Background Resection of colorectal cancer (CRC) metastases provides good survival but is probably underused in real-world practice. Methods A prospective Finnish nationwide study enrolled treatable metastatic CRC patients. The intervention was the assessment of resectability upfront and twice during first-line therapy by the multidisciplinary team (MDT) at Helsinki tertiary referral centre. The primary outcome was resection rates and survival. Findings In 2012–2018, 1086 patients were included. Median follow-up was 58 months. Multiple metastatic sites were present in 500 (46%) patients at baseline and in 820 (76%) during disease trajectory. In MDT assessments, 447 (41%) were classified as resectable, 310 (29%) upfront and 137 (18%) after conversion therapy. Six-hundred and ninety curative intent resections or local ablative therapies (LAT) were performed in 399 patients (89% of 447 resectable). Multiple metastasectomies for multisite or later developing metastases were performed in 148 (37%) patients. Overall, 414 liver, 112 lung, 57 peritoneal, and 107 other metastasectomies were performed. Median OS was 80·4 months in R0/1-resected (HR 0·15; CI 95% 0·12–0·19), 39·1 months in R2-resected/LAT (0·39; 0·29–0·53) patients, and 20·8 months in patients treated with “systemic therapy alone” (reference), with 5-year OS rates of 66%, 40%, and 6%, respectively. Interpretation Repeated centralized MDT assessment in real-world metastatic CRC patients generates high resectability (41%) and resection rates (37%) with impressive survival, even when multisite metastases are present or develop later. Funding The funders had no role in the study design, analysis, and interpretation of the data or writing of this report.

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Neurofibromatosis 1 (NF1, von Recklinghausen's disease) is an autosomal dominant neurocutaneous-skeletal syndrome in which low bone mineral density (BMD) and osteoporosis are common. Low BMD is, however, not the sole component of fracture risk. In the current study, 460 Finnish patients with NF1 were identified from the hospital medical records and their fracture risk was evaluated. The control population included 3988 appendectomy patients whose age and gender distribution was similar to that of the NF1 patients. Medical records of NF1 and control cohorts were screened for fractures according to the International Statistical Classification of Diseases and Related Health Problems, 10th Revision (ICD-10) between January 2000 and October 2011. The results show that patients with NF1 had increased age-dependent fracture risk compared to controls. Specifically, patients with NF1 aged 41 years and older had a risk ratio of Â5.2 for fractures compared to controls, and children with NF1 had a Â3.4 risk ratio for fractures compared to children without NF1. In contrast, the fracture risk was not increased in NF1 patients aged 17 to 40 years. When fractures not traditionally related to osteoporosis such as fractures of fingers, toes, and skull were excluded, the results were essentially the same. No gender related differences were observed. In conclusion, patients with NF1 have increased fracture risk depending on age. We recommend considering prophylactic measures, such as lifestyle advice, to prevent fractures from occurring. ß

Neurofibromatosis type 1 (NF1) is an autosomal-dominant neuro-cutaneous-skeletal syndrome. Neurofibromatosis type 1 is one of the Rasopathies, and at the cellular level NF1 results in a hyperactive Ras pathway. In the current investigation, our aim was to study lateral skull X-rays (cephalograms) to assess NF1-related craniofacial morphology. A total of 85 Finnish patients with NF1, including four patients with plexiform neurofibroma of the 5th cranial nerve, and their age- and gender-matched controls, were enrolled in the study. The results showed that patients with NF1 typically had a short mandible, maxilla, and cranial base compared with healthy controls, irrespective of age, but the results were statistically significant only in adults. The length of the mandible, the maxilla and the cranial base correlated with the height of patients under 19 yr of age, but this correlation was absent in adult patients. Thus, a tall adult patient with NF1 may have short jaws and a short cranial base. In conclusion, the NF1 gene apparently influences the growth of craniofacial bones, thus contributing to the craniofacial morphology in NF1.