Epidermal Growth Factor Receptor Is Related to Poor Survival in Glioblastomas: Single-Institution Experience

PURPOSE: There are conflicting results surrounding the prognostic significance of epidermal growth factor receptor (EGFR) status in glioblastoma (GBM) patients. Accordingly, we attempted to assess the influence of EGFR expression on the survival of GBM patients receiving postoperative radiotherapy. MATERIALS AND METHODS: Thirty three GBM patients who had received surgery and postoperative radiotherapy at our institute, between March 1997 and February 2006, were included. The evaluation of EGFR expression with immunohistochemistry was available for 30 patients. Kaplan-Meier survival analysis and Cox regression were used for statistical analysis. RESULTS: EGFR was expressed in 23 patients (76.7%), and not expressed in seven (23.3%). Survival in EGFR expressing GBM patients was significantly less than that in non-expressing patients (median survival: 12.5 versus 17.5 months, p=0.013). Patients who received more than 60 Gy showed improved survival over those who received up to 60 Gy (median survival: 17.0 versus 9.0 months, p=0.000). Negative EGFR expression and a higher radiation dose were significantly correlated with improved survival on multivariate analysis. Survival rates showed no differences according to age, sex, and surgical extent. CONCLUSION: The expression of EGFR demonstrated a significantly deleterious effect on the survival of GBM patients. Therefore, approaches targeting EGFR should be considered in potential treatment methods for GBM patients, in addition to current management strategies.

A Case of Gerstmann-Straussler-Scheinker Disease

BACKGROUND: Gerstmann-Straussler-Scheinker disease (GSS) is a type of human transmissible spongiform encephalopathy (TSE) that is determined genetically. CASE REPORT: A 46-year-old woman presented with a slowly progressive ataxic gait and cognitive decline. She was alert but did not cooperate well due to severe dementia and dysarthria. High signal intensities in the cerebral cortices were evident in MRI, especially in diffusion-weighted images (DWI). A prion protein gene (PRNP) analysis revealed a P102L (proline-to-leucine) mutation in codon 102. CONCLUSIONS: This is the first reported case of GSS (confirmed by PRNP analysis) in Korea. Distinctive MRI findings are also presented.

Pituitary Hemorrhage : Classification and Related Factors

OBJECTIVE: Clinical features of pituitary hemorrhage vary from asymptomatic to catastrophic. The purpose of this study was to evaluate the factors related to severity of hemorrhage of pituitary adenoma. METHODS: Pituitary hemorrhage was noted in 32 of 88 patients who underwent operations between January 2000 and December 2007. Clinical status was classified into group I (no hemorrhage symptoms), II (mild to moderate symptoms without neurological deficit), and III (with neurological deficit), and was compared to radiological, pathological, and operative findings. All patients were operated by transsphenoidal approach, and hemorrhage-related symptoms were relieved. RESULTS: Groups I, II, and III comprised 15, 10 and 7 patients, respectively. In group I, hemorrhage volume was under 1 mL in 11 (73.3%), but, it was above 1 mL in 7 (70%) of group II and in all cases of group III. Hemorrhage stage based on MRI findings was chronic or subacute in 11 (73.3%) of group I, acute in 6 (60%) of group II, and acute or hyperacute in 6 (85.7%) of group III. Pathological examination revealed chronic-stage hematomas in 5 (50%) group II patients. Functioning adenomas were found in 5 (33.3%) group I patients but none in group II or III patients. Silent adenomas were found in 4 (26.7%), 8 (80%), and 3 (42.9%) in groups I, II, and III, respectively. CONCLUSION: Clinical features of pituitary hemorrhage may differ with the radiological and immunohistopathlogical findings. Persistent symptoms are related to the chronic stage of hematoma requiring surgery for symptom relief. Neurological deficits are caused by large amount of acute hemorrhage requiring emergency operation. Silent adenoma is related to the severity of pituitary hemorrhage.

