A Japanese infant presenting with hypocalcemic seizures resulting from hypovitaminosis D induced by non-celiac gluten sensitivity.

Vitamin D deficiency is a major cause of hypocalcemic seizures in infants. Chronic enteropathy can cause both malnutrition and vitamin deficiency disorders, such as celiac disease, in Western Caucasians. However, gluten-related disorders are considered uncommon in most Asian countries, and there have been no reports of any infant being diagnosed with a gluten-related disorder in Japan. Here, we describe a case where a Japanese infant, with non-celiac gluten sensitivity, presented with hypocalcemic seizures resulting from a vitamin D deficiency. In this case, an eight-month-old boy had an afebrile seizure, and blood tests revealed both hypocalcemia and prolonged prothrombin time resulting in his transfer to our hospital. The presence of fatty stools and evidence of multiple vitamin deficiencies indicated some form of fat malabsorption. His laboratory and histological findings showed enteropathy, and he was thus diagnosed with non-celiac gluten sensitivity. Therefore, he was treated with a gluten-free diet supplemented with vitamins. This case suggests that infants with a vitamin D deficiency caused by celiac disease or non-celiac gluten sensitivity should be carefully monitored when they are given oral supplements of vitamin D, to prevent any adverse side-effects associated with the varied roles of vitamin D in the immune response.

Pathological and clinical features of cystic and noncystic glioblastomas.

The aim of this study is to review the different histological and clinical characteristics of glioblastoma multiforme (GBM) with and without cysts (cystic and noncystic GBM, respectively). Thirty-seven GBM were collected; these were tumors for which more than 80% of the volume was surgically resected, including a portion of the peripheral parenchyma of the brain. Based on preoperative magnetic resonance (MR) imaging studies, tumors were tentatively classified as cystic GBM if more than 50% of their volume appeared to be liquid; otherwise, they were considered to be noncystic GBM. Tumor volumes were estimated from contrast-enhanced T1-weighted MR images. Edema was deduced from the maximum width of contrast-enhanced edges. Peritumoral pathological analysis showed distinct margins, indicating little or no infiltration of tumor cells into white matter. Five cases were classified as cystic and 32 were noncystic GBMs. There was a statistically significant difference in age (Mann-Whitney U test; P < 0.05) between the patients with cystic tumors (median, 44 years; range, 26-59 years) and those with noncystic tumors (median, 54 years; range, 26-81 years). Four of the cystic tumors and eight of the noncystic tumors were more than 5 cm in maximum diameter. Cystic GBMs had a well-defined tumor interface and less than 2-cm-thick peritumoral edema compared to the noncystic GBMs (Fisher's exact test; P < 0.05). For patients with cystic GBMs, median survival time after surgery was 19.8 months and the 2-year survival rate was 50%. Patients with noncystic GBMs had a median survival time of 12.8 months and a 2-year survival rate of only 17%. Median time to tumor recurrence was 13.3 months for patients harboring cystic GBMs and 8.5 months for those with noncystic GBMs (log-rank test; P < 0.05). Thus, the prognosis for cystic GBM was significantly better than that for noncystic GBM, possibly because cystic GBMs showed comparatively little infiltration of the peritumoral brain parenchyma.

Histological features of intracranial germinomas not disappearing immediately after radiotherapy.

The histological features of germinomas were investigated to differentiate tumors which completely disappear immediately after irradiation and those that persist. Eighteen previously untreated patients underwent germinoma biopsy and irradiation or combined irradiation and chemotherapy. Four tumors were located only in the pineal gland, eight in the suprasellar region, two in multiple locations, one in the basal ganglia, and three in other regions. Histologically, the germinomas could be divided into type A found in 13 cases which consisted mainly of large neoplastic cells and small lymphocytes, the so-called two-cell pattern, and type B found in five cases which consisted predominantly of fibrous tissue and granulomatous reaction containing occasional neoplastic cells. Follow-up computed tomography or magnetic resonance imaging showed the enhanced mass lesion disappeared in all cases of type A germinomas within 1 month after treatment, but persisted in all cases of type B germinomas for at least 1 month. Type B tumors required up to 12 months to show complete radiographic resolution. Persistent germinomas consisted predominantly of fibrous tissue and granulomatous reaction containing occasional neoplastic cells.

Light microscopic demonstration of the microlumen of ependymoma: a study of the usefulness of antigen retrieval for epithelial membrane antigen (EMA) immunostaining.

