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Case history

image A 4-year-old boy presented with a 4–6 week history of headache, reduced activity levels and unsteadiness on walking. He had been previously well and had had normal developmental milestones. There was nothing of note in his personal, social or family history. His parents were concerned that, although initially attributed to a viral illness, his symptoms had worsened in the last 2 weeks.

Initial investigations revealed a large space-occupying lesion in the posterior fossa, arising from the left cerebellar hemisphere on MRI scan, and early hydrocephalus. Following review among the paediatric oncology multidisciplinary team, the boy underwent surgery and debulking of the tumour. Staging investigations did not reveal evidence of disease outside the brain. Cytology from cerebrospinal fluid (CSF) taken 15 days after surgery was negative for malignant cells.

Histopathological review of the surgical specimen revealed medulloblastoma, described as classical variant. A postoperative MRI undertaken 48 h after surgery suggested no residual enhancing disease.

Eighteen days after resection of the tumour, his parents brought him to the oncology clinic to discuss further treatment. At this consultation the patient remained unsteady but had stable neurology compared with his situation prior to surgery; he had a well-healed scar and no new cerebellar signs.

He received a course of postoperative radiotherapy and concomitant vincristine chemotherapy. Radiotherapy was delivered starting on day 28 after surgery. A total dose of 23.4 Gy was administered to the spine and whole brain, with a further boost dose of 30.6 Gy to the primary site. Combination chemotherapy with vincristine, lomustine and cisplatin was continued for eight cycles. Follow-up MRI scans 6 weeks after radiotherapy revealed no evidence of active disease; the patient was then followed up 6 monthly with MRI scans.

What was the goal of cancer treatment for this patient?

What are the important prognostic factors for this patient and how do they influence the management plan?

How is the molecular pathology of the tumour used to determine treatment?

What is the evidence base for his treatment?

Of what long-term impact of treatment will the patient's family need to be made aware?

What was the goal of cancer treatment for this patient?

The aim of treatment in this case was cure. Medulloblastoma is a curable malignancy in children, with median 5 year survival rates of 50–60% in population-based studies. In patients who survive 5 years, long-term survival is the norm, with cancer-specific survival rates of 80%.1 In these circumstances, minimizing the long-term effect of treatment is an important secondary goal, since treatment side effects may have a lifelong impact. This highlights the importance of individual management decisions to achieve high cure rates while minimizing long-term toxicities in a risk-adapted strategy.

What are the important prognostic factors for this patient and how do they influence the management plan?

Patient, tumour and treatment variables are all important in determining prognosis in medulloblastoma. Age ...

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