gms | German Medical Science

Introduction: The co-existence of tumors of the nervous system (CNS) and symptomatic hydrocephalus is frequent and can cause severe clinical problems, although, the issue is underestimated in the literature.

Materials and methods: We have reviewed our database on cases with CNS tumors and symptomatic hydrocephalus. We present challenging cases and the first attempt of a clinical classification. A review of the literature is given.

Results: We found 4.6% of patients with central nervous system tumors to suffer from symptomatic hydrocephalus. Vice versa 12.5% of permanent cerebro-spinal fluid diversion procedures were performed in patients with CNS tumors. In general, patients with CNS tumors and hydrocephalus can be classified by the individual impact of tumor and hydrocephalus on treatment and prognosis: (1) surgical tumor removal is sufficient in treating the tumor and the hydrocephalus (which is the case in most infratentorial tumors and tumors along the CSF pathway). (2) Tumor-induced hydrocephalus is treated while the tumor itself does not require surgical treatment (tectal glioma, pineal region germinoma). (3) The tumor and the hydrocephalus need specific surgical treatment what can be challenging: raised CSF protein content may complicate shunt insertion. In cases of meningeal spread the diagnosis may be hardened when CSF puncture does not reveal malignant cells – open meningeal biopsy may be needed. Hydrocephalus may be temporary, as observed in a cases of tumor-related subarachnoid bleeding and diffuse astrocytoma with leptomeningeal spread.

Conclusion: In 4–5% of patients with tumors of the CNS surgical treatment for associated hydrocephalus is needed. Differential therapeutic approaches are required including microneurosurgery, neuroendoscopy, reservoir placement, serial CSF puncture and modern shunt valve technology.