N. Ono

An unusual variant of ependymoma with extensive tumor cell vacuolization

Abstract We report a case of ependymoma with unusual vacuolar features arising in the left occipital lobe of a 2-year-old child. The tumor was composed of cells with single or multiple cytoplasmic vacuoles and clear cells. Some cells showed a signet ring-like configuration. Clear cells were compactly arranged and showed an oligodendroglioma-like appearance. In addition, there were cellular ependymoma-like areas including perivascular pseudorosettes. On immunohistochemistry, glial fibrillary acidic protein and vimentin were mainly detected in cytoplasmic processes, and epithelial membrane antigen (EMA) staining showed granular and small vesicular reactivity. Ultrastructural investigation demonstrated intercellular microrosettes with or without cilia and long zonula adherens-type junctions that are typical of ependymoma. Furthermore, many intracytoplasmic lumina (ICL) were observed. Some ICL had microvilli and some did not. The latter varied in size, and may have fused with each other to develop giant ICL which could correspond to the signet ring-like configuration. Small ICL without microvilli had an appearance similar to that of distended endoplasmic reticula. Serial semithin and ultrathin sections revealed that EMA-positive structures were consistent with ICL containing microvilli and intercellular microrosettes. To determine the presence of unusual vacuolated ependymoma, electron microscopical examination was required. However, light microscopy was useful for detecting EMA-positive microvesicular and granular structures.

Suprasellar germinomas; relationship between tumour size and diabetes insipidus.

SummaryThe clinical and neuro-endocrinological aspects of 17 suprasellar germinoma patients treated between 1972–1991 are reported. Surgical extirpation was not initially attempted, but all patients received irradiation with or without a biopsy. Sixteen of those have led useful lives with appropriate hormonal replacement therapy during a mean follow up of 8 years.Seven tumours at diagnosis were less than 2 cm in diameter (type 1), nine tumours more than 2 cm (type 2), and one double midline tumour was of unknown size. All 7 type 1 patients required 1-deamino-8-D-arginine-vasopressin (DDAVP) to control diabetes insipidus (DI), but only 2 of the 9 type 2 patients have needed DDAVP since completion of the treatment. Patients with smaller tumours required more DDAVP following tumour disappearance, than those with larger tumours. The prognostic indicators for the post-treatment course of DI and retarded growth appeared to be the tumour size and the age at diagnosis.We also emphasize the absence of metastasis in unoperated cases and the use of tumour markers as a diagnostic criterion which obviates surgical acquisition of tissue to make the diagnosis. Possible reasons are discussed and the literature reviewed.

Assessment of malignancy of glioma by positron emission tomography with 18F-fluorodeoxyglucose and single photon emission computed tomography with thallium-201 chloride

Abstract The histological diagnosis and proliferative potential measured by bromodeoxyuridine (BrdU) labelling index (LI) were corelated with preoperative CT and contrast-enhanced, MRI, 18F-flurodeoxyglucose positron emission tomography (PET) and 201T1 single photon emission computed tomography (SPECT) in 43 patients with various grades of glioma. 201T1 SPECT had slightly higher sensitivity to tumours with BrdU LI N 5 % (showing 10/10) than 18F-FDG PET (7/8 tumours). 18F-FDG PET was better for identifying tumours of BrdU LI < 1 % (13/15) than 201T1 SPECT (13/22). Accumulation of 201T1 in the tumour was slightly different from contrast enhancement on CT and/or MRI, and gave “false-postive” results in some low-grade gliomas. However, 201T1 SPECT, which is available in many hospitals and may cost less, provided useful information to supplement that from CT and MRI.

Aromatic nucleophilic substitution reactions of 1-dialkylamino-substituted activated benzenes with various amines in dimethyl sulfoxide

Abstract In the reactions of 1-d1a1kylamino-2,4,6-trinitro- and 1-dialkylam1no-2, 4-dinitrobenzenes with various amines in dimethyl sulfoxide, 1-dialkylamino group is easily replaced with primary n-alkylamines at room temperature, and in a low yield with pyrrolidine only among secondary amines.