Spontaneous Intraorbital Hemorrhage : A Case Report

Intraorbital hemorrhage is a rare clinical condition caused by orbital trauma, surgery around the orbit, intraorbital vascular abnormalities, and neoplasm. It was reported to occur spontaneously without any known causes and in association with orbital pseudotumor in a very few cases. A 59-year-old, female patient admitted with sudden onset of severe exophthalmos and pain on the left eye. Orbital CT and MR imaging suggested hemorrhage in the upper part of retrobulbar area of the left orbit. Cerebral angiography was taken to rule out any possible vascular abnormalities. On the left carotid cerebral angiography, the run-off of the distal ophthalmic artery was not seen and the engorgement of the supraophthalmic artery was noted. Systemic administration of corticosteroid did not improve the clinical status and craniectomy was done and retrobulbar hematoma was removed, and the clinical symptoms and signs were improved. Authors report a case of spontaneous intraorbital hemorrhage with the clinical features similar to those of orbital pseudotumor, requiring surgical decompression.

Ewing's Sarcoma Mimicking a Meningioma in Radiological Findings: A Case Report

Ewing's sarcoma is an uncommon primary bone tumor. Primary Ewing's sarcoma of the cranium is extremely rare and constitutes only 1% of all Ewing's sarcoma cases. Usually, primary Ewing's sarcoma of the carnium manifests as an expansile osteolytic malignant bone tumor with or without intracranial extension. We report here the radiological findings of a case of Ewing's sarcoma mimicking a meningioma in an 18-year-old man.

The Increase in Hepatic Uncoupling by Fenofibrate Contributes to a Decrease in Adipose Tissue in Obese Rats

Fenofibrate is a drug that has been suggested to inhibit weight gain by increasing the catabolism of fatty acid in the hepatic mitochondria. We hypothesized that fenofibrate induces an increase in energy expenditure in the hepatic mitochondria, which results in the reduction of adipose tissue. In this study we measured hepatic uncoupling protein (UCP)-2, -3, core temperatures and abdominal fat composition with MRI in Otsuka Long-Evans Tokushima Fatty rats. The fenofibrate group (n=7) was fed fenofibrate (320 mg/kg) mixed chow. The control group (n=7) was fed chow only. The body weight (531.6+/-7.6 g) of the fenofibrate group was significantly lower than that (744.3+/-14.9 g) of the control group (p<0.005). The areas of visceral and subcutaneous fat in the fenofibrate group (11.0+/-0.9 cm2, 4.2+/-0.3 cm2) were significantly less than those in the control group (21.0+/-0.7 cm2, 7.4+/-0.4 cm2) (p=0.046, respectively). The esophageal and rectal temperatures of the fenofibrate group (37.7+/-0.1 degrees C, 33.1+/-0.2 degrees C) were significantly higher than those of the control group (37.3+/-0.1 degrees C, 32.2+/-0.1 degrees C) (p=0.025, p=0.005). There was de novo expression of UCP-3 in the liver of the fenofibrate group. These data suggest that increased energy dissipation, via hepatic UCP-3 by fenofibrate, contribute to decreased weight gain in obese rats.

Impact of Cyclooxygenase-2 Expression on the Survival of Glioblastoma / 대한방사선종양학회지

