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Intracranial angiolipoma: report of two cases.

Intracranial tumours composed of adipose tissue and abnormal blood vessels are rarely recognized. We report two such cases; one showed the radiological and histological appearances of a combined lipoma and an arteriovenous malformation, the other the appearances of a lipoma with a prominent capillary component. Computed tomography did not give any indication of their vascular nature, but surgery was complicated by intra- and postoperative haemorrhage.

Adult↗

Primary intracranial myxopapillary ependymomas: report of two cases and review of the literature.

Myxopapillary ependymoma is a variant of ependymoma occurring almost exclusively in the conus medullaris or filum terminale. Myxopapillary ependymoma found primarily in the brain is extremely rare. Two such cases appearing at the 4th ventricle and cerebral falx are reported. The imaging features of such tumors are a primary cystic mass with strong enhancement at its solid part. Myxopapillary ependymoma should be a possible differential diagnosis when an intracranial cystic tumor is found.

Adult↗

Insulin-like growth factor I receptor signaling system in JC virus T antigen-induced primitive neuroectodermal tumors--medulloblastomas.

Medulloblastomas represent about 25% of all pediatric intracranial neoplasms. These highly malignant tumors arise from the cerebellum, affecting mainly children between ages 5 and 15. Although the etiology of medulloblastomas has not yet been elucidated, several reports suggest that both the cellular protein insulin-like growth factor I (IGF-I) and the early protein of the human polyomavirus JC (JCV T antigen) may contribute to the development of these tumors. The results of this study show a potential functional cooperation between these two proteins in the process of malignant transformation. Both medulloblastoma cell lines and medulloblastoma biopsies are characterized by the abundant presence of the IGF-I receptor (IGF-IR) and its major signaling molecule, insulin receptor substrate 1 (IRS-1). Importantly, IRS-1 is translocated to the nucleus in the presence of the JCV T antigen. Nuclear IRS-1 was detected in T antigen-positive cell lines and in T antigen-positive biopsies from patients diagnosed with medulloblastoma. The IRS-1 domain responsible for a direct JCV T antigen binding was localized within the N-terminal portion of IRS-1 molecule and the competition for IRS-1 T antigen binding by a dominant-negative mutant of IRS-1 inhibited growth and survival of JCV T antigen-transformed cells in anchorage-independent culture condition.

Brain Neoplasms↗

Intracranial tumours in the elderly: diagnosis and treatment.

A series of 111 intracranial tumours in elderly patients, investigated by modern methods, is reviewed. The clinical picture of progressive neurological deficit, and/or intellectual impairment, did not differ greatly from that of younger patients, but the manifestations of raised intracranial pressure (headache and papilloedema) were much less common in the elderly. Intermittency of symptoms appeared to be a relatively common phenomenon with meningiomas, occurring in four of 12 symptomatic cases. Operative treatment, for removal of meningiomas or relief of obstructive hydrocephalus, produced improvement in almost all cases, with a low morbidity and mortality. High-dose steroid therapy produced substantial, if temporary, benefit in 51% of hemisphere tumours.

Aged↗

Loss of heterozygosity at 6q is frequent and concurrent with 3p loss in sporadic and familial capillary hemangioblastomas.

Capillary hemangioblastoma is a benign tumor, occurring sporadically or as a manifestation of von Hippel-Lindau (VHL) disease. Inactivation of the VHL gene at 3p25-26 has been demonstrated in all VHL-associated hemangioblastomas. However, the VHL gene has been found to be inactivated in only 20% to 50% of sporadic tumors. So far, no other gene has been reported to be involved in the development of hemangioblastomas. DNA losses at 6q are frequent alterations in hemangioblastomas, as shown by comparative genomic hybridization. We therefore analyzed 15 hemangioblastomas for loss of heterozygosity (LOH) on chromosome 3p and 6q to reveal the frequency of allelic losses and to determine minimal deleted areas. We detected LOH at 6q for one or more markers in 11 (73%) out of 15 cases (in 9 of 11 sporadic and in 2 of 4 VHL-associated tumors). The analyses revealed a minimal 3-megabase (Mb) deleted region at 6q23-24, where 9 of 11 (82%) informative cases showed LOH. LOH at 3p was seen in 14 out of 15 tumors. LOH occurred concurrently at 6q and 3p in 67% of cases. Our data strongly suggests that a tumor suppressor gene located at 6q23-24 is involved in tumorigenesis of hemangioblastomas, in addition to the VHL gene.

Adult↗

Sudden, unexpected deaths due to primary intracranial neoplasms.

