Giant-cell-rich pseudotumour in Paget's disease.

Paget's disease (PD) of the bone is a disorder of bone remodelling that may be polyostotic or monostotic. Although development of a sarcoma in PD is well-recognised, it is less well recognised that pseudosarcomas in bone and soft tissue can also arise in this condition. In this report we document the case of a large giant-cell-rich pseudotumour that developed in the tibia and overlying soft tissues in a case of polyostotic PD. Bone and soft tissues were highly vascular and contained abundant haemorrhage with focal areas of new bone formation and a diffuse infiltrate of osteoclastic giant cells. The lesion has not recurred or produced metastases 3 years after removal. Clinicians should be aware that a benign giant-cell-rich pseudotumour can develop in PD and that it needs to be distinguished from other giant-cell-rich tumours.

Improvement in psychological wellbeing among adolescents with a substance use disorder attending an outpatient treatment programme.

OBJECTIVES: Adolescents with substance use disorders (SUDs) exhibit high rates of comorbid psychological problems. This study aimed to examine the impact of an outpatient substance use treatment programme upon the psychological wellbeing of adolescents. METHODS: A prospective study was carried out examining psychological symptoms in a group of adolescents attending the Youth Drug and Alcohol (YoDA) Addiction Service in Dublin. Participants were treated with evidenced based psychological models such as cognitive behavioural therapy, motivational interviewing and systemic family therapy. The Becks Youth Inventory was utilised to assess psychological symptoms at treatment entry and repeated three months later at follow up. RESULTS: Among 36 adolescents who were included in this study, poly-substance misuse was the norm. Almost three-quarter had a cannabis use disorder (CUD). There were significant reductions in mean subscale scores of depression (56.0 to 50.8, p = 0.003), anger (55.2 to 49.5, p < 0.001) and disruptive behaviour (61.6 to 56.5, p = 0.002) at follow up. Although there wasn't a statistically significant reduction in mean scores for anxiety, we observed a significant proportion of participants (p = 0.008) improving and moving out of a moderate to severe symptom range when examined by category. This was also the case for self-concept (p = 0.04). Furthermore this study revealed a positive correlation between the reduction in days of cannabis use and reduction in depressive scores (Pearson correlation 0.49, p = 0.01) among those with a CUD. CONCLUSION: The findings indicate that substance use treatment for adolescents is associated with important psychological and behavioural improvements.

Locally aggressive fibrous dysplasia.

Although fibrous dysplasia (FD) is a benign fibro-osseous lesion, locally aggressive behaviour has rarely been described but is poorly characterised. In this study, we document clinical, radiological and pathological (including molecular genetics) findings in three cases of locally aggressive FD, two of which involved the ribs. Lesions in these cases, one of which was a recurrent lesion, were followed up for 2-7 years. All of the lesions showed typical histological features of FD but were characterised by extension through the bone cortex into the extra-osseous soft tissue. The lesions did not exhibit overexpression/amplification of CDK4 and MDM2; in two of the cases, a GNAS mutation was identified. Our findings confirm that FD can rarely exhibit locally aggressive behaviour with extension beyond the bone compartment into the surrounding soft tissue; these lesions can be distinguished from low-grade intramedullary osteosarcoma by lack of amplification/overexpression of CDK4 and MDM2 and the presence of a GNAS mutation.