Intracranial Germinoma Presenting With Hemiatrophy- Follow-up Results and Literature Review -Two Case Reports-

Abstract

Two boys aged 8 and 12 years presented with intracranial germinoma manifesting as cerebral hemispheric atrophy, hemiparesis, and decline in academic performance. First magnetic resonance (MR) imaging showed no enhanced lesions, but follow-up MR imaging obtained 12 or 23 months after the first visit showed new mass lesions in the temporal lobe or pituitary gland. Biopsy of these lesions resulted in a histological diagnosis of germinoma. Chemoradiation therapy produced complete remission but both patients continued to manifest mild hemiparesis and impaired cognitive function. Review of 18 previous and our 2 patients presenting with hemispheric atrophy revealed strong male predominance (18:2), mean age of 12.8 ± 5.2 (standard deviation) years, hemiparesis in all patients, and cognitive decline in 9 patients. Initial MR imaging detected mass lesions in only 9 patients, but follow-up MR imaging performed 10-38 months later detected new mass lesions in 5 patients. Radiation with/without chemotherapy provided good tumor control in all patients, but none of the 9 patients with cognitive dysfunction recovered their intellectual ability. We recommend early diagnosis based on biopsy of the atrophic site and timely treatment to improve the quality of life in these patients.

Radiologic features of primary intracranial ectopic germinomas

“…[10,23,30] Our imaging findings most likely provide evidence for this clinical problem. Aggravated ipsilateral atrophy of the brain and cerebral peduncle can be observed in the patient who underwent longitudinal MR studies over 2 years.…”

“…[7–9] Some reports have suggested that the hemiatrophy was the imaging feature of germinomas arising from the basal ganglia and thalamus. [2,10,11] The explanation for this sign was attributed to the infiltration of white matter tracts by the tumors. [8] However, to our knowledge, there is no report using diffusion tensor imaging (DTI) to visualize the involved white matter fiber tracts.…”

Intracranial germinoma causing cerebral haemiatrophy and hypopituitarism

“…Intracranial germinomas that are off-midline (mainly basal ganglia) may present as progressive hemispheric dysfunction 6. Most present with haemiparesis and about half of patients develop cognitive decline.…”

“…Pineal tumours occur twice as often as suprasellar germ cell tumours, with a 2:1–3:1 male preponderance. Intracranial germinomas arise in off-midline regions, such as the basal ganglia, thalamus and cerebral hemispheres, in 4%–20% of cases 6. Pineal lesions usually present with early signs and symptoms of raised intracranial pressure from obstructive hydrocephalus, such as headache, papilloedema and altered mental status.…”

İntrakranyal Olgu Sunumlari

“…Kalsifikasyon nadirdir. Özellikle erken dönemde belirgin kontrastlanma görül-meyebilir [8]. MRS'de laktat-lipid piki, NAA/ kreatin oranında azalma görülür.…”