To determine the origin of dotlike epithelial membrane antigen (EMA) immunoreactivity of ependymoma, which is consistent with the eosinophilic globular body in hematoxylin and eosin (H&E) stain, an immunoelectron microscopic study was undertaken. The usefulness of antigen retrieval pretreatment in detecting the dotlike EMA immunoreactivity in ependymomas was also studied. The materials were 29 ependymomas, 7 autopsy brains as a normal control, and 50 brain tumors of various types. The study confirmed that most of the brown dots in EMA immunostain in ependymoma represented microlumina of tumor cells. In ependymomas, plain EMA immunostaining showed dotlike positivity in only six cases (21%), and antigen retrieval pretreatment increased the number of positives up to 26 cases (90%). Antigen retrieved CD99 detected 23 positive cases (80%) in ependymomas. On the basis of the results, although some false positive findings were raised by antigen retrieval pretreatment, the authors positively recommend adoption of the technique, especially when ependymoma remains as one of the differential diagnoses of the tumor.

Pineal parenchymal tumor with marked retinoblastic differentiation: case report.

A 53-year-old woman was found to have a tumor in the pineal region. Histologically, Homer-Wright rosettes were sporadically distributed in a diffuse proliferation of round tumor cells that were immunoreactive for synaptophysin and chromogranin. A few perivascular pseudorosettes were also present, and the perivascular tumor cells were immunoreactive for glial fibrillary acidic protein and vimentin. By electron microscopy, well-developed junctions and pronounced interdigitation of abutting plasma membranes were noted in many tumor cells, as well as abundant intracytoplasmic microtubules. These findings indicated that the tumor was a pineal parenchymal tumor accompanied by an extraordinary epithelial-like differentiation, suggesting retinoblastic photoreceptor cell differentiation.

Granulofilamentous meningioma.

A 55-year old female was referred to the Department of Neurosurgery, Kitasato Hospital, because of a hearing impairment. Neuroimaging revealed a typical meningioma attached to the falx in the right frontal region. During surgery, an encapsulated, circumscribed, reddish-gray, slightly hard tumor attached to the falx was completely removed by an interhemispheric approach. On light microscopy, many of the tumor cells contained eosinophilic inclusions with single or multiple vacuoles that displaced the cytoplasm. The nuclei of the tumor cells were eccentric. There were no signs of malignancy in the specimen. Electron microscopy revealed that most of the eosinophilic inclusions were composed of filaments measuring 12 nm in diameter. There have been several reports of benign meningiomas with eosinophilic inclusions composed of intermediate filaments. The microscopic differences between these types of tumor and rhabdoid meningiomas are very subtle, and it is important the two types of tumors are not confused. Benign meningiomas with eosinophilic inclusions comprising intermediate filaments, for example the tumor described in this report, have been diagnosed as granulofilamentous meningiomas, which is a subtype of benign meningioma.

Pilocytic astrocytoma with abundant oligodendroglioma-like component.

An 18-year-old girl presented with a history of visual disturbance without headache, nausea, or vomiting in May 2010. In July 2010, the patient visited our hospital because of visual disturbance. Head magnetic resonance images revealed hydrocephalus caused by a ring-enhancing mass lesion located in the vermis. Total tumor removal was performed. Histological findings revealed that honeycomb cells resembling oligodendrocytes accounted for most parts of the tumor. Rosenthal fibers and hyaline droplets were seen in a small portion. The tumor cells were immunoreactive for GFAP and Olig2, but none of the tumor cells were immunoreactive for Symaptophysin, EMA, or IDH 1. according to these findings, the tumor was diagnosed as pilocytic astrocytoma with an abundant oligodendroglioma-like component. Pilocytic astrocytoma is known to be associated with an oligodendroglioma-like component; however, the differential diagnosis for oligodendroglioma may be difficult when an oligodendroglioma-like component occupies most of the tumor.

Subependymal giant cell astrocytoma with oncocytic change.

A 49-year-old woman presented with a history of periodic episodes of nausea and vomiting starting in 2006. In June 2009, the patient lost consciousness and was transported to our hospital. Head computed tomography (CT) revealed hydrocephalus caused by an enhancing mass lesion with calcification located in the right lateral ventricle around the foramen of Monro. Total tumor removal was performed. Histologic findings revealed fibrillated spindle tumor cells and giant tumor cells with abundant cytoplasm. The spindle tumor cells were immunoreactive for GFAP and S-100 protein, but none of the giant tumor cells were immunoreactive for GFAP or S-100 protein. Electron microscopic examination revealed abundant mitochondria in the tumor cell cytoplasm. According to these findings, this tumor was diagnosed as subependymal giant cell astrocytoma (SEGA) with oncocytic change, which is extremely rare.

