Squamous cell carcinoma arising in an intracranial dermoid cyst--case report.

A 47-year-old male presented with headache. Magnetic resonance imaging revealed an enhanced mass lesion in the cerebellar vermis and left cerebellar hemisphere and in the cisterna magna. Gross total removal of the tumor was performed. Histological examination demonstrated squamous cell carcinoma in the dermoid cyst. The patient subsequently received localized radiation therapy of total 55 Gy. He has been without tumor recurrence for 6 years since the surgery. We recommend local radiation therapy over 50 Gy following surgery.

Correlation of the expression of nuclear factor-kappa B, tumor necrosis factor receptor type 1 (TNFR 1) and c-Myc with the clinical course in the treatment of malignant astrocytomas with recombinant mutant human tumor necrosis factor-alpha (TNF-SAM2).

We present our experience with a combination chemotherapy regimen consisting of ranimustine (MCNU) and recombinant human mutant tumor necrosis factor-alpha (TNF-SAM2) for malignant astrocytomas. We also investigated the expression of nuclear factor-kappa B (NF-kappa B), tumor necrosis factor receptor type 1 (TNFR1), and c-Myc in human astrocytoma tissues in vivo in patients treated with TNF-SAM2 by RT-PCR and immunohistochemical analysis to examine whether there is any correlation between the prognosis of these patients after TNF-SAM2 treatment and the expression of these factors. The initial regimens were prescribed as adjuvant therapy in conjunction with radiotherapy following standard surgical treatment. Newly diagnosed patients were treated with up to four cycles of this regimen (TNF-SAM2, MCNU, and radiotherapy: TMR group). Four patients with anaplastic astrocytomas and 13 patients with glioblastomas (11 men and 6 women) aged 24 to 68 years (median 55.7 years) were eligible and evaluated for response and toxicity. The estimated median survival time was 354 weeks with anaplastic astrocytomas, and 79 +/- 10.8 weeks with glioblastomas. One- and 2-year survival rates were 100% and 100% with anaplastic astrocytomas, and 69.2% and 30.8% with glioblastomas. Grade 3 and 4 hematological toxicities were not experienced. None of the patients experienced a treatment delay due to toxicity. All other acute toxicities were anticipated and manageable. Two of the 4 patients with anaplastic astrocytomas were positive for the expression of NF-kappa B, TNFRl and c-Myc. The expression of NF-kappa B, TNFR1 and c-Myc was investigated in 10 of the 13 patients with glioblastoma, and c-Myc, TNFRl and NF-kappa B were detected in 9, 7, and 8 of these 10 patients' surgical specimens, respectively. Despite the small number of patients, these clinical results suggest that combined chemotherapy with mutant TNF-alpha (TNF-SAM2) was safe and well tolerated, and may confer a survival benefit for patients with malignant astrocytomas in comparison to our historical controls. Its effectiveness as an adjuvant therapy deserves a properly stratified randomized trial. Although there was no significant correlation between the efficacy of TNF-SAM2 treatment and the expression of NF-kappa B, our results suggest that the constitutive activation of NF-kappa B subunits in malignant astrocytomas, especially in glioblastoma, could be associated with resistance to TNF-alpha immunotherapy. These results could offer new insight to help establish a new chemotherapeutic strategy for malignant astrocytomas.

Calcifying pseudotumor of the neural axis--case report.

A 22-year-old female presented with a calcifying pseudotumor of the neural axis manifesting as generalized convulsive seizure twice within 1 year. Computed tomography revealed a small, calcified mass lesion located in the right parietal lobe adjacent to the skull. The tumor was composed of an extensively calcified mass with accompanying peripheral epithelioid cells and focal mature bone structure, consistent with the diagnosis of a calcifying pseudotumor of the neural axis. Following complete excision of the tumor, the patient has been free from seizures for 8 years.

Hemangioblastoma mimicking tentorial meningioma: preoperative embolization of the meningeal arterial blood supply--case report.

