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Background: Growing teratoma syndrome(GTS)is the progression of a mature teratoma during or following radiochemotherapy for germ cell tumors. We report two surgical cases of GTS. CASE 1: A 24-day-old new-born presented with vomiting and head enlargement. Blood alfa-feto protein(AFP)and beta-human chorionic gonadotropin(β-hCG)were within or at the upper limits of the normal ranges. Magnetic Resonance Imaging(MRI)demonstrated a large mass in the posterior fossa causing the severe hydrocephalus. Tumor removal was immediately performed. Histological diagnosis given was immature teratoma. While chemotherapy effectively reduced the level of tumor makers, multiple recurrence was noticed on MRI 70 days after the surgery. GTS was suspected and total removal was performed. Histological examination revealed a mature teratoma. The patient is growing normally thereafter, 2.5 years after the onset. CASE 2: A 16-year-old male presented with binasal hemianopsia. Blood AFP and β-hCG were within or at the upper limits of the normal ranges. MRI demonstrated an intrasellar mass protruding upward. Tumor removal was performed and histological diagnosis given was mixed germ cell tumor. While radiochemotherapy effectively normalized the tumor makers, recurrence was noticed on MRI 190 days after the surgery. Total removal was performed with the diagnosis of GTS. Histological examination revealed a mature teratoma. The patient lives a normal school life thereafter as followed up after a year after the onset., Conclusion: It is important to diagnose and perform the surgery early enough to enable total removal of the mass presenting as GTS because total surgical removal is the only treatment for GTS.

We reported an extremely rare case of cerebellar hemangioblastoma with marked pleomorphism and reviewed the literature. A 68-year-old male presented with a one-month history of headache and vomiting. Neurological examination revealed right-sided dysmetria and truncal ataxia. Contrast-enhanced T1-weighted MR imaging revealed a heterogeneously enhancing tumor with solid and cystic components in the right cerebellum. The solid portion of the tumor was low intensity on diffusion-weighted imaging and low intensity on susceptibility-weighted imaging. 18 F-fluorodeoxyglucose PET showed low uptake in the cerebellar tumor and the whole body examination was negative for malignancy. Vertebral angiogram demonstrated moderate tumor staining and no early filling veins. The patient underwent total removal of the tumor through suboccipital craniotomy. Microscopically, the solid tumor contained a cellular rich component consisting of stromal cells and a markedly pleomorphic component including atypical and multinucleated giant cells. The MIB-1 positive rate was 8.2%, which was slightly higher compared to that of hemangioblastomas. We observed strong staining for inhibin-α, aquaporin 1 and neuron specific enolase (NSE) in the tumor cells. PAX-2, cytokeratin and epithelial membrane antigen (EMA) were completely negative in the tumor cells, whereas the tumor cells demonstrated focal staining for CD10. The histological diagnosis was hemangioblastoma. Follow-up MR images showed no evidence of recurrent tumor 14 months after the resection. The study using a combination of immunohistochemical markers (e.g. inhibin-α, aquaporin 1 and PAX-2) is useful for differential diagnosis of hemangioblastoma from metastatic renal cell carcinoma.

Gliomatosis cerebri is a diffuse growth pattern of glioma consisting of exceptionally extensive infiltration of at least three cerebral lobes. We report a case of histologically confirmed glioblastoma multiforme in the cerebellar vermis which occurred 9 years after treatment for gliomatosis cerebri. A 33-year-old woman presented to our department for evaluation of visual disturbance. T2-weighted magnetic resonance (MR) imaging revealed hyperintense lesions in the bilateral frontal and parietal lobes. Histological examination of biopsy specimens from the left frontal lobe lesion demonstrated diffuse infiltration of glial neoplastic cells with preservation of the underlying cytoarchitecture, leading to the diagnosis of gliomatosis cerebri. She received 60 Gy hyperfractionated irradiation to the whole brain, and the lesion responded partially. The patient remained stable for 4 years, but T2-weighted MR imaging 5 years after the initial treatment showed enlargement of the hyperintense area. She received nimustine hydrochloride chemotherapy, and again partial response was observed. However. T1-weighted MR imaging after administration of gadolinium-diethylenetriaminepenta-acetic acid detected enhanced lesions in the cerebellar vermis, cerebellar hemisphere, and left posterior limb of the internal capsule 9 years after the initial treatment, although no abnormal findings were observed on initial and follow-up MR imaging. She underwent subtotal removal of the lesion in the cerebellar vermis. The surgical specimens were characterized by dense proliferation of atypical tumor cells with scattered mitosis and endothelial proliferation. The histological diagnosis was glioblastoma multiforme. The patient received gamma knife irradiation for the remnant lesion in the cerebellar vermis, and the lesions in the cerebellar hemisphere and left posterior limb of the internal capsule, and chemotherapy with temozolomide. However, multiple enhanced lesions were detected in the cerebellar vermis 2 months after the start of the temozolomide chemotherapy, and she died 8 months later. This case suggests that glioblastoma multiforme could develop in the long term after initial treatment for gliomatosis cerebri, and in a location separate from the initial lesion.

