gms | German Medical Science

Objective: Pineal region tumors present 1% of all intracranial neoplasms and 3% - 11% of intracranial tumors in children. The diagnosis of pineal region tumors is often prolonged because of the wide variability of symptoms especially in the early phase of this disease caused by intermittent hydrocephalus, congestion of central veins or mass effects. The aim of this review is to detect generic signs or combined symptoms allowing a specific and shortened diagnosis.

Methods: 67 cases, 34 of them were children, of pineal region tumor treated by surgery at our neurosurgical department between 1987 and 2010 were retrospectively reviewed. Medical reports were analyzed for multiple patient characteristics including histological diagnosis, age at time of diagnosis, postoperative outcome, symptoms leading to diagnosis and time to diagnosis as well as first presenting symptoms and time to diagnosis. Early and late onset of symptoms were correlated to age, gender, histological diagnosis and presenting symptoms.

Results: In 67 patients a histological diagnosis was made. Most of the tumors were benign cysts (22%), germinomas (19%), pilocytic astrocytomas (17%), pineoblastomas (13%) and pineocytomas (12%). The median age at the time of diagnosis was 23 years with a wide range form 0 years to 72 years. Mortality was 4%. Most early onset symptoms were headache (67%), diminished visual acuity (47%), ataxia (32%) and deficits in cognition (32%). In nearly all cases, these symptoms occurred in combination and intermittently. The mean time between first presenting symptoms to diagnosis was 6 months with a range from 2 weeks to 10 years. The median time for symptoms leading to the diagnosis was 5 days. There was no significance between occurance of symptoms and age, gender, histological diagnosis or presenting symptoms.

Conclusions: In our retrospective patient collective, the time from the first symptoms to the diagnosis in pineal region tumors is mostly unsatisfactory. There were no significant correlations between occurance of first symptoms and other patient characteristics. A common factor as a key to shortened diagnosis seems to be the combined and intermittent presentation of symptoms in the early stadge. Detailed information and involvement of the outpatient sector, especially of pediatricians could be helpful in identifying pineal region tumors much earlier.