A 72-year-old male presented with a primary hemangioblastoma of the posterior fossa with unusual dural attachment and meningeal arterial blood supply from the external carotid artery and marginal tentorial artery. Preoperative embolization facilitated complete resection of the tumor with no resultant neurological deficit. Hemangioblastoma must be included in the differential diagnosis of tumors with dural involvement. Preoperative embolization is very useful in such tumors.

A case report of caudal regression syndrome associated with an intraspinal arachnoid cyst.

We report here a rare case of caudal regression syndrome associated with an intraspinal arachnoid cyst. The patient was a 6-month-old baby girl with multicomplex congenital abnormalities: sacrococcygeal dysgenesis and ventral curvature, large terminal cyst (myelocystocele), spinal arachnoid cyst, cerebellar hypertrophy (suspected), high imperforate anus, partial dysgenesis of the large intestine, omphalocele, atresia of the vagina, bilateral incomplete ureter duplication, incomplete pseudoduplicated bladder and bilateral talipes equinovarus. We performed plastic repair of the myelocystocele and perineal lesion for caudal regression syndrome and partial removal of the cyst wall for the intraspinal arachnoid cyst. She has been well for 3 years postoperatively, and her mental development is normal.

Treatment of malignant astrocytomas with recombinant mutant human tumor necrosis factor-alpha (TNF-SAM2).

We present our experience with a combination chemotherapy regimen consisting of ranimustine (MCNU) and recombinant human mutant tumor necrosis factor-alpha (TNF-SAM2) for malignant astrocytomas. The initial regimens were prescribed as adjuvant therapy in conjunction with radiotherapy following standard surgical treatment. Newly diagnosed patients were treated with up to four cycles of this regimen (TNF-SAM2, MCNU, and radiotherapy: TMR group). Seventeen patients (11 men and 6 women) aged 24 to 68 years (median 54.6 years) were eligible and evaluated for response and toxicity. The estimated median survival time was 354 weeks with anaplastic astrocytomas, and 76 weeks with glioblastomas. One- and 2-year survival rates were 100% and 100% with anaplastic astrocytomas, and 69.2% and 29.7% with glioblastomas. Grade 3 and 4 hematological toxicities were not experienced. None of the patients experienced a treatment delay due to toxicity. All other acute toxicities were anticipated and manageable. Twenty three patients (11 men and 12 women) aged 22 to 66 years (median 50.7 years) were evaluated as a historical control of patients who received chemotherapy with MCNU alone in conjunction with radiotherapy following standard surgical treatment (MCNU and radiotherapy: MR group). The estimated median survival time was 205 weeks with anaplastic astrocytomas, and 62 weeks with glioblastomas. One- and 2-year survival rates were 88.9% and 66.7% with anaplastic astrocytomas, and 71.4% and 7.1% with glioblastomas in this group. There were no significant differences in survival rates between patients in the TMR and MR groups with either anaplastic astrocytoma or glioblastoma. However, despite the small number of patients, those with anaplastic astrocytoma in the TMR group tended to survive longer than those in the MR group. These results suggest that combined chemotherapy with mutant TNF-alpha may benefit those with anaplastic astrocytoma, and thus warrants further evaluation. On the other hand, the lack of activity does not warrant any further study of this schedule of TNF-SAM2 for the treatment of glioblastoma.

Expression and cellular localization of low-density lipoprotein receptor-related protein/alpha 2-macroglobulin receptor in human glioblastoma in vivo.