PURPOSE: To investigate the degree and effect of cyclooxygenase (COX)-2 expression on the survival of patients with glioblastoma multiforme (GM). MATERIALS AND METHODS: Between 1997 and 2006, thirty consecutive GM patients treated with surgery and postoperative radiotherapy (dose range: 44~65.1 Gy, median dose: 61.2 Gy) were included in the study. Three patients were excluded that discontinued radiotherapy before receiving a dose of 40 Gy due to mental deterioration. The expression of the COX-2 protein in surgical specimens was examined by immunohistochemical analysis. Survival analysis and verification were performed with respect to sex, age, performance status, resection extent, radiotherapy dose, and degree of COX-2 expression using the Kaplan-Meier method and the log rank test. RESULTS: The median length of follow-up was 13.3 months (range: 6~83 months). Staining for COX-2 was positive in all patient samples. Staining for COX-2 that was positive for over 75% of the tumor cells was found in 24 patients. Staining for COX-2 that was positive in less than 25% of tumor cells was found in 3 patients (10.0%), staining for COX-2 that was positive in 25 to 50% of tumor cells was found in 1 patient (3.3%), staining for COX-2 that was positive in 50 to 75% of tumor cells was found in 2 patients (6.7%) and staining for COX-2 that was positive in 75 to 100% of tumor cells was found in 24 patients (80.0%). The median survival and two-year survival rate were 13.5 months and 17.5%, respectively. The survival rate was influenced significantly by the degree of resection (tumor removal by 50% or more) and radiotherapy dose (59 Gy or greater) (p0.05), and the two-year survival for these groups was 33.3 and 13.3%, respectively (p>0.05). CONCLUSION: The absence of a statistical correlation between the degree of COX-2 expression and survival in GM patients, despite the high rate of COX-2 positive tumor cells in the GM patient samples, requires further studies with a larger series to ascertain the prognostic value of the degree of COX-2 expression in GM patients.

Primary Cerebellar Hodgkin's Lymphoma

Authors report a very rare case of primary Hodgkin's lymphoma limited to the cerebellum. A 64-year-old female patient presented with headache, nausea, and vomiting. Magnetic resonance imaging of the brain revealed a nodular enhancing mass in the left cerebellar hemisphere. Tumor was removed totally with retromastoid suboccipital approach. Diagnosis was Hodgkin's lymphoma of mixed cellularity type, consisting of a dense mixed inflammatory infiltrate containing scattered large atypical mononuclear Hodgkin cells and multinucleated Reed-Sternberg cells. Systemic work-up for the Hodgkin's lymphoma was followed with negative result. Postoperative radiation therapy was given and the patient is disease-free at 16 months after the operation.

Ectopic Growth Hormone-Secreting Pituitary Adenoma of the Clivus

Ectopic pituitary adenoma, occurring outside the sella turcica without any continuity with intrasellar pituitary gland is very rare. So far, less than 90 such cases have been reported in the literature. Regarding to ectopic locations, suprasellar region, sphenoid sinus and clivus have been reported in decending order of frequency. To our best knowledge, growth hormonesecreting ectopic pituitary adenoma in the clivus has never been reported. With the pertinent literature review, we present our unique case with its characteristic magnetic resonance imaging and immunohistochemical features.

Silent Adenomas of Pituitary Gland: It's Immunohistochemical Features and Clinical Characteristics

OBJECTIVE: The aim of the study was to review the clinical and radiological findings of those non-functioning adenomas(NFAs) with positive immnoreactivity for anterior pituitary hormones. METHODS: Sixty patients with pituitary adenoma were treated at the author's institution between January 2000 and July 2005. All consecutive patients were underwent transsphenoidal surgery by same operator. In addition to the routine histopathological examination, surgical specimen was examined by immunohistochemical staining against adenohypophyseal cells. And clinical analysis was performed by retrospective review of medical records, neuroimaging examinations and immunohistochemical technique. We classified these pituitary adenomas into functioning adenomas (group F), immunopositive NFAs (group S, so-called silent adenoma) and immuno-negative NFAs (group N), and compared clinical and radiological differences between group F, N, and S. RESULTS: Of the 60 cases, group F was 25, group S was 25, and group N was 10. Among the group S, 5 cases showed reactivity against PRL, 1 against GH, 1 against both PRL and GH, 1 against TSH and GH, 2 against ACTH, 11 against FSH and 4 against both LH and FSH. Radiologically, invasiveness was noted in 8 in group S, compared to 3 in group N and 1 in group F (p=0.02). Intratumoral bleeding was noted in 7 of group S, 2 of group N and 2 of group F (p>0.05). CONCLUSION: Silent adenomas were thought to behave more aggressive than other subgroups of pituitary adenomas. And so we suggest the immunohistochemical study against adenohypophyseal cells may be helpful for evaluating clinical course of pituitary adenoma, expecially for, NFAs.

