Large intracranial vessel occlusive vasculopathy after radiation therapy in children: clinical features and usefulness of magnetic resonance imaging.

PURPOSE: To assess the relationship between large intracranial vessel occlusive vasculopathy (vasculopathy) and radiation therapy, and to clarify the clinical efficacy of magnetic resonance (MR) imaging in the diagnosis and screening of the vasculopathy. METHODS AND MATERIALS: We retrospectively evaluated the medical records and serial MR images for 32 pediatric patients, in whom radiation therapy had been given to fields including the circle of Willis and major cerebral arteries. All children had periodically undergone follow-up neurologic assessment and MR imaging examinations at Kanagawa Children's Medical Center for more than one year after radiation therapy (range 1.3-14 years). Patients who had not remained free of tumor progression up to the time of final evaluation were excluded. RESULTS: Vasculopathy developed in 6 of 32 patients 2-13 years after radiation therapy. Three of them presented with transient ischemic attacks (TIA) and the other three showed infarctions without preceding TIA. Steno-occlusive changes of major cerebral arteries were identified by MR imaging in all six patients, but not obtained in the remaining 26 patients. In the patients with TIA, MR imaging demonstrated steno-occlusive changes at the time of TIA, before irreversible infarction. They have been doing well subsequent to encephaloduroarteriosynangiosis. In the three patients who presented infarction without preceding TIA, MR imaging did not demonstrate the vascular change before the onset of infarction, and two of them developed neurologic deficits. The mean exposure dose for the circle of Willis and major cerebral arteries in these six patients was significantly higher than that in the remaining 26 patients without this sequela (61 Gy vs. 50 Gy, p < 0.05). The mean age at radiation therapy of the six patients was lower, but the difference was not significant. CONCLUSION: The incidence of vasculopathy after radiation therapy has a considerable correlation with radiation dose and age at radiation therapy. MR examination is useful for the diagnostic evaluation of vasculopathy, and it is also effective in screening for vasculopathy in patients with TIA, and may be helpful in the prevention of neurologic sequela.

Hypothalamic hamartoma successfully treated by operation. Case report.

An 18-month-old boy was diagnosed as having a hypothalamic hamartoma. When he was 1 year old, he developed precocious puberty, and at 18 months old, endocrinological tests revealed abnormally high follicle-stimulating hormone, luteinizing hormone, and testosterone levels. The center of the hamartoma was subtotally excised, as confirmed on the postoperative computerized tomography scan. Precocious puberty subsided after the operation.

A cloverleaf skull syndrome probably of Beare-Stevenson type associated with Chiari malformation.

A case of cloverleaf skull (CLS) syndrome with Chiari malformation was reported. The patient developed congenital hydrocephalus, upper airway obstruction and breath holding spells. Ventriculo-peritoneal shunt improved the hydrocephalus, but the patient progressively developed apneic episodes in spite of intubation. Brain magnetic resonance imaging (MRI) disclosed severe Chiari malformation. Laminectomy of the cervical vertebrae and craniectomy at the occipital bone were performed to relieve the brain stem compression. Postoperatively, the respiratory symptoms improved slightly, but 8 weeks later the patient suddenly died. Literature survey revealed that this is the second case report of CLS associated with Chiari malformation. It was assumed that the respiratory problems in our patient are ascribable to the Chiari malformation as well as the upper airway obstruction. The patient here reported seems to be a case of Beare-Stevenson cutis gyrata syndrome, in view of an association of multiple characteristic dysmorphic features, in addition to CLS.

Ossifying fibromyxoid tumor of soft parts in a child: a case report.

Ossifying fibromyxoid tumor (OFMT) is a relatively rare tumor, most of which occur in adults with preferential localization in subcutis or muscles of the extremeties. Although Schwannian or cartilage origin has been suggested, histogenesis of this tumor still is unclear. The authors present a 6-year-old girl with retroperitoneal OFMT showing paraspinal extension, who is alive and tumor free 9 years after excision. The current case is the youngest reported patient showing unusual deep trunk site with surgically identified association with the spinal nerve. Both the clinical and histopathologic features strongly suggested Schwannian origin of this tumor.

Cytologic features of desmoplastic infantile ganglioglioma: a report of two cases.

