The aim of this study was to assess the current trends in the estimation of survival and the preferred forms of treatment of pathological fractures among national and international general and ...oncological orthopaedic surgeons, and to explore whether improvements in the management of these patients could be identified in this way.
All members of the Dutch Orthopaedic Society (DOS) and the European Musculoskeletal Oncology Society (EMSOS) were invited to complete a web-based questionnaire containing 12 cases.
A total of 96 of 948 members of the DOS (10.1%; groups 1 and 2) and 33 of 182 members of the EMSOS (18%; group 3) replied. The estimation of survival was accurate by more than 50% of all three groups, if the expected survival was short (< 3 months) or long (> 12 months). General orthopaedic surgeons preferred using an intramedullary nail for fractures of the humerus and femur, irrespective of the expected survival or the origin of primary tumour or the location of the fracture. Oncological orthopaedic surgeons recommended prosthetic reconstruction in patients with a long expected survival.
Identifying patients who require centralized care, as opposed to those who can be adequately treated in a regional centre, can improve the management of patients with pathological fractures. This differentiation should be based on the expected survival, the type and extent of the tumour, and the location of the fracture. Cite this article: Bone Joint J 2018;100-B:1392-8.
Myelolipomas are very rare benign tumours consisting of hematopoietic cells and mature adipose tissues. They are most commonly found in the adrenal glands. However, there have been several reported ...cases of extra-adrenal myelolipomas, most commonly in the presacral region. Nearly all presacral lesions are small and asymptomatic; thus, most are discovered incidentally on imaging studies.
We report two cases of presacral myelolipomas. The first is a 48-year-old female presenting with atypical back pain, found to have a mass in her presacral region with a size of 3,3 cm. The second case is a 59-year-old female, who presented for evaluation of a hip fracture, found to have a 4,7 cm presacral lesion. Both presacral myelolipomas were discovered incidentally and were confirmed by percutaneous guided fine-needle aspiration biopsy. Both were treated conservatively.
Accepted indications for the surgical excision of myelolipomas are symptomatic tumour, size >4 cm, metabolically active tumour, and a suspicion of malignancy on an imaging study. However, previous reports have documented that nearly half of the conservatively managed myelolipomas with a mean initial size of 5,1 cm, has increased in size or became symptomatic over a 3-years period.
We conclude that symptomatic presacral myelolipomas or lesions larger than 4 cm should be en-bloc resected, and we present an intuitive decision-making algorithm.
•Presacral myelolipoma is an orphan disease.•We emphasize the role of imaging in the differential diagnosis of presacral tumours.•Percutaneous biopsy is rarely indicated to distinguish the diagnosis.•We recommend enbloc resection for lesion larger than 4 cm.•We present an intuitive decision-making algorithm.
Purpose. The aim of this study was to translate and culturally adapt the Toronto Extremity Salvage Score (TESS) to Dutch and to validate the translated version. Methods. The TESS lower and upper ...extremity versions (LE and UE) were translated to Dutch according to international guidelines. The translated version was validated in 98 patients with surgically treated bone or soft tissue tumors of the LE or UE. To assess test-retest reliability, participants were asked to fill in a second questionnaire after one week. Construct validity was determined by computing Spearman rank correlations with the Short Form- (SF-) 36. Results. The internal consistency (0.957 and 0.938 for LE and UE, resp.) and test-retest reliability (intraclass correlation coefficients 0.963 and 0.969 for LE and UE, resp.) were good for both questionnaires. The Dutch LE and UE TESS versions correlated most strongly with the SF-36 physical function dimension (r=0.737 for LE, 0.726 for UE) and the physical component summary score (r=0.811 and 0.797 for LE and UE). Interpretation. The Dutch TESS questionnaire for lower and upper extremities is a consistent, reliable, and valid instrument to measure patient-reported physical function in surgically treated patients with a soft tissue or bone tumor.
Purpose
Syndromes with focal overgrowth are rare and diagnosis is difficult because manifestations are highly variable and symptoms overlap between syndromes. Diagnosis depends on clinical history, ...physical examination, and radiologic and histologic findings. This report describes a case of focal overgrowth of the left seventh rib and half of the adjacent thoracic vertebra, with overlying infiltrating lipoma.
Methods
A 13-year-old boy presented with an asymptomatic chest wall mass caused by enlargement of the seventh rib and an overlying soft-tissue mass accompanied by enlargement of half of the seventh thoracic vertebra. MRI showed infiltration of lipomatous tissue in the muscles, but no interfascicular accumulation of adipose tissue in the thoracic spinal nerve.
Results
A similar case was presented in 1985 but without MR imaging.
Conclusion
We report on a second case of focal overgrowth of a rib and half of the adjacent vertebra, and overlying lipoma. In addition to the first case, we present MR images demonstrating infiltration of the adipose tissue.
In this Brief Communications Arising Reply, the affiliation for author P. H. Templer was incorrectly listed as 'Department of Ecology & Evolutionary Biology, University of California Irvine, Irvine, ...California 92697, USA' instead of 'Department of Biology, Boston University, Boston, Massachusetts 02215, USA'. This has been corrected online.
This study aims to assess first, whether mutations in the epidermal growth factor receptor (EGFR) and Kirsten rat sarcoma (kRAS) genes are associated with overall survival (OS) in patients who ...present with symptomatic bone metastases from non-small cell lung cancer (NSCLC) and secondly, whether mutation status should be incorporated into prognostic models that are used when deciding on the appropriate palliative treatment for symptomatic bone metastases.