In 10,995 consecutive medicolegal autopsies, there were 19 deaths due to an unsuspected primary intracranial neoplasm. Nine (47.4%) of the tumors were in the astrocytoma-glioblastoma category. The remainder included four cases of oligodendroglioma and one case each of medulloblastoma, microglioma, meningioma, teratoma, colloid cyst and pituitary chromophobe adenoma. In six cases, death occurred following abrupt loss of consciousness, or else the patient was found dead. In five of these six cases, there were no known preceding symptoms. The remaining 13 patients exhibited the characteristic symptoms produced by intracranial neoplasms, including symptoms of increased intracranial pressure, epilepsy, and psychiatric manifestations. Only one patient presented with a focal neurologic deficit which resolved in 24 hours. A comparison of the duration and type of symptomatology exhibited by these patients with a hospital patient population in which death was caused by a previously diagnosed primary intracranial neoplasm and an autopsy was performed underscored 1) the shorter duration of acute symptomatology, 2) the nonlocalizing nature of the symptoms manifested, 3) the lack of progression or change in symptoms in those patients in whom epilepsy was the primary manifestation of their underlying disease, and 4) the lower incidence of focal neurologic deficits as the presenting symptoms in our series.

Adolescent↗

Expression of vascular endothelial growth factor and its receptors in pilocytic astrocytoma.

Vascular endothelial growth factor (VEGF), a potent angiogenic and vascular permeability factor, is important in the angiogenesis of glioblastoma. A major difference between pilocytic astrocytoma, a grade I tumor, and the grade II fibrillary astrocytoma is the vascular proliferation, highly vascularized stroma, and great propensity for cyst formation in the former. In order to explore factors regulating such angiogenesis and cyst formation in pilocytic astrocytoma, we examined expression of VEGF and its receptors (KDR and Flt-1) using in situ hybridization. In all 14 cases a high level of VEGF transcripts could be demonstrated. These were found in specific regions, namely, in the tumor cyst wall, in areas of hyaline cystic degeneration, in stellate reticulated astrocytes around microcysts in the biphasic compact and loose areas, and in tumor cells with degenerative pleomorphic multicoated nuclei. KDR and Flt-1 were expressed in the tumor vasculature, with particularly high levels seen in coiled young proliferating vessels, especially those in the cyst wall. Given the known angiogenic and vascular permeability activities of VEGF, we propose that VEGF plays an important role in molding the characteristic morphologic features of this tumor, namely, the formation of cysts, microcystic pattern, hyaline cystic degeneration, hyaline vessels, and vascular proliferation. Mechanisms that block the VEGF pathway could constitute a potential therapeutic strategy for the treatment of this tumor.

Adolescent↗

Primary sarcomas of the brain and spinal cord: a study of 18 cases.

Primary sarcomas of the central nervous system are exceedingly rare. We reviewed the clinicopathologic features of 18 primary central nervous system sarcomas diagnosed from 1959 through 1999. Median age at diagnosis of the nine female and nine male patients was 28 years (range 3-63 years). Median tumor size was 4 cm (range 1.3-8 cm). Fifteen tumors arose in the cerebrum (83%), two in the cerebellum, and one in the spinal cord. Histopathologically, the most common tumor types included fibrosarcoma (six), malignant fibrous histiocytoma (five), and undifferentiated sarcoma (three). Immunohistochemical and ultrastructural studies supported the histologic diagnosis in 17 and six cases, respectively. All patients had subtotal to gross total tumor resection; 16 also received radiotherapy and/or chemotherapy. Twelve tumors (67%) were high-grade. Follow-up was obtained in all instances (median 2.3 years). Nine patients died of the disease, eight with high-grade tumors. Survival at 5 years for patients with high-grade tumors was 28% compared with 83% for those with low-grade neoplasms (p = 0.03). Primary central nervous system sarcomas most often affect young and middle-aged adults. Most involve the cerebrum and show fibrous, "fibrohistiocytic," or no specific differentiation. The prognosis for high-grade sarcomas seems better than that for glioblastoma multiforme.

Adolescent↗

Solitary fibrous tumor of the cerebellopontine angle with salivary gland heterotopia: a unique presentation.

We report the unique association of a solitary fibrous tumor of the cerebellopontine angle with ectopic salivary gland tissue in a 53-year-old woman. The patient, diagnosed 21 years earlier with a right cerebellopontine angle fibrous meningioma, presented with a recurrent mass, which, upon surgical removal, showed features of a solitary fibrous tumor. Strong and diffuse immunoreactivity to CD34 and BCL-2 and a negative epithelial membrane antigen immunostain confirmed the diagnosis. Admixed with the spindle cell neoplasm, a bland glandular element composed of small glands and variably dilated tubules was noted. The vague tubuloacinar arrangement of the glands and the presence of acinar cells with a granular, periodic acid-Schiff-positive, basophilic cytoplasm, resembling serous type acini, were features of benign, although ectopic, salivary gland tissue. The presence of a myoepithelial cell layer, surrounding some of the acini and highlighted by the smooth muscle actin immunostain, strengthened this interpretation.

Biomarkers, Tumor↗

Dense dermoids in craniocervical region.

Computed tomography of dermoids and epidermoids usually demonstrates areas of variable hypodensity, located most frequently in the sellar region and in the cerebellopontine angle. This report presents two cases of hyperdense dermoids of the craniocervical region which are compared with six other cases described in the literature.

Adolescent↗