ABSTRACT
Brain metastases from non-small cell lung cancer (NSCLC) are associated with a poor prognosis. In selected cases, surgical resection of brain metastases may be indicated, but the identification of patients suitable for surgery remains difficult. We collected data on patient and tumour characteristics known or suspected to be associated with survival by chart review. Data was merged with available data from the local cancer registry. We identified 64 NSCLC patients with resected brain metastases. Median overall survival after resection was 9.1 months with only two patients (3%) surviving more than 71 and 80 months. One and 2-year survival were 42 and 12.5%. Median survival for males and patients with more comorbidities was shorter (8 vs. 10 months [p = 0.11] and 6 vs. 9 months [p = 0.06]). Patients with squamous cell carcinomas (33% of the patients) had a significantly worse survival than patients with other histologies (7 vs. 10 months [p = 0.02]) with no patient living longer than 2 years. Squamous cell histology was associated with worse prognosis after resection of brain metastases in patients with non-small cell lung cancer. Histology, among other parameters, may also be taken into account when choosing the appropriate patients for resection of brain metastases.
Subject(s)
Brain Neoplasms/secondary , Brain Neoplasms/surgery , Carcinoma, Non-Small-Cell Lung/pathology , Carcinoma, Squamous Cell/pathology , Lung Neoplasms/pathology , Aged , Brain Neoplasms/mortality , Carcinoma, Non-Small-Cell Lung/mortality , Carcinoma, Squamous Cell/mortality , Combined Modality Therapy , Comorbidity , Female , Humans , Lung Neoplasms/mortality , Male , Middle Aged , Prognosis , Proportional Hazards Models , Retrospective Studies , Time Factors , Treatment OutcomeABSTRACT
Von Hippel-Lindau (VHL) disease is an autosomal dominantly inherited tumour predisposition syndrome with an incidence of 1:36,000 newborns, the estimated prevalence in Europe is about 1-9/100,000. It is associated with an increased risk of developing various benign and malignant tumours, thus affecting multiple organs at different time points in the life of a patient. Disease severity and diversity as well as age at first symptoms vary considerably, and diagnostic delay due to failure of recognition is a relevant issue. The identification of a disease-causing VHL germline mutation subsequently allows family members at risk to undergo predictive genetic testing after genetic counselling. Clinical management of patients and families should optimally be offered as an interdisciplinary approach. Prophylactic screening programs are a cornerstone of care, and have markedly improved median overall survival of affected patients. The aim of this review is to give an overview of the heterogeneous manifestations of the VHL syndrome and to highlight the diagnostic and therapeutic challenges characteristic for this orphan disease. A comprehensive update of the underlying genetic and molecular principles is additionally provided. We also describe how the St. Gallen VHL multidisciplinary group is organised as an example of interdisciplinary cooperation in a tertiary hospital in Switzerland.