We reported a rare case of cervical glioblastoma with intracranial dissemination at an early stage of clinical course and reviewed the literature. An 8-year-old girl presented with failure of vision 3 months prior to admission to our hospital. Neurological examination on admission disclosed no definitive abnormalities except for bilateral visual disturbance and optic atrophy. Cranial MR images revealed a homogeneously enhancing tumor in the left sylvian fissure. Multiple spotty T2-hyperintensity lesions without contrast enhancement were also disclosed in bilateral cerebellum. Spinal MR images showed an enhancing tumor at C7 and tiny enhancing lesions on the surface of T11. The patient underwent an uneventful excision of the exophytic tumor at C7. The histological diagnosis was glioblastoma. The enhancing tumor in the left sylvian fissure treated by Linac stereotactic radiotherapy with a marginal dose of 38.4Gy in 12 fractions has diminished, whereas the residual tumor at C7 remained unchanged after radiation of 44Gy. In contrast, the multiple spotty lesions without contrast enhancement dispersedly spread in the cerebellum and infiltrated into brain stem despite 4 courses of chemotherapy using ifosfamide, cisplatin, and etoposide. Stereotactic biopsy of the multiple spotty lesions in the cerebellum was performed. Histological examination revealed anaplastic astrocytoma. The patient died 2 weeks after the biopsy despite additional chemotherapy and focal irradiation to the cerebellum. Early detection and selection of optimal therapeutic strategies are important in management of spinal glioblastoma with subarachnoid dissemination, since neuroradiological findings and therapeutic sensitivity are varied according to differentiation of disseminated tumors.

We reported a rare case of pleomorphic TSH-producing pituitary adenoma with calcification and reviewed the literature. A 25-year-old female was admitted to our hospital with a complaint of anterior neck swelling. An endocrinological examination demonstrated elevated serum levels of free triiodothyronine (FT3: 5.6 pg/ml), free thyroxin (FT4: 2.2 ng/dl), TSH (5.85 microIU/ml), and TSH a-subunit (5.9 ng/ml), whereas a physical examination revealed no goiter. CT scan showed a suprasellar isodensity mass with dense calcification. Contrast-enhanced T1-weighted images revealed a less enhancing tumor extending from the left upper portion of pituitary fossa into suprasellar cistern. The patient underwent gross total removal of the tumor via the right pterional approach. Microscopically, medium-sized to enlarged tumor cells with marked pleomorphism and prominent calcification were observed. The tumor cells displayed positive reaction for TSH (beta-subunit). The MIB-1 index averaged 2.9%. The histological diagnosis was a pleomorphic TSH-producing pituitary adenoma. Postoperatively, the serum levels of FT3, FT4, TSH, and TSH alpha-subunit decreased to normal range. Follow-up MR images showed no evidence of recurrent tumor 3 years after the resection. All of six patients with densely calcified TSH-producing pituitary adenoma, previously reported in the literature, remained well without tumor recurrence. We suggest that this type of TSH-producing pituitary adenoma may be associated with favorable prognosis despite histologically pleomorphic appearance.