BACKGROUND: Desmoplastic infantile ganglioglioma (DIG) is a rare intracranial tumor of infancy, characterized by solid and cystic components, voluminous size and supratentorial location. Although its histologic features have been reported, there has been no cytologic description of the tumor. Cytologic findings on imprint and aspirated material from two cases of histologically verified DIG are discussed. CASES: A 12-month-old male with cutis marmorata telangiectatica congenita and a 44-month-old female with episodes of spasm were referred to our center. Radiologic examination revealed a large, cystic, supratentorial mass in both patients. The mass was surgically removed, and histology revealed prominent desmoplasia with a mixture of astroglial and neuronal cells. Cytologic findings on imprint material and the needle aspirates taken from the cystic area of the tumor revealed a few isolated or sheetlike arrangements of small cells positive for glial fibrillary acidic protein and a few large cells with abundant cytoplasm and prominent nucleoli, positive for neuronal marker. CONCLUSION: Although distinction between ganglioglioma and DIG may be difficult by cytology alone, with the characteristic clinical presentation and radiologic findings, the possibility of DIG should be kept in mind when the specimens contain both astroglial and neuronal elements.

Dandy-Walker syndrome associated with occipital meningocele and spinal lipoma--case report.

A neonate presented with Dandy-Walker syndrome associated with occipital meningocele and spinal lipoma, manifesting as soft masses on the skull and lumbosacral regions. Magnetic resonance imaging demonstrated a large posterior fossa cyst between the fourth ventricle and occipital meningocele, but the aqueduct was patent and there was no sign of hydrocephalus. A cyst-peritoneal shunt was emplaced at the age of 8 days followed by partial removal of the spinal lipoma and untethering of the cord at the 3 months. Follow-up examination of age 3 years found almost normal development, although the cyst still persisted.

[Two cases of diastematomyelia: its evaluation with MR imaging].

We experienced two cases of diastematomyelia. Magnetic Resonance Imaging (MRI) was very useful in definitive diagnosis and detection of associated abnormalities. Case 1 was a 5-year-old boy. He was admitted because of foot-length discrepancy. He also presented scoliosis, hypertrichosis and pigmentation in his back skin, and foot deformity. Myelography and CT myelography revealed bony septum and split cord at midthoracic level, and two separated taut filum terminalis in the lumbosacral region. Sagittal MR image delineated the taut filum terminale adhering to the lipomatous tissue at the end of dural sac. Removal of the septum and division of the taut filum terminalis were performed. Case 2 was a newborn baby. She was admitted because of bulging of the back skin in the lumbosacral region and foot deformity. Plain CT revealed bony septum at lumbar level. Myelography was not performed. MR image demonstrated the split cord in its entirety, the bony septum and its structures in detail, and the associated lipoma. Removal of the septum and resection of the lipoma were performed. Diastematomyelia has been able to be definitely diagnosed only by myelography or CT myelography. In our case, MR image gave visualization of septum and split cord in its entirety, and also detected the associated anomalies such as lipoma, tight filum terminale. MRI has possibility of becoming a diagnostic technique in place of myelography and CT myelography.

[Controlling precocious puberty--surgical excision of hypothalamic hamartoma causing precocious puberty].

Among the causes of precocious puberty, hypothalamic hamartoma comprises a small percentage. However, the frequency of precocious puberty in the presence of hypothalamic hamartoma is quite high. Recently, results of surgery in 14 cases of hypothalamic hamartoma were reported. Precocious puberty completely subsided in three cases and slight improvement was achieved in another three cases. We performed surgery in four patients with hypothalamic hamartomas, with the goal of decreasing the symptoms of precocious puberty. The patients were two females (aged 1 yr, 3 mo and 6 mo) and two males (aged 3 yr, 7 mo and 1 yr, 9 mo). The main symptoms were precocious puberty and mental retardation of varying degrees. The males had excessive growth of body and external genitalia, while the females had genital bleeding and premature breast development. In each case, computed tomographic scans disclosed a round, isodense mass in the interpeduncular cistern, attached to the base of the hypothalamus. Contrast enhancement was negative. Endocrinologically, in case 1, testosterone was 92.6 ng/ml, FSH was 16 mIU/ml, and LH was 2.2 mIU/ml. Although LH was within normal limits, it overresponded to LH-RH stimulation. In case 2, estrogen was 13.5 ng/day, LH was 5.2 mIU/ml, FSH was 5.3 mIU/ml, and LH showed an exaggerated response to LH-RH stimulation. In case 3, testosterone was 362 ng/ml, LH was 8.8 mIU/ml, FSH was 4.8 mIU/ml, and LH showed an abnormally high response to LH-RH stimulation. In case 4, LH was 18.4 mIU/ml, FSH was 12.0 mIU/ml, and both hormones were stimulated abnormally strongly by LH-RH.(ABSTRACT TRUNCATED AT 250 WORDS)

[Management results of primary intracranial malignant lymphoma].

Nineteen cases of primary malignant lymphoma of the central nervous system were reported. The clinical characteristics, diagnostic procedure and therapeutic method, especially concerning chemotherapy, were analysed. The following conclusions were obtained: 1) histological classifications and surgical procedures were not factors involved in prognosis. 2) radiotherapy was regarded as an effective but a palliative treatment. 3) combined chemotherapy for systemic malignant lymphoma, such as VEMP, VEP, COPP, seemed to be of little use for intracranial malignant lymphoma. 4) intra-arterial administration of high-dose methotrexate, ACNU and intravenous administration of high-dose cytosine arabinoside can be expected to be a useful chemotherapeutic approach.

