Classic adamantinoma is an ultra rare bone cancer typically and exclusively arising in the bones of the lower leg. It is microscopically very special as it does not only contain bone, but also contains epithelium. This is unusual, since epithelium is usually only found at the surface of tissues, like in the skin, and not in bone. Thus, the tumour is composed of two different components: bone and epithelium. There are also two less aggressive bone tumour types that specifically occur in the bones of the lower leg, especially in children and young adults, called osteofibrous dysplasia and osteofibrous dysplasia like adamantinoma.
In contrast to classic adamantinoma, these two lesions contain much less epithelium, and do not behave as a cancer. The three tumour types within this tumour family are challenging to diagnose as it can be difficult to distinguish them clinically, radiologically and microscopically. Therefore leading scientists think they are related in some way.
A research project has been awarded to Professor Judith Bovée at the University of Leiden (The Netherlands) to better understand these differences. To do this, they would need to know how the two components in these tumours influence each other, as this is currently unknown. This is important, since patients would benefit from more clearly defined microscopic and/or molecular criteria as this would improve the diagnostic accuracy, outcome prediction and treatment planning.
What are the aims of this research project?
This project aims at understanding the interaction between the two components (epithelium and bone) that makes this tumour family unique and different from other bone cancers. By better understanding the interaction, the research team aims to develop more robust criteria that can be used by pathologists when diagnosing these tumours microscopically, or molecularly. This way, diagnostic accuracy, outcome prediction and treatment planning will be improved.How could this project improve treatment options for adamantinoma patients?
Treatment planning can be improved if we become better in diagnosing and predicting the behaviour of these tumours. Our discoveries could then help doctors to make a better-informed decision on the proper treatment strategy. This is especially relevant for the younger patient group who may face either spontaneous regression, allowing a wait- and-see strategy (in case of osteofibrous dysplasia) or growth with multiple recurrences, requiring more aggressive complete removal of the tumour (in case of osteofibrous dysplasia-like adamantinoma). In case of classic adamantinoma, which is more often seen in adults, the adamantinoma can spread to other parts of the body (metastasize). If we unravel the mechanism by which the two components influence each other, we can use this knowledge to design new ways to treat these patients with classic adamantinoma that cannot be operated or that develop metastases.
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