Low-density lipoprotein receptor-related protein/alpha 2-macroglobulin receptor (LRP) has been proposed to mediate the cellular uptake and clearance of inactivated protease-inhibitor complexes in regulating proteinase activity at the cell surface, which is necessary for cellular migration and invasive processes. In this study, we investigated the presence of both LRP and urokinase-type plasminogen activator receptor (uPAR) in glioblastoma by reverse transcriptase-polymerase chain reaction (RT-PCR), and the cellular localization of LRP in glioblastoma tissues by immunohistochemical analysis. LRP mRNA was frequently expressed in glioblastomas and anaplastic astrocytomas compared with low-grade astrocytomas by RT-PCR analysis, and was well correlated with uPAR expression. The immunohistochemistry of LRP on sequential frozen sections showed that neoplastic glial cells and endothelial cells of glioblastomas exhibited intense LRP immunoreactivity, whereas LRP was almost undetectable in low-grade astrocytomas or in normal glial cells and endothelial cells of normal brain tissue. Glioblastomas from 11 patients in which the expression of LRP mRNA was observed by PCR displayed strong to moderate LRP immunoreactivity, with predominantly diffuse cytoplasmic and cell-surface localization. In normal brain tissues, LRP immunoreactivity was identified in the pyramidal neurons of the cerebral cortex. These results indicate that LRP is present both in the cellular cytoplasm and on the cell surface of glioblastomas with an increased expression of uPAR. Altered LRP expression might contribute to the stimulation of cell-surface proteolytic activity that in turn facilitates the invasiveness of glioblastoma in vivo.

Hypothalamic hamartoma associated with multiple congenital abnormalities. Two patients and a review of reported cases.

We report 2 patients with hypothalamic hamartoma associated with multiple congenital abnormalities and analyze 42 (including our own) reported cases, including our 2 cases, of hypothalamic hamartoma or hypothalamic hamartoblastoma with multiple congenital abnormalities, to understand the timing of their occurrence and clarify the prognosis. To this end, we classified them into lethal and nonlethal cases. We found poly- and syndactyly, cleft or high-arched palate and nose abnormalities to be important manifestations of this syndrome. Major organ abnormalities and CNS and endocrine abnormalities occurred frequently among the lethal cases, very likely indicative of a disturbance of embryogenesis between gestational days 34-37 and thus implicated in a negative prognosis.

Incidence of hypocomplementemia in elementary and junior high school children with urinary abnormalities.

Abnormalities were detected in 2669 of 326,257 elementary and junior high school children (169,856 males and 156,401 females) who were screened at school for urinary abnormalities. Serum complement (C3) level was measured in all 2669 children having urinary abnormalities (811 males, 1856 females). Three had a serum C3 level that was more than three standard deviations below the mean value. Type I membranoproliferative glomerulonephritis (MPGN) was diagnosed on histological examination in one of these three children, while the other two did not undergo renal biopsy because they had serum C3 levels of 40 and 44 mg/dL, respectively, and because their urinary abnormalities were transient. It was considered that there is not much significance in testing the serum complement in the urine screening done at school and the cost/benefit ratio is low. The results appeared to reflect the frequency of persistent hypocomplementemic MPGN in Japan in recent years.

Increased expression of low density lipoprotein receptor-related protein/alpha2-macroglobulin receptor in human malignant astrocytomas.

Low-density lipoprotein receptor-related protein (LRP) plays an important role in regulating proteinase activity, which is necessary for cellular invasive processes. In this study, we investigated the presence of both LRP and urokinase-type plasminogen activator receptor (uPAR) in astrocytoma tissues and in glioma cell lines by PCR and immunohistochemical analysis. LRP mRNA was expressed frequently in glioblastomas, as compared with low-grade astrocytomas by PCR analysis and was well correlated with uPAR expression. These results were consistent with the immunohistochemical localization of LRP in glioblastomas. Immunohistochemistry of LRP on sequential frozen sections showed that neoplastic glial cells and endothelial cells of glioblastomas exhibited intense LRP immunoreactivity, whereas LRP was almost undetectable in low-grade astrocytomas and in normal glial cells and endothelial cells of normal brain tissues. In normal brain tissues, LRP immunoreactivity was identified in the pyramidal neurons of the cerebral cortex. In metastatic brain tumors (metastatic lung adenocarcinomas) and primary lung adenocarcinomas, LRP expression was low to undetectable, suggesting that LRP expression is regulated differently in these tumors than in malignant astrocytomas. These results indicate that LRP is overexpressed in malignant astrocytomas, especially in glioblastomas, and the increased expression of LRP appears to correlate with the expression of uPAR and the malignancy of astrocytomas. Our results suggest strongly that LRP may play a role in facilitating glioblastoma invasiveness and neovascularization within tumor tissues by regulating cell surface proteolytic activity.