Primary central nervous system large B-cell lymphoma with prolific, mixed T-cell and macrophage infiltrates, mimicking multiple sclerosis.

Although tissue confirmation is essential for a diagnosis of primary central nervous system large B-cell lymphoma (PCNSBL), accurate assessment may still be difficult, even when tissue is obtained. We report a 59-year-old man, first diagnosed as multiple sclerosis by open biopsy at another institution, who was then correctly diagnosed as PCNSBL after stereotactic biopsy at our hospital. The initial biopsy showed heavy lymphoid and macrophage influx with visible demyelination. On rebiopsy, a diffuse infiltrate of small to medium-sized lymphocytes was prominent and largely stained as T cells (CD3) by immunohistochemistry. There was also an admixture of macrophages, but this time, relatively low numbers of large malignant cells were also identified. The latter stained as B cells (CD20), enabling a diagnosis of B-cell lymphoma, and the condition responded fully to high-dose methotrexate. It is thus possible for PCNSBL to be histologically misinterpreted as a result of ancillary inflammation, characterized here as a profusion of T cells and macrophages.

Neurocysticercosis: assessing where the infection was acquired from.

Histopathological specimen of a neurocysticercosis patient, who had been living in several endemic countries, was retrospectively analyzed for assessing the origin of the infection. Mitochondrial DNA analysis strongly suggested that the patient became infected with the parasite in Nepal at least 10 years before the onset of the disease.

Farside gravity field of the moon from four-way Doppler measurements of SELENE (Kaguya).

The farside gravity field of the Moon is improved from the tracking data of the Selenological and Engineering Explorer (SELENE) via a relay subsatellite. The new gravity field model reveals that the farside has negative anomaly rings unlike positive anomalies on the nearside. Several basins have large central gravity highs, likely due to super-isostatic, dynamic uplift of the mantle. Other basins with highs are associated with mare fill, implying basalt eruption facilitated by developed faults. Basin topography and mantle uplift on the farside are supported by a rigid lithosphere, whereas basins on the nearside deformed substantially with eruption. Variable styles of compensation on the near- and farsides suggest that reheating and weakening of the lithosphere on the nearside was more extensive than previously considered.

Pathological features of intracranial germinomas with reference to fibrous tissue and granulomatous change.

Intracranial germinomas are accompanied occasionally by a significant granulomatous change and abundant fibrous tissue, and this has made their pathological diagnosis difficult. However, the incidence of the granulomatous reaction and the presence of fibrous tissue together with their clinical characteristics in intracranial germinomas have not been fully investigated. Twenty-four germinomas, none of which had received preoperative treatment, were clinicopathologically examined. The location of the tumor was the pineal region (5 cases), the suprasellar region (13 cases), multiple lesions (2 cases), the basal ganglia region (1 case), and other regions (3 cases). Histologically, the germinomas could be divided into two types: (1) type A (18 cases) consisted mainly of large neoplastic cells and small lymphocytes, showing a two-cell pattern; (2) type B (6 cases) consisted predominantly of fibroinflammatory tissues containing occasional neoplastic cells (5/6 cases) and, rarely, neoplastic cells (1/6 cases). Perioperatively, two-cell-pattern germinomas (type A) were characterized as soft tumors and fibroinflammatory germinomas (type B) as hard tumors. Thus, the fibroinflammatory type B accounted for 25% of the intracranial germinomas. Although there were no topographical and clinical differences between the two types, we conclude that immunohistological studies to detect neoplastic germ cells are warranted in cases of small stereotactic biopsies of hard type B tumors.

GH and PRL-Producing Pituitary Adenoma with Neuron-Like Differentiation.

A pituitary adenoma with neuron-like differentiation in the sella turcica is reported. Sections of the tumor showed a mixture of adenoma cells, ganglionic cells, and neuropil-like structures by light microscopy. Both pituitary adenoma cells and large cells recognized as ganglionic cells by H&E were strongly immunoreactive for both growth hormone (GH) and prolactin (PRL), which indicated that these large cells had properties similar to those of pituitary adenoma cells. Furthermore, electron microscopy (EM) revealed characteristic low electron-dense secretory granules as well as GH-type large electron-dense secretory granules in adenoma cells, neuropils, and swollen bulbs of neuronal endings, which indicated that these three populations may be of the same origin. Furthermore, we could not find typical cell bodies of ganglionic cells by EM. These results are consistent with a hypothesis that attempts to explain the origin of the neuronal components by the neuronal differentiation of adenoma cells. Thus, the best designation of our tumor may be "pituitary adenoma with neuron-like differentiation."