Cystic Hemangiopericytoma in the Third Ventricle

Primary intracranial hemangiopericytoma is rare and resemble meningioma on imaging study. It shows meningeal attachment, and is usually isointense with gray matter on T1-weighted MR image with heterogeneous enhancement and prominent vascular flow voids on T2-weighted image. Cystic type of hemangiopericytoma is very rare and only 3 cases have been reported in the literature which arised in the middle fossa, cerebellum, and occipital area. Ventricular hemangiopericytomas were reported in 9 cases, and all of them were solid type. Authors experienced a peculiar case of cystic hemangiopericytoma in the 3rd ventricle and report it with review of the literature.

Angiosarcoma of the Temporal Region: A Case Report / 대한이비인후과학회지

Angiosarcoma is a rare malignant vascular tumor that most commonly arises in the skin and superficial soft tissues of the head and neck. It accounts for less than 1% of all sarcomas, and 50% of these lesions occur in the head and neck. There are some clinical characteristics, such as insidious presentation, multifocal nature, aggressive behavior and poor prognosis. We report a case of angiosarcoma in a temporal region. A 75-year-old man presented with a 2-month history of left bloody otorrhea. Previously, he had two operations for the left chronic otitis media at a local clinic. After the second operation, bloody otorrhea began and was not controlled. We conducted revision canal wall down operation again, but failed to control bloody otorrhea. Biopsy was performed at the posterior portion of the open cavity. Histologic examination showed necrosis and atypical vascular proliferation, consistent with angiosarcoma.

Three Dimensional Volume Rendering Virtual Endoscopy of the Ossicles Using a Multi-Row Detector CT: Applications and Limitations

PURPOSE: This study was conducted to know the applications and limitations of three dimensional volume rendering virtual endoscopy of the ossicles using a multi-row detector CT. MATERIALS AND METHODS: This study examined 25 patients who underwent temporal bone CT using a 16-row detector CT as a result of hearing problems or trauma. The axial CT scan of the temporal bone was performed with a 0.6 mm collimation, and a reconstruction was carried out with a U70u Sharp of kernel value, a 1 mm thickness and 0.5-1.0 mm increments. After observing the ossicles in the axial and coronal images, virtual endoscopy was performed using a three dimensional volume rendering technique with a threshold value of -500 HU. The intra-operative otoendoscopy was performed in 12 ears, and was compared with the virtual endoscopy findings. RESULTS: Virtual endoscopy of the 29 ears without hearing problems demonstrated hypoplastic or an incomplete depiction of the stapes superstructures in 25 ears and a normal depiction in 4 ears. Virtual endoscopy of 21 ears with hearing problems demonstrated no ossicles in 1 ear, no malleus in 3 ears, a malleoincudal subluxation in 6 ears, a dysplastic incus in 5 ears, an incudostapedial subluxation in 9 ears, dysplastic stapes in 2 ears, a hypoplastic or incomplete depiction of the stapes in 16 ears and no stapes in 1 ear. In contrast to the intra-operative otoendoscopy, 8 out of 12 ears showed a hypoplastic or deformed stapes in the virtual endoscopy. CONCLUSION: Volume rendering virtual endoscopy using a multi-row detector CT is an excellent method for evaluation the ossicles in three dimension, even though the partial volume effect for the stapes superstructures needs to be considered.