We reported a rare case of supratentorial primitive neuroectodermal tumors (sPNET) and reviewed the literature. A 15-year-old girl presented with a one-month history of headache and vomiting. CT scans showed a huge, solid and cystic tumor with calcification, occupying the left anterior cranial fossa. The solid portion of the tumor was hypointense on T1-weighted images, slightly hyperintense on T2-weighted images, hyperintense on diffusion- weighted images, isointense on fluid-attenuated inversion recovery (FLAIR) images, and strongly enhanced after administration of contrast medium. The expansile tumor had a broad attachment to the dura matter of the anterior cranial fossa. The patient underwent an uneventful extirpation of the tumor. Microscopically, the solid tumor contained small, round poorly-differentiated cells with pleomorphic nuclei and brisk mitotic activity. The tumor cells were immunoreactive for synaptophysin and GFAP, whereas lack of MIC2 gene product expression was confirmed using the monoclonal antibody 12E7. The MIB-1-positive rate was 43%. The histological diagnosis was sPNET. Postoperatively, a dose of 30.6Gy was administrated to the whole brain and a boost of 19.6Gy to the T2-hyperintensity lesion. The patient successively received a dose of 30.4Gy to the whole spine and Linac stereotactic radiotherapy with a marginal dose of 16.8Gy at the tumor bed. Three months after radiotherapy the patient received chemotherapy using carboplatin and etoposide. Follow-up MR images showed no evidence of recurrent tumor 5 months after the radiochemotherapy. On the basis of MR findings on both diffusion-weighted and FLAIR images, preoperative diagnosis of sPNET may be important for choosing appropriate therapeutic strategies for this tumor.

A rare case in presented of a patient with edema formation along the optic pathway, which was caused by metastatic suprasellar tumor mimicking craniopharyngioma. A 63-year-old female with visual disturbance, diabetes insipidus, and symptomatic panhypopituitarism was admitted to our hospital. CT images showed a suprasellar mass lesion. MR images demonstrated a heterogeneously enhanced tumor and edema along the optic nerve and tract. Although no cystic component is shown in radiological findings, these features are commonly observed in cases of craniopharyngioma. The patient underwent removal of the tumor by the transcranial approach. Since histological examination revealed adenocarcinoma, the tumor was diagnosed as metastatic brain tumor originating from non-recurrent rectal cancer operated on eight years previously. Conventional radiotherapy with a dose of 50GY was performed for the residual tumor. The tumor size was unchanged, but the edema had disappeared 7 months after the operation. Edema spreading along the optic tract was reported as a characteristic MR finding for diagnosis of craniopharyngiomas as distinguished from other common parasellar tumors. However, it is suggested that edema of the optic pathway seems to be caused not only by craniopharyngioma but also other suprasellar lesions. Even if there was a characteristic radiological finding for some specific diseases, metastasis should be kept in mind as a differential diagnosis, because of its various appearance in neuroimages.

We report a rare case of a primary intracerebral malignant fibrous histiocytoma (MFH) with malignant progression from a premalignant stage. A 73-year-old male presented with mild hemiparesis on the right side of his body 4 months prior to admission. MR images showed a homogenously enhanced mass in the left parietal lobe. The patient underwent an uneventful extirpation of the solid mass. Microscopically, the solid tumor contained histiocytic cells and spindle-shaped fibroblastic cells arranged in a storiform pattern. The tumor cells were immunoreactive for vimentin, MAC 387 (macrophage marker) and alpha-1-antitrypsin. The MIB-1-positive rate was 1%. The histological diagnosis was fibrous histiocytoma. Postoperative MR images demonstrated no abnormally enhanced lesions. The patient's neurological symptoms and signs gradually improved. One year after surgery, he developed right hemiparesis. MR images showed a heterogeneously enhanced mass in the left sensorimotor area. This recurrent tumor was totally resected. Microscopically, the tumor was composed of atypical spindle-shaped cells, forming a storiform pattern. Pleomorphism was prominent, and necrotic foci were also seen. The MIB-1 labeling index averaged 11%. The histological findings corresponded to those of MFH. Unfortunately, the patient died of interstitial pneumonia contracted during postoperative radiotherapy. Early detection and resection at a stage of low malignant potential, followed by radiochemotherapy, are crucial for the management of this mesenchymal tumor.

We reported a rare case of third ventricular chordoid glioma and reviewed the literature. A 25-year-old male presented with a two-year history of voracious appetite. Three months prior to admission, he developed progressive memory impairment. CT scan showed a well circumscribed, slightly hyperdense mass without calcification in the third ventricle. The 4.5-cm oval mass occupying the anterior part of the third ventricle was homogeniously enhanced on MR images after administration of contrast medium. Small cystic components were present in the periphery of the mass. CT-guided stereotactic biopsy was performed. Microscopically, epithelioid tumor cells were embedded in mucinous stroma containing a lympho-plasmacytic infiltration. The tumor cells were immunoreactive for GFAP and vimentin. The MIB-1-positive rate was 1.2%. The histological diagnosis was a chordoid glioma. One month after the biopsy, a right frontal craniotomy was performed to remove the tumor via the anterior transcallosal interfornitial approach. The tumor arising from the anterior part of the third ventricle wall was soft, mildly vascular and light-grayish. The tumor was completely resected. The histological findings were identical with those of the biopsy specimens. Postoperatively, the patient developed diabetes inspidus, hyperthermia, worsening memory impairment, and transient hyponatremia. The patient's symptoms gradually improved and follow-up MR images showed no evidence of recurrent tumor 17 months after the resection. Despite low-grade appearance, attachment of the tumor to the hypothalamus preclude complete resection, and this may result in tumor recurrence and less favorable prognosis in the current reports. Early detection and aggressive resection followed by stereotactic radiotherapy are important in the management of chordoid glioma.