Cerebellar gliomas with exophytic growth--three case reports.

Three patients presented with cerebellar hemispheric astrocytic tumors which showed an exophytic growth pattern. The neuroimaging appearances of these cases mimicked a cerebellopontine angle tumor in two cases, and a posterior fossa extra-axial tumor in the other, which arose from the left cerebellar hemisphere with exophytic extension into the left crural and quadrigeminal cisterns and compressed the midbrain directly. All patients underwent surgical resection, and two patients also received adjuvant radiation therapy and chemotherapy. Intraoperative findings confirmed that the tumors had intramedullary origins from the cerebellar hemisphere, and extended exophytically into the subarachnoid space forming an extra-axial mass lesion. The histological diagnoses were mixed malignant oligo-astrocytoma (grade III), astrocytoma (grade II), and glioblastoma (grade IV). Cerebellar gliomas with exophytic growth to the cerebellopontine angle cistern should be considered in the differential diagnosis of cerebellopontine angle tumors.

Symptomatic Chiari malformation and associated pathophysiology in pediatric and adult patients without myelodysplasia.

The clinical characteristics of eight pediatric and five adult patients with Chiari malformation were evaluated. Six pediatric and five adult patients had associated syringomyelia. All patients initially underwent a suboccipital craniectomy with upper cervical (C-1 and/or C-2) laminectomy and duraplasty, and/or shunting procedures. The clinical characteristics of the pediatric and adult groups were compared. The mean interval between onset of symptoms and operation was shorter in the pediatric group (3 yrs 6 mos) than in the adult group (7 yrs 1 mo). Pediatric patients without syringomyelia had the shortest mean interval of 1 year 8 months. Preoperatively, the clinical features were more severe in the adult patients than in the pediatric patients. Postoperatively, seven of eight pediatric patients improved and one stabilized, while two of five adult patients improved, one stabilized, and in two the disease continued to progress despite multiple corrective procedures. Cine magnetic resonance imaging revealed correction of the abnormal cerebrospinal fluid (CSF) flow at the craniovertebral junction and decreased to-and-fro movement in the syrinx after posterior fossa decompression, which were closely correlated with the improvement of clinical features in pediatric patients. However, adult patients required further procedures because of the multifactorial nature of the disease. Evaluation of abnormal CSF pathways at the craniovertebral junction is important for investigating the pathogenesis of Chiari malformation and associated syringomyelia.

Maffucci's syndrome associated with intracranial chordoma: case report.

This report discusses Maffucci's syndrome associated with intracranial chordoma. Of the 170 reported cases of this syndrome, only 27 have been associated with intracranial lesions and none was involved with intracranial chordoma. Chordomas are occasionally difficult to differentiate from chondroma and chondrosarcoma. An immunohistochemical analysis was able to prove, however, that the chordoma of this case significantly differed from chondroma and chondrosarcoma.

[Symptomatic Rathke's cleft cyst: a clinicopathologic study of 9 cases].

Nine cases of symptomatic Rathke's cleft cyst are reported. Their most frequent signs and symptoms included headache, chiasmal syndrome and hypopituitarism, while one of the cases developed a sudden onset of headache and vomiting following diabetes insipidus. Endocrinological findings showed a decreased ACTH, gonadotropin and growth hormone more frequently while there were 2 cases of hyperprolactinemia and 1 case of diabetes insipidus. In a neuroradiological examination, a plain skull X-ray showed 5 cases of ballooning of the sella turcica, and a CT scan demonstrated a low to high density of the cyst and 2 cases of marginal enhancement of the cyst. MRI mostly demonstrated a well delineated mass at the sella extending mostly into the suprasellar region and a low to high intensity of the cyst in the T1-weighted image. Two cases were marginally enhanced after gadolinium DTPA administration. The pathological examination, done on 6 cases, showed either single or multiple layers of the epithelium which were mostly ciliated. The epithelium was positive in PAS and Alcian blue in all cases and a histochemical examination showed 3 cases to be positive in EMA and 2 cases positive in CEA. A resection of the cyst wall and an opening of the cyst is thus recommended in symptomatic cases. Therefore, the transsphenoidal approach should be the choice of treatment in an intra- and suprasellar extension of the cysts with sellar enlargement.(ABSTRACT TRUNCATED AT 250 WORDS)

[Clinicopathological evaluation of EVAL embolization in arteriovenous malformations].