[A case of idiopathic spinal epidural hematoma with a fructuation of the symptoms].

We reported a 9-year-old girl with idiopathic spinal epidural hematoma. She complained of her back and neck pain only during night for a few days, followed by days with no symptoms (a few days remission of her symptoms). This episode repeated, and she was suspected to have a psychogenic reaction. However, 28 days later after the first symptoms appeared, paralysis of lower extremities, anesthesia and urinary bladder dysfunction (neurogenic bladder) appeared. Spinal MRI showed a spinal epidural hematoma at the C 5-Th 2 level. An emergency operation of laminectomy and evacuation of the hematoma was done, and she has recovered gradually. We diagnosed her as having idiopathic spinal epidural hematoma. Excerbations of symptoms during nights were thought to be due to an elevation of venous pressure by lying, and transient remissions were due to intermittent small hemorrhages.

Surgical management of cervicomedullary compression in achondroplasia.

Achondroplasia is often associated with cervicomedullary dysfunction. MRI sagittal imaging was performed to determine whether surgical intervention was necessary to relieve cervicomedullary compression. Cervicomedullary compression was classified into severe and mild types based on the ratios of the brain stem diameter at the foramen magnum, the site of the most severe stenosis to the diameter at the pontomedullary junction and C3 level, where it is normal. This classification was found to be closely correlated with the severity of clinical symptoms and important in determining whether surgical intervention is necessary. Moreover, since the incidence of sudden death is high in patients aged 4 years or younger with achondroplasia, surgical intervention should be considered in all such patients if cervicomedullary compression is present.

Radiation-associated xanthomatous meningioma in a child.

Radiation-associated meningioma in a child is rare. We report a case of xanthomatous meningioma, a rare subtype even in the adult population, which occurred in a 10-year-old boy who had undergone irradiation therapy for ependymoma at the age of 2 years. Histological examination of the tumor revealed a mixture of meningothelial cells and abundant foamy xanthomatous cells, with no apparent atypical cellular features. Xanthomatous meningioma associated with therapeutic high-dose irradiation in a child has not yet been documented. Immunohistochemical studies and the clinical course are evaluated with a review of the literature.

Rapidly growing calcified cerebellar astrocytoma in infants.

We report the case of an infant with a cerebellar astrocytoma that showed marked calcification within only 6 months. In general, only slow-growing tumors tend to calcify. To our knowledge, no other case of such rapid calcification in cerebellar astrocytoma has been reported.

Nasal chondromesenchymal hamartoma of infancy: the first Japanese case report.

The first Japanese case of nasal chondromesenchymal hamartoma, a rare infantile nasal neoplasm, is presented. A 4-month-old Japanese boy was referred to our Centre because of intranasal mass and oculomotor disturbance. Radiological images showed the intranasal tumor extending to the paranasal sinus, orbit, and anterior frontal fossa. Subtotal resection and radiation therapy to residual tumor were performed. There has been no recurrence of the tumor for 13 years. Histologically, the lesion demonstrated admixture of various mesenchymal elements, including cellular spindle cell stroma with occasional myxoid change, nodules of mature/immature cartilaginous tissue, focal osteoclast-like giant cells, and erythrocyte-filled spaces resembling aneurysmal bone cyst. The histology was consistent with the findings presented by McDermott et al. 1986. Immunohistochemically, the spindle cells were positive for vimentin and smooth muscle actin. Chondrocytes in the mature cartilaginous tissue were positive for S-100 and vimentin; chondrocytes in the immature cartilaginous tissue were positive for S-100, vimentin, and smooth muscle actin. Ultrastructurally, the spindle cells showed features of either fibroblast or myofibroblast.

Choroid plexus papilloma of the III ventricle in an infant.

A case of choroid plexus papilloma of the III ventricle associated with hydrocephalus in an 1-month-old boy is reported. The tumor was removed after placement of a ventriculoperitoneal shunt; however, the histologically mainly benign tumor grew very rapidly. The cause of this rapid growth may have been a drop in the intraventricular pressure after the shunting procedure.

Spinal cartilaginous meningioma in a child: a case report.

Cartilage-containing meningiomas are extremely uncommon, and such tumors located in the spinal cord of a child have not previously been reported. We describe a case of cartilaginous spinal meningioma in a 13-year-old girl. The tumor lacked typical histopathological features of meningioma on light microscopic examination, and the final diagnosis was possible only after an extensive study by electron microscopic examination. The case is evaluated with a focus on differential diagnosis and review of the literature.