We studied 139 patients with NSCLC treated between 2007 and 2014 for symptomatic bone metastases and whose mutation status was known. The association between mutation status and overall survival was analysed and the results applied to a recently published prognostic model to determine whether including the mutation status would improve its discriminatory power.
The median OS was 3.9 months (95% confidence interval (CI) 2.1 to 5.7). Patients with EGFR (15%) or kRAS mutations (34%) had a median OS of 17.3 months (95% CI 12.7 to 22.0) and 1.8 months (95% CI 1.0 to 2.7), respectively. Compared with EGFR-positive patients, EGFR-negative patients had a 2.5 times higher risk of death (95% CI 1.5 to 4.2). Incorporating EGFR mutation status in the prognostic model improved its discriminatory power.
Survival prediction models for patients with symptomatic bone metastases are used to determine the most appropriate (surgical) treatment for painful or fractured lesions. This study shows that NSCLC should not be regarded as a single entity in such models. Cite this article:
2017;99-B:516-21.
To aid in therapy selection for patients with spinal bone metastases (SBM), predictive models have been developed. These models consider SBM from breast cancer a positive predictive factor, but do ...not take phenotypes based on estrogen (ER), progesterone (PR) and human epidermal growth factor 2 (HER2) receptors into account. The aim of this study was to ascertain whether receptors are associated with survival, when the disease has progressed up to SBM. All patients who were treated for SBM from breast cancer between 2005 and 2012 were included in this international multi-center retrospective study (n = 111). Reports were reviewed for ER, PR and HER2 status and subsequently subdivided into one of four categories; luminal A, luminal B, HER2 and triple negative. Survival time was calculated as the difference between start of treatment for SBM and date of death. Analysis was performed using the Kaplan–Meier method and log-rank tests. Median follow-up was 3.7 years. Survival times in the luminal B and HER2 categories were not significantly different to the luminal A category and were joined into a single receptor positive category. Eighty-five patients (77 %) had a receptor positive phenotype and 25 (23 %) had a triple negative phenotype. Median survival time was 22.5 months (95 %CI 18.0–26.9) for the receptor positive category and 6.7 months (95 %CI 2.4–10.9) for the triple negative category (p < 0.001). Patients with SBM from breast cancer with a triple negative phenotype have a shorter survival time than patients with a receptor positive phenotype. Models estimating survival should be adjusted accordingly.
The purpose of this retrospective study was to differentiate between the MRI features of normal post-operative change and those of residual or recurrent disease after intralesional treatment of an ...atypical cartilage tumour (ACT)/grade I chondrosarcoma.
We reviewed the case notes, radiology and histology of 75 patients, who had been treated for an ACT/grade I chondrosarcoma by curettage, phenolisation and bone allografting between 1994 and 2005. The first post-operative Gd-enhanced MRI scan was carried out within one year of surgery. Patients had a minimum of two scans and a mean follow-up of 72 months (13 to 169). Further surgery was undertaken in cases of suspected recurrence.
In 14 patients (18.6%) a second procedure was undertaken after a mean period of 59 months (8 to 114). Radio frequency ablation (RFA) was used in lesions of < 10 mm and curettage, phenolisation and bone grafting for those ≥ 10 mm. Only six of these (8% of total) had a histologically-proven recurrence. No increase in tumour grade was seen at time of recurrence.
Based on this study, we have been able to classify the post-operative MRI appearances into four groups. These groups differ in follow-up, and have a different risk of recurrence of the lesion. Follow-up and treatment vary for the patients in each group. We present a flow diagram for the appropriate and safe follow-up for this specific group of patients. Cite this article: Bone Joint J 2016;98-B:1674-81.
Background: Inhaled corticosteroids (ICS) provide short term benefits in asthma but the long term effects are still unknown. Methods: 281 patients diagnosed with moderate to severe asthma in 1963–75 ...were re-examined in 1991–9. Information was collected on forced expiratory volume in 1 second (FEV1), bronchial hyperresponsiveness, atopy, smoking, use and dosage of oral and ICS. Patients were included in the analyses if they had at least three FEV1 measurements during two consecutive years after the age of 30 and used ICS during follow up. Results: Analyses were performed on 122 patients. During a median follow up period of 23 years, 71 men and 51 women had on average 37 and 40 individual FEV1 measurements, respectively. Linear mixed effect models showed that men had a mean annual decline in FEV1 of 20.6 ml/year less after ICS initiation than before (p = 0.011), and in women the decline in FEV1 was 3.2 ml/year less (p = 0.73). In individuals with <5 pack years of smoking the decline in FEV1 was 36.8 ml/year less after ICS institution in men (p = 0.0097) and 0.8 ml/year less in women (p = 0.94), the difference between the sexes being significant (p = 0.045). These effects were not observed in those with ⩾5 pack years smoking. A higher daily dose of ICS was associated with a smaller decline in FEV1 in men (p = 0.006), an effect not observed in women. Conclusion: Treatment with ICS in adult patients with moderate to severe asthma was associated with a reduction in the decline in FEV1 over a 23 year follow up period in men who had smoked <5 pack years. This effect was dose dependent and was not present in women or in men with ⩾5 pack years of smoking at follow up. The lack of effect of ICS on the decline in FEV1 in women needs further study.
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