Optic Nerve Germinoma and Transient Spontaneous Regression—More Than Meets the Eye

Abstract

Infiltration of the optic pathway by germ cell tumors is exceptional and can lead to confusion with glioma or inflammatory conditions. We present the case of a 14-year-old girl with an optic nerve germinoma extending to the hypothalamus and manifesting as panhypopituitarism and visual loss. The patient experienced spontaneous regression of the lesion followed by secondary deterioration requiring treatment. Four other cases of spontaneously regressing intracranial germinoma followed by regrowth have been reported in the literature. This report highlights the importance of clinical and radiologic monitoring of intracranial germinoma, even in the event of initial spontaneous improvement.

Primary anterior visual pathway germinoma in a 13-year-old boy: A case report

“…[2] According to our literature search, there are 12 reported cases of primary anterior visual pathway germinoma, most of these cases were male, three cases were female [Table 1]. [2][3][4]6,8,9,12,16,[20][21][22] e mean age of diagnosis of all reported cases including our case was 21 years. e painless progressive vision deterioration was the main complaint of all reported cases.…”

“…[11] Primary germinoma of the optic nerve or chiasm is extremely rare, with limited reported cases in the literature. [2][3][4]6,9,16,[20][21][22] e primary anterior visual pathway germinoma diffusely infiltrates the optic nerve or chiasm, which impacts the prognosis of these cases. [2][3][4]6,9,16,[20][21][22] Distinguishing primary anterior visual pathway germinoma from glioma based on radiologic features has proved to be challenging; moreover, the treatment regimens differ between primary anterior visual pathway germinoma and glioma, hence why tissue diagnosis is crucial.…”

“…[2][3][4]6,9,16,[20][21][22] e primary anterior visual pathway germinoma diffusely infiltrates the optic nerve or chiasm, which impacts the prognosis of these cases. [2][3][4]6,9,16,[20][21][22] Distinguishing primary anterior visual pathway germinoma from glioma based on radiologic features has proved to be challenging; moreover, the treatment regimens differ between primary anterior visual pathway germinoma and glioma, hence why tissue diagnosis is crucial. [13,21] e literature demonstrates that intracranial germinoma has favorable outcomes compared to other pediatric malignant tumors.…”

Suprasellar pure germinoma mimicking low-grade glioma on neuroimaging

“…Typical MRI features include either homogeneous or heterogeneous contrast enhancement associated with reduced diffusivity on diffusion-weighted and apparent diffusion coefficient sequences 7. Cases of intracranial germ cell tumours mimicking low-grade gliomas on neuroimaging and specifically when optic pathways are involved have been reported obviating the need for biopsy to establish confirmatory diagnosis 4 5 8–10. Bevacizumab, a humanised monoclonal antibody against vascular endothelial growth factor A, has demonstrated radiographical and clinical response in paediatric low-grade gliomas including those involving the suprasellar region,11–13 even in cases without histological confirmation of low-grade glioma 14.…”