Predicting survival of patients with spinal metastatic disease using PathFx 3.0 - A validation study of 668 patients in Sweden.

Introduction: PathFx is a computer-based prediction model for estimating survival of patients with bone metastasis. The model has been validated in several studies, but this is the first validation using exclusively patients with spinal metastases. Research question: Is PathFx 3.0 a tool useful for predicting survival for patients with spinal metastatic disease? Material and methods: 668 patients (67% male, median age 67 years) presenting with spinal metastases at two university hospitals in Sweden 1991-2014 were included. Of those, the majority (82%, n â€‹= â€‹551) underwent surgery. Data on all patients was analyzed with PathFx version 3.0, generating a probability of survival at 1, 3, 6, 12, 18 and 24 months. The predictions were compared to real survival data and the precision in estimation was evaluated with Receiver-Operating Characteristic curve (ROC) analysis where the Area Under Curve (AUC) was calculated. Brier score and decision curve analyses were also assessed. Results: The AUC for 1-, 3-, 6- and 12 months survival predictions were 0.64 (95% CI 0.5-0.71), 0.71 (95% CI 0.67-0.75), 0.70 (95% CI 0.66-0.77) and 0.74 (95% CI 0.70-0.78). For 18- and 24 months survival the AUC were 0.74 (95% CI 0.69-0.78) and 0.76 (95% CI 0.72-0.81). The Brier scores were all 0.23 or lower depending on the estimated survival time. Discussion and conclusion: PathFx 3.0 is a reasonably reliable tool for predicting survival in patients with spinal metastatic disease. As the PathFx computer model can be updated to reflect advancements in oncology, we suggest this type of model, rather than rigid point-based scoring systems, to be used for estimating survival in patients with metastatic spinal disease in the future.

Survival after surgery for spinal metastatic disease: a nationwide multiregistry cohort study.

OBJECTIVES: To evaluate survival after surgery and indications for surgery due to spinal metastatic disease. DESIGN: A retrospective longitudinal multiregistry nationwide cohort study. SETTING: 19 public hospitals in Sweden with spine surgery service, where 6 university hospitals account for over 90% of the cases. PARTICIPANTS: 1820 patients 18 years or older undergoing surgery due to spinal metastatic disease 2006-2018 and registered in Swespine, the Swedish national spine surgery registry. INTERVENTIONS: Decompressive and/or stabilising spine surgery due to spinal metastatic disease. PRIMARY OUTCOME: Survival (median and mean) after surgery. SECONDARY OUTCOMES: Indications for surgery, types of surgery and causes of death. RESULTS: The median estimated survival after surgery was 6.2 months (95% CI: 5.6 to 6.8) and the mean estimated survival time was 12.2 months (95% CI: 11.4 to 13.1). Neurologic deficit was the most common indication for surgery and posterior stabilisation was performed in 70.5% of the cases. A neoplasm was stated as the main cause of death for 97% of the patients. CONCLUSION: Both median and mean survival times were well above the generally accepted thresholds for surgical treatment for spinal metastases, suggesting that patient selection for surgical treatment on a national level is adequate. Further research on quality of life after surgery and prognostication is needed.

Does knowledge of the primary tumour affect survival after surgery for spinal metastatic disease? A retrospective longitudinal cohort study.

OBJECTIVES: To compare survival after surgery for patients with spinal metastatic disease with known primary tumour (KPT) versus patients with unknown primary tumour (UPT). PARTICIPANTS: 393 patients 18 years or older (270 men and 123 women, mean age 67.3 years) undergoing surgery at Uppsala University Hospital in Swedenbetween 2006 and 2016due to spinal metastatic disease . 271 patients (69%) had a KPT at the time of surgery and 122 (31%) had an UPT. INTERVENTIONS: Decompressive and/or stabilising spine surgery due to spinal metastatic disease. PRIMARY OUTCOME: Survival (median and mean) after surgery. RESULTS: The estimated median survival time after surgery for patients with KPT was 7.4 months (95% CI 6.0 to 8.7) and mean survival time was 21.6 months (95% CI 17.2 to 26.0). For patients with UPT, the median estimated survival time after surgery was 15.6 months (95% CI 7.5 to 23.7) and the mean survival time was 48.1 months (95% CI 37.3 to 59.0) (Breslow, p=0.001). Unknown primary cancer was a positive predictor of survival after surgery (Cox regression, HR=0.58, 95% CI 0.46 to 0.73). CONCLUSION: In this study, patients with spinal metastasis and UPT had a longer expected survival after surgery compared with patients with KPT. This suggests that patients with UPT and spinal metastasis should not be withheld from surgery only based on the fact that the primary tumour is unknown.

Predictive Scores Underestimate Survival of Patients With Metastatic Spine Disease: A Retrospective Study of 315 Patients in Sweden.

STUDY DESIGN: Retrospective cohort study. OBJECTIVE: To validate the precision of four predictive scoring systems for spinal metastatic disease and evaluate whether they underestimate or overestimate survival. SUMMARY OF BACKGROUND DATA: Metastatic spine disease is a common complication to malignancies. Several scoring systems are available to predict survival and to help the clinician to select surgical or nonsurgical treatment. METHODS: Three hundred fifteen adult patients (213 men, 102 women, mean age 67 yr) undergoing spinal surgery at Uppsala University Hospital, Sweden, due to metastatic spine disease 2006 to 2012 were included. Data were collected prospectively for the Swedish Spine Register and retrospectively from the medical records. Tokuhashi scores, Revised Tokuhashi Scores, Tomita scores, and Modified Bauer Scores were calculated and compared with actual survival data from the Swedish Population Register. RESULTS: The mean estimated survival time after surgery for all patients included was 12.4 months (confidence interval 10.6-14.2) and median 5.9 months (confidence interval 4.5-7.3). All four scores had significant correlation to survival (P < 0.0001) but tended to underestimate rather than overestimate survival. Modified Bauer Score was the best of the four scores to predict short survival, both regarding median and mean survival. Tokuhashi score was found to be the best of the scores to predict long survival, even though the predictions were inaccurate in 42% of the cases. CONCLUSION: Predictive scores underestimate survival for the patients which might affect important clinical decisions. LEVEL OF EVIDENCE: 3.

Lattice corneal dystrophy, gelsolin type (Meretoja's syndrome).

PURPOSE: This paper reviews current knowledge about the pathogenesis, clinical manifestations and treatment of lattice corneal dystrophy, gelsolin type (LCD2, Meretoja's syndrome). METHODS: Material is derived from literature searches, a case study of a Finnish patient living in Sweden, and interviews in Helsinki with Professor Ahti Tarkkanen and Dr Sari Kiuru-Enari, both of whom have extensive first-hand experience in treating patients with the disease. RESULTS: The disease is now reported from several countries in Europe, as well as Japan, the USA and Iran. Treatment is symptomatic and is based on eye lubrication combined with rigorous monitoring of intraocular pressure to reduce corneal haze and postpone the need for keratoplasty. When systemic symptoms occur, the ophthalmologist should consult other specialists. CONCLUSIONS: The disease is probably under-reported and is almost certainly to be found in more countries, including Sweden. Every ophthalmologist should be vigilant and consider this diagnosis when discovering a corneal lattice dystrophy, especially because the disease is an inherited, lifelong chronic condition with systemic symptoms.