Orbital neuroma is a rare disease accounting for a certain percentage of all orbital tumors. However, because of the anatomical features of the orbit, the origin of the tumor has been diagnosed in only a few cases. The authors report a case of orbital neuroma arising from a short ciliary nerve that was confirmed during surgery. The patient was a 72-year-old female. She visited our hospital with complaints of left visual disturbance, exophthalmus and diplopia. Neurological examination on admission also revealed Marcus gunn pupil sign. CT scan and MRI imaging showed a left orbital tumor located along the superior surface of the optic nerve and adjacent to the posterior pole of the eye ball. She underwent superior orbitotomy through a transcranial approach. The tumor was encapsulated and adhered tightly to the sclera, short ciliary nerve, and optic nerve. For this reason, it was removed without a part of the adhered capsule. Since histological examination revealed neuroma, the tumor was diagnosed as short ciliary nerve neuroma. Postoperatively no additional deficits occurred. The exophthalmus, diplopia and Marcus gunn pupil sign disappeared and visual acuity was improved after the surgery. In such a case of short ciliary nerve neuroma, it is possible to remove the tumor without any neurological deficit, if careful consideration of microsurgical anatomy is made.

A case of frontal atypical teratoid/rhabdoid tumor (AT/RT) was presented in the findings of single-photon emission computerized tomography (SPECT) using 201Tl-chloride (Tl) and 123I-metaiodobenzylguanidine (MIBG), and positron emission tomography using 18F-fluorodeoxyglucose (FDG) and (11C-methyl)-L-methionine (Met). A 16-year-old female had an episode of Jacksonian seizures one month prior to admission. CT scans showed an isodensity mass with heterogeneous enhancement in the left frontal lobe. MR images revealed a tumor with solid and cystic components and perifocal edema. SPECT demonstrated intense accumulation of Tl and MIBG in the enhancing lesion 15 min and 30 min after intravenous injection of tracers, respectively. The 6-hr delayed SPECT showed no retention of MIBG in the enhancing lesion. FDG-PET and Met-PET revealed high uptake of tracers in the enhancing lesion. Met did not accumulate in the frontal white matter, which appeared hyperintense on T2-weighted MR images. The patient underwent an uneventful extirpation of the solid mass, where Met had accumulated. Microscopically, the solid tumor contained rhabdoid cells, spindle-shaped cells resembling mesenchymal cells, and nests of small cells. The tumor cells were immunoreactive for vimentin, cytokeratin, and epithelial membrane antigen. The MIB-1 labeling index was 25%. The histological diagnosis was AT/RT. Postoperative course was uneventful. A dose of 32.4 Gy was administrated to the whole brain and a boost of 27.8 Gy to the T2-hyperintensity lesion. Five months after the radiotherapy, MRI showed neither abnormal enhancing lesions nor the T2-hyperintensity lesion. Multifarious studies using SPECT and PET are useful for differential diagnosis and for choosing optimal therapeutic strategy for this type of tumor.