The Structures Mimicking Aneurysm on 3-Dimensional Volume Rendering CT Angiography Using A 16-Row Detector CT

PURPOSE: This study was conducted to identify the structures that can mimic aneurysm on CT angiography with using the 3-D volume rendering technique and a 16-row detector CT. MATERIALS AND METHODS: This study examined 206 patients who underwent CT angiography with a 16-row detector CT from May 2003 to July 2004. For the CT angiography, the contrast media was injected at a rate of 4 cc per second. The scanning was then done when 5 seconds delay time had elapsed after the CT number of the common carotid artery had reached 150. The baseline images were obtained by reconstructing the row data with a kernel value of H31f, a slice thickness of 1 mm and an increment of 0.5 mm. Finally, the 3-D images were constructed by using a volume rendering technique. The structures that were confused with aneurysm on CT angiography were further evaluated by MPR or MIP to ascertain whether they were aneurysms. For comparison, digital subtraction angiography was done in 86 patients and surgery was done in 5 patients. RESULTS: On CT angiography, 51 patients did not show any abnormalities, and 80 patients showed definitive aneurysms, as was ascertained by their sizes, shapes and locations, or by the aneurysm clips only. The remaining 75 patients showed 90 aneurysm mimicking structures that were 35 infundibular dilatations or preaneurysmal blebs, 24 focal dilatations of the branches of the deep middle cerebral veins, 6 focal dilatations of the sphenoparietal sinuses adjacent to the middle cerebral artery, 18 focal dilatations of the inferior sagittal sinuses adjacent to the pericallosal artery and 7 low density calcifications of the vessel walls that were adjacent to the aneurysm-prevalent arteries. CONCLUSION: CT angiography based on volume rendering technique was able to identify the structures that were confused with aneurysm. Accordingly, careful observation is required for interpreting the reconstructing 3-D images since a wide variety of structures can be confused with aneurysm on CT angiography.

A Case of Reversible Posterior Leukoencephalopathy Involving in the Spinal Cord with Pheochromocytoma

Reversible posterior leukoencephalopathy with hypertension is characterized by an acute and severe rise in blood pressure with headache, altered mental status, cortical visual disturbance, seizure and transient edematous changes in neuroimaging. The most common abnormality in neuroimaging is presumed edema involving the cortical and subcortical white matter predominant in the posterior region of the cerebral hemisphere and rarely the cerebellum and the brain stem, but not in the spinal cord. We experienced a case of 10-year-old girl with hypertensive encephalopathy involving the brainstem and the spinal cord.

Antitumor Activity of TRAIL Recombinant Adenovirus in Human Malignant Glioma Cells

Tumor necrosis factor-Related Apoptosis-Inducing Ligand (TRAIL) has been reported to specifically kill malignant cells but to be relatively nontoxic to normal cells. One of disadvantages to previous in vivo protocols was the need for large quantities of TRAIL recombinant protein to suppress tumor growth. To evaluate the antitumor activity and therapeutic value of the TRAIL gene, we constructed adenoviral vectors expressing the human TRAIL gene (Ad.hTRAIL) and transferred them into malignant glioma cells in vitro and tumors in vivo, as an alternative to recombinant soluble TRAIL protein. The results show that TRAIL-sensitive glioma cells infected Ad.hTRAIL undergo apoptosis through the production and expression of TRAIL protein. The in vitro transfer elicited apoptosis, as demonstrated by the quantification of viable or apoptotic cells and by the analysis of cleavage of poly (ADP-ribose) polymerase. Furthermore, in vivo administration of Ad.hTRAIL at the site of tumor implantation suppressed the outgrowth of human glioma xenografts in SCID mice. These results further define Ad.hTRAIL as an anti-tumor therapeutic and demonstrate its potential use as an alternative approach to treatment for malignant glioma.

Mondor's Disease of the Breast: Clinical Manifestations and Imaging Findings

Mondor's disease or thrombophlebitis of the superficial veins of the breast is a rare benign condition and a self-limiting disease. The combination of the clinical manifestations and the imaging (mammographic & sonographic) findings is the typical sign of Mondor's disease of the breast. Therefore, an understanding of such an entity and knowledge of the clinical and imaging findings will make it possible to avoid the unnecessary biopsy or surgery. We report the case of a 53-year-old woman diagnosing as Mondor's diasease, who underwent evaluation of a tender, papable cord- like mass in her left breast associated with discoloration of the overlying skin.

Transarterial Embolotherapy in Patients with Duodenal Hemorrhage Using Microcoils and Gelfoam Particles

PURPