TtD were older age and some tumor sites (pelvis, extremities of limbs). Increased tumor volume and decreased histologic response to chemotherapy were associa...

Time to Diagnosis of Ewing Tumors in Children and Adolescents Is Not Associated With Metastasis or Survival: A Prospective Multicenter Study of 436 Patients

BACKGROUND:

Treatment of malignant sarcomas of the pelvis poses a challenge for local disease control and oncologic outcome. Many reports have described the dismal outcomes. Most studies are retrospective series coming out of single centers, thus biased toward patient selection and are of limited statistical power.



METHODS:

The authors used the Surveillance, Epidemiology, and End Results database to analyze 1185 pelvic sarcoma cases from 1987 to 2006. Kaplan-Meier and Cox regression were used to analyze the significance of prognostic factors. The analysis was repeated for different histopathological subtypes to determine specific prognostic factors in each case.



RESULTS:

Incidence of pelvic sarcoma in 2006 was 89 per 100,000 persons; it has significantly increased since 1973 (P < .05). The overall 5-year survival for all the patients with pelvic sarcoma was 47%, with osteosarcoma having the worst 5-year survival at 19% and patients with chordoma having the best 5-year survival at 60%. Independent prognostic factors included age, stage, grade, size of primary lesion, histopathology, and treatment-related factors. Comparing the patients only with high-grade lesions, patients with Ewing sarcoma have the best prognosis.



CONCLUSIONS:

This is an analysis of patients with pelvic sarcomas derived from a population-based registry. Survival and prognostics vary with histopathological diagnoses. Although surgical resection was associated with superior outcomes for osteosarcoma and chondrosarcoma, there was no significant difference in outcomes of patients with Ewing sarcoma treated with surgery and/or radiotherapy. Cancer 2011. © 2010 American Cancer Society.

https://onlinelibrary.wiley.com/doi/pdf/10.1002/cncr.25684

Malignant sarcoma of the pelvic bones: treatment outcomes and prognostic factors vary by histopathology.

Background Treatment of Ewing sarcoma of the pelvic bones remains one of the most difficult tasks in the treatment of bone sarcomas. Whether surgery or radiation therapy is the best local treatment is still a matter of debate. The aim of the present study was to compare sacral and nonsacral sites with regard to the treatment and outcome of pelvic Ewing sarcomas. Methods Patients with Ewing sarcoma of the osseous pelvis diagnosed between 1986 and 2011 were identified through the Scandinavian Sarcoma Group registry. Data regarding tumor size, local treatment (surgery or radiation therapy), metastatic disease, surgical margins, local recurrence, and overall survival were analyzed. Results Of the 117 patients examined, eighty-eight had tumors in the innominate bones and twenty-nine, in the sacrum. Radiation therapy was the sole local treatment for 40% of the innominate bone tumors in contrast to 79% of the sacral tumors. The five-year disease-free survival rate in the latter group (66%) was greater than that in the group with tumors in the innominate bones (40%) (p = 0.02 adjusted for size). Conclusions Disease-free survival among patients with Ewing sarcoma was improved when the tumor was localized in the sacrum compared with the innominate bones, where these tumors are generally larger. Local radiation therapy alone appears to result in good local tumor control and may be the treatment of choice for sacral tumors.

Improved Prognosis for Patients with Ewing Sarcoma in the Sacrum Compared with the Innominate Bones: The Scandinavian Sarcoma Group Experience.

The goal of the present work was to assess the potential advantage of intensity-modulated radiotherapy (IMRT) over three-dimensional conformal radiotherapy (3D-CRT) planning in pelvic Ewing’s sarcoma. A total of 8 patients with Ewing sarcoma of the pelvis undergoing radiotherapy were analyzed. Plans for 3D-CRT and IMRT were calculated for each patient. Dose coverage of the planning target volume (PTV), conformity and homogeneity indices, as well as further parameters were evaluated. The average dose coverage values for PTV were comparable in 3D-CRT and IMRT plans. Both techniques had a PTV coverage of V95 > 98 % in all patients. Whereas the IMRT plans achieved a higher conformity index compared to the 3D-CRT plans (conformity index 0.79 ± 0.12 vs. 0.54 ± 0.19, p = 0.012), the dose distribution across the target volumes was less homogeneous with IMRT planning than with 3D-CRT planning. This difference was statistically significant (homogeneity index 0.11 ± 0.03 vs. 0.07 ± 0.0, p = 0.035). For the bowel, Dmean and D1%, as well as V2 to V60 were reduced in IMRT plans. For the bladder and the rectum, there was no significant difference in Dmean. However, the percentages of volumes receiving at least doses of 30, 40, 45, and 50 Gy (V30 to V50) were lower for the rectum in IMRT plans. The volume of normal tissue receiving at least 2 Gy (V2) was significantly higher in IMRT plans compared with 3D-CRT, whereas at high dose levels (V30) it was significantly lower. Compared to 3D-CRT, IMRT showed significantly better results regarding dose conformity (p = 0.012) and bowel sparing at dose levels above 30 Gy (p = 0.012). Thus, dose escalation in the radiotherapy of pelvic Ewing’s sarcoma can be more easily achieved using IMRT.

Pelvic Ewing sarcomas. Three-dimensional conformal vs. intensity-modulated radiotherapy.

Purpose. To review various treatment modalities on outcome of patients with pelvic Ewing’s sarcoma. Methods. Between January 1948 and December 2004, 18 male and 15 female patients aged 3 to 48 (mean, 20) years with pelvic Ewing’s sarcoma were retrospectively reviewed from the Scottish Bone Tumour Registry. Results. The commonest site involved was the ilium. The main presenting symptom was pain, followed by swelling and restricted hip movements. Treatments included surgery, radiotherapy, chemotherapy, or any of them in combination. Patients who had no metastasis at presentation (p<0.01) and underwent chemotherapy with ifosfamide (p<0.01) had significantly longer survival. Age (p=0.09), gender (p=0.95), delay in presentation (p=0.31), tumour site (p=0.9), surgery (p=0.73), and radiotherapy (p=0.23) were not predictive of survival in the univariate analysis. The 5-year event-free survival rate in patients with no metastasis at presentation was 35%.

https://journals.sagepub.com/doi/pdf/10.1177/230949900801600313

MJ Jane

Papers

Pelvic Ewing’s sarcoma: a review from Scottish Bone Tumour Registry