The clinical and pathological aspects of two large arteriovenous malformations which were removed totally after preoperative embolization using ethylene vinyl alcohol copolymer (EVAL) were studied. The material, which is not adhesive, is handled easily during the procedure of embolization. However, it involves some risks because it might migrate to and occlude the normal branches of the brain and pass through the nidus to the venous system. Histopathological study of AVM nidus which was removed showed embolic materials within the vessels and inflammatory reaction of the vessel wall and its surrounding tissue. There were patchy hemorrhages within the AVM nidus and its surrounding brain tissue. Recanalization was also found within the occluded vessels. These findings suggest that preoperative embolization has some risk of causing intracerebral hemorrhage after the embolization. AVM should be removed surgically as a radical treatment if the patient is able to tolerate the operation.

[Clinicopathological study of meningiomas of the tentorium and its surrounding structures].

We report the clinical features, radiological studies, operative procedures and results, and follow-up data in 29 patients with meningiomas of the tentorium and its surrounding structures. The cases represented 22.5% of all the intracranial meningiomas operated on in a 15 year period and were divided into three groups, depending on their main attachments, tentorial, cerebellopontine angle (dorsal aspect of the petrous ridge) and others. Tumor size was generally large and 13 cases were larger than 5 cm. The most common tumor site was along or near the superior petrosal sinus and transverse-sigmoid junction in cases involving the tentorium, and medial to the porus acousticus in cases involving the cerebellopontine angle. Different operative approaches to these tumors were carried out, depending on their location. The tumors in the lateral or medial petrous ridge were approached mainly with a suboccipital craniectomy using a retromastoid incision. Total removal was carried out in 80% of the tentorial cases, in 46.2% of cerebellopontine angle cases, and in the 83.3% in the others. Total operative mortality rate was zero. Follow-up periods ranged up to 5 years 5 months in the tentorial cases, 4 years 6 months in cerebellopontine angle cases, and 7 years 1 month in the others. Long-term results were good in 21 cases (72.4%), fair in 3 cases (10.3%) and poor in 2 (6.9%). Three patients died due to tumor recurrence. One of them suffered lung metastasis, and two of them suffered extensive local recurrences. We recommend the retromastoid approach combined with the petrosal approach, if the CPA tumor is large enough and extends to the retroclival region.

Papillary meningioma with pulmonary metastasis. Case report.

Successful surgical treatment by extirpation of remote metastases is extremely rare. A patient was admitted in whom multiple metastases to the lung occurred 8 years after extirpation of a papillary meningioma of the posterior fossa. The meningioma did not recur at the original site. The pulmonary metastases were surgically removed. The histological and electron microscopic findings of this case are presented.

[Benign osteoblastoma of the parietal bones].

Benign osteoblastoma accounts for less than 1% of the primary bone tumors and the calvarial lesion is extremely rare. There are only 12 reported cases in the literature as far as we could collect. We have presented a case of benign osteoblastoma originated from the parietal bone. This 9-year-old boy struck his head on the parietal region and noticed the bulging at the same site. A month later he visited to Fukuoka University Hospital because of persistent bulging of the same site. Neurological examinations were normal. Plain skull roentgenogram on Towne view showed radiolucent area in the midline of parietal bones with irregular margins and in tangential view the outer table revealed thinning and expanding. Coronal CT scan demonstrated same abnormality and intact inner table. The tumor was located within the dipole in the bilateral parietal bones and was removed by a simple curettage. The microscopic feature showed numerous osteoblasts, osteoblastic rimming and many multinucleated giant cells. These indicated a typical benign osteoblastoma. He has been doing well one year after the operation without any evidence of recurrence.