ABSTRACT
Tumors located in the brainstem are difficult to diagnose initially because of their fluctuating and frequently indistinct symptomatology. There are difficulties in correlating imaging characteristics with histological and biological features because of clinical controversies surrounding the risks and benefits of diagnostic biopsy within the brainstem. They are difficult to treat, as surgery has a role limited to the relief of raised intracranial pressure in some situations and tumor resection in a small and specific minority. Adjuvant therapy with radiation is curative in the minority and therefore is only palliative in the remainder. Apart from steroids, chemotherapy and other drug treatments are given only within clinical trials, which have yet to identify an effective agent or combination of agents in this major anatomical tumor subgroup (diffuse intrinsic pontine). Steroids can provide temporary relief of the signs and symptoms at presentation, but their sustained use leads inevitably to worsening disability and distressing side effects, including personality change. These facts, when put together for a child and family facing up to the diagnosis, provoke strong emotion, which can lead to disbelief and an overwhelming urge to seek alternative opinions and treatments. Skills in communication therefore are central to the management of a child with a tumor
in this region of the brain. Most of all, however, reliable selection of patients for whom there is effective treatment, coupled with the development of research strategies to investigate tumor biology and novel therapies for whom there are no current effective treatments, must be the goal shared by those who are referred these patients for specialist advice.
