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CNS germ cell tumor (CNSGCT) of childhood

Presentation and delayed diagnosis

From the Comprehensive Pediatric Brain Tumor Program, Children's National Medical Center, and The Departments of Neurology, Pediatrics, Neurosurgery, Pathology, and Radiology, The George Washington University, Washington, DC.

Address correspondence and reprint requests to Dr. John R. Crawford, Department of Neurology, Children's National Medical Center, 111 Michigan Ave NW, Washington, DC 20010 jcrawfor{at}cnmc.org

Objective: To describe the relationship between symptomatology and time to diagnosis of an institutional series of patients with CNS germ cell tumor (CNSGCT) over a 16-year period.

Methods: Thirty consecutive patients newly diagnosed with CNSGCT (mean age 10.9 years; range 6 to 17 years; 70% boys) were evaluated at our institution between 1990 and 2006.

Results: Duration of symptoms prior to diagnosis ranged from 5 days to 3 years (mean 8.4 months). Tumor location included pineal (14), suprasellar (8), pineal/suprasellar (3), pineal/thalamic (4), and basal ganglionic/thalamic (3). Five patients had disseminated disease at the time of diagnosis. Features including headache, nausea, vomiting, and visual changes led to earlier diagnosis. Symptoms including movement disorders, enuresis, anorexia, and psychiatric complaints delayed diagnosis in 9 of 30 patients, diagnosed 7 months to 3 years (mean 22.3 months) from symptom onset. In 7 of 9 patients with delayed diagnosis, enuresis was present. Seventeen of 30 patients had signs of endocrine dysfunction at presentation that included diabetes insipidus (4), hypothyroidism (8), and growth hormone deficiency (4). Ophthalmologic findings of decreased visual acuity, visual field deficits, or ocular abnormalities were present in 13 patients. Duration of symptoms did not correlate with tumor subtype or event-free survival. In three patients with basal ganglionic/temporal lobe, thalamic, or pineal/suprasellar signal abnormalities on MRI, neuroradiographic diagnosis was difficult.

Conclusions: Diagnosis of CNS germ cell tumor is often delayed, and presentation may include movement disorders or mimic psychiatric disease. MRI interpretation can be challenging and may require serum/CSF markers and biopsy for diagnosis.

This work was supported in part by the Neurological Sciences Academic Development Award (NSADA) K12 NS052 159-01A1.

Disclosure: The authors report no conflicts of interest.

Received August 21, 2006. Accepted in final form January 9, 2007.

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