Ependymoblastoma is considered to be a primitive malignant glioma with ependymal differentiation. This rare tumor occurs in very early life and shows rapid growth and a diffuse infiltration through the leptomeningeal space. The tumor cells are highly immature, with numerous mitoses and multilayered ependymal rosettes. The ependymoblastoma described in this report was found in a 17-year-old girl. In spite of detailed clinical and postmortem examinations, no definite primary site was identified in the neuraxis. The lesion spread predominantly throughout the leptomeningeal space. Histological analysis strongly suggested that this tumor originated from a heterotopic glial nest in the subarachnoid space. The absence of immunohistochemical neural tissue markers, glial fibrillary acidic protein, S-100 protein, neuron-specific enolase, and neurofilaments ruled out neuronal or glial differentiation. The authors were unable to find any previous report of primary leptomeningeal ependymoblastoma.
Tumor resection is recommended in anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis, however it is often difficult during an early stage of the disease. We report here the efficacy of early tumor removal in a patient with anti-NMDAR encephalitis. This 21-year-old woman was admitted to another hospital with rapidly progressive psychiatric symptoms, a decreased level of consciousness, and seizures. Abdominal CT showed a pelvic mass. On day 1 of admission to our center, she developed hypoventilation requiring mechanical support. She had orofacial dyskinesias with well-coordinated, pseudo-piano playing involuntary finger movements. Based on these clinical features, she was immediately scheduled for tumor resection on day 3. While awaiting surgery, she began to receive high-dose intravenous methylprednisolone. After tumor removal, she received plasma exchange, followed by intravenous immunoglobulin and additional high-dose methylprednisolone. Two weeks after tumor removal, she started following simple commands and progressive improvement, although she remained on mechanical ventilation for 10 weeks due to nocturnal central hypoventilation. Anti-NMDAR antibodies in serum/CSF were detected. Pathological examination showed immature teratoma with foci of infiltrates of B- and T-cells. Early tumor resection with immunotherapy facilitates recovery from this disease, but central hypoventilation may require long mechanical support. Non-jerky elaborate finger movements suggest antibody-mediated disinhibition of the cortico-striatal systems.
A 69-year-old man was admitted semicomatose with high-grade fever and meningeal signs. Magnetic resonance imaging showed a supra-and intrasellar lesion. Hormone studies on admission showed increased serum prolactin, adrenocorticotropic hormone (ACTH), and cortisol titers. However, the serum ACTH and cortisol levels returned to normal after treatment of meningitis with an antimicrobial agent. The histological diagnosis was pituitary adenoma. Immunohistological staining showed positive reaction for prolactin but not for ACTH. This is a rare case of prolactinoma with a high serum ACTH level caused by meningitis.
A 48-year-old woman was admitted to our institution complaining of headache and a 2-month history of blurred vision and increased urinary volume. T 1 -weighted magnetic resonance (MR) imaging with gadolinium showed a ring-enhanced mass lesion in the pituitary. Fluid-attenuated inversion recovery MR imaging showed high-intensity lesions in the optic nerves, hypothalamus, and thalamus. The histological diagnosis was pituitary abscess. The blurred vision was caused by inflammation, but not compression, of the optic nerves due to the pituitary abscess outside the hypophysis.
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