A case of frontal hypertrophic cranial pachymeningitis was presented with positron emission tomography (PET) using (11C-methyl)-L-methionine (11C-Met). A 55-year-old male developed right hemiparesis after generalized tonic convulsion one month prior to admission. MR images revealed patchy enhancement extending from the dura mater to the cerebral parenchyma surrounding high T2-weighted signal in the left frontal region. Left carotid angiogram showed atresia of the rostral superior sagittal sinus and obliteration of the cortical veins associated with compensatory venous channels coursing in the frontal deep white matter. PET demonstrated high 11C-Met uptake in the area corresponding to the enhancing lesion on the MR images. The ratio of lesion/normal cortex was 1.58 as an indicator of selective uptake in the lesion. The contralateral temporal gray matter was representative of a normal cortex. In contrast, 11C-Met did not accumulate in the frontal white matter where T2-weighted MR images showed abnormal high intensity lesion. This suggested that the frontal white matter lesion was derived from vasogenic edema due to venous infarction. The patient underwent an uneventful exploratory biopsy. The dura mater had proliferated to a thickness of 3.5mm and was tightly adherent to the left middle frontal gyrus. Microscopically, the thickened dura mater where the tracers had accumulated was composed of abundant collagenous fibers together with diffuse infiltration of inflammatory cells, including predominantly lymphocytes, plasma cells, and neutrocytes. The lymphocytes, which proved positive in both UCHL-1 and L26 staining, had no atypism. Histological findings corresponded to hypertrophic pachymeningitis. Met-PET clearly represented viable and infiltrative zones of inflammatory cells. The patient's neurological symptoms and signs gradually improved. Follow-up MR images three months after the surgery showed the enhancing lesion to be diminished and a marked regression of the vasogenic edema. Spatial determination of viable lesions permitting differentiation from biological inactive or vasogenic edema is an important guideline in selecting an appropriate surgical procedure in the diagnosis and treatment of hypertrophic cranial pachymeningitis. Met-PET would appear useful in delineating inflammatory lesions such as hypertrophic cranial pachymeningitis.

The aim of this study was to evaluate effects of radiochemotherapy on blood flow and metabolism using C15O2, C15O, 15O2, and 18F-fluorodeoxyglucose positron emission tomography (PET). Conventional, external radiotherapy (180-200 cGy/5 fractions/week) using 60Co or a 10 MV linear accelerator was given in conjunction with chemotherapy of nimustine hydrochloride (ACNU) and tegafur (FT-207) in eight patients with gliomas. Regional cerebral blood flow (rCBF), blood volume (rCBV), oxygen extraction fraction (rOEF), oxygen consumption (rCMRO2), and glucose consumption (rCMRG1) were longitudinally examined before the treatment, and at an early stage (within one month) and a late stage (three to thirty-one months with a mean of 12 months) after radiochemotherapy. Contralateral gray and white matter to the tumor was selected as regions of interest (ROIs), because these portions were irradiated with larger doses compared to other brain parenchymal regions through parallel opposing fields. Maximum doses to the ROIs varied between 20 and 61 Gy. Calculated as a function of time-dose-fractionation, nominal standard doses (NSD) ranged from 566 to 1729 ret, and 344 to 1062 neuret; equivalent doses (ED) equaled 432 to 1327 rads. At the early stage, rCBF and rCBV increased in the contralateral gray and white matter; gray matter rOEF decreased significantly (p less than 0.05 by a paired-t test) from that of the pretreatment study, probably due to decompression of the tumor bulk and radiochemotherapy.(ABSTRACT TRUNCATED AT 250 WORDS)

A high resolution positron emission tomography (PET), HEADTOME III, has enabled us to visualize heterogeneous parts, i.e. viable, necrotic, and edematous portions in malignant gliomas, and to quantify regional hemocirculation and metabolism of the tumors using 15O and 18F-fluorodeoxyglucose tracers. Hemocirculatory and metabolic indices of regional cerebral blood flow (rCBF), blood volume (rCBV), oxygen extraction fraction (rOEF), oxygen consumption (rCMRO2) and glucose consumption (rCMRGl) were studied in eight patients with untreated malignant gliomas. Regions of interest (ROIs) in PET images were focused on lesions corresponding to contrast enhancing areas, central low density areas of the tumors, and peritumoral low density areas in CT scans. In the viable portion of the gliomas, rCBV (5.20 +/- 1.18ml/100ml, mean +/- SD, n = 8) was significantly higher than that of the contralateral gray matter (p less than 0.05), which is suggestive of high vascularity; rOEF (0.36 +/- 0.16) and rCMRO2 (1.66 +/- 0.45ml/100ml/min) values markedly decreased (p less than 0.05, p less than 0.01). On the other hand, rCBF (36.3 +/- 13.0ml/100ml/min) and rCMRGl (5.94 +/- 1.15mg/100ml/min) were similar to that of the contralateral gray matter. A relative dissociation between oxygen and glucose metabolism indicates anaerobic glycolysis in the energy metabolism of malignant gliomas. In the central low density area, rCBF, rCBV, rOEF, rCMRO2, rCMRGl values decreased significantly from the viable portion and the contralateral gray matter. rOEF was markedly reduced in the central low density area as compared with that of the peritumoral low density area. The rOEF reduction indicates that oxygen metabolism of gliomas is the first to fail, accompanied by autoregulatory impairment of vessels.(ABSTRACT TRUNCATED AT 250 WORDS)