Long-Term Clinical Effects of Radiation Therapy for Primitive Gliomas and Medulloblastomas: A Role for Radiosurgery

The effect of conventional irradiation on primitive gliomas and medulloblastomas was examined for 21 patients and a role for radiosurgery was evaluated. Five patients survived more than 8 years after treatment. However, side effects were recognized not only on neuronal and hormonal functions but also on neurovascular structures in the developing brain. The remaining patients died due to tumor recurrence, dissemination and/or metastases. Although these tumors were radioresponsive, conventional irradiation was inadequate in more than half the cases. A role for Gamma Knife radiosurgery should be considered, especially for young children and recurrences. Radiosurgery could also conceivably contribute to the prevention of tumor dissemination. In all events a multidisciplinary treatment should be considered.

Radiosensitive Craniopharyngiomas: The Role of Radiosurgery

Clinical characteristics of radiosensitive craniopharyngiomas and histologically identical tumours were re-evaluated from among 53 patients. There were 9 squamous cell type and 3 mixed type tumours. Early effects of radiosurgery for two recent cases are reported. Radiosurgery may have an important role to play in the treatment of craniopharyngiomas, especially of the squamous cell type.

CONGENITAL GLIOSARCOMA; SO-CALLED SARCOGLIOMA

A mixed glioma and sarcoma in a 3-month-old infant is presented as a rare case of gliosarcoma with a good response to treatment. This congenital case is quite different from those in adults: the tumor cells were mainly composed of sarcomatous elements; glial components were not anaplastic without obvious endothelial hyperplasia, but presented as reticulin-free islands, mimicking a reactive glioma in a sarcoma. It may be termed “sarcoglioma” to distinguish from a classic gliosarcoma. The origin of the rare mixed tumor may be related to a dysgenesis of both mesenchymal and glial elements.

Delayed brain hemorrhage associated with prophylactic whole brain irradiation for pediatric malignant brain tumor: a case report

Intraparenchymal hemorrhage in the left frontal lobe suddenly occurred in a 7-year-old girl who had undergone partial removal of an undifferentiated brain stem glioma and received craniospinal (30 Gy) and posterior fossa booster (20 Gy) irradiation at the age of 20 months. The brain hemorrhage was thought to be delayed irradiation effect. Follow-up neuro-imaging at age 9 years showed two more small occult chronic and subacute hemorrhages in the brain. The possibility of repeated hemorrhage as a delayed reaction to brain irradiation is emphasized.

Adjunctive treatment for recurrent childhood ependymoma of the IV ventricle: chemotherapy with CDDP and MCNU.

The prognosis of recurrent IV-ventricle ependymoma in children is poor. Three cases of recurrent ependymoma were treated with combination chemotherapy usingcis-diamine dichloro platinum (II) (CDDP) and methyl-6-(3-(2-chloroethyl)-3-nitrosoureido)-6-deoxy-α-d-glucopyranoside (MCNU). The patients were 2-, 3-, and 6-year-old boys. The interval between the first operation with irradiation and recurrence was 1 year and 5 months to 2 years. Two cases showed a recurrence at the original site; in the other case, a right sylvian tumor deposit was found via computed tomography. The therapeutic regimen for recurrnt ependymomas was as follows: (1) the tumor was debulked if possible; (2) additional local irradiation of 30 Gy was administered; (3) combined treatment of 100 mg/m2 CDDP and 80 mg/m2 MCNU over 24 h was given 5 times with an interval of 6 weeks between treatments. The patients tolerated the therapy well with only mild side effects. Remission lasted 1 year in two patients and has lasted for 8 months in one.

Long-Term Follow-up Study of Conventional Irradiation for Brain Tumours in Children: A Role for Radiosurgery

21 younger patients (less than 10 years of age) with brain tumours, treated by conventional irradiation, were followed 5 to 20 years (mean 12), using CT scan and/or MR imaging, in order to evaluate adverse effects on the developing brain. Pathological changes such as brain atrophy, lesions in the white matter, calcifications in the brain, and angiopathy were observed in 13 (62%) out of 21 cases. The incidence of abnormalities was related to the age at treatment and the follow-up period. All six cases treated at or under 5 years old and followed more than 10 years showed pathological changes in the brain. In order to minimize the radiation damage, 5 patients with brain tumours less than 5 years old were treated by gamma knife surgery. The early results encourage further trials. Radiosurgery may play a role as an alternative treatment or as a component of future multidisciplinary treatment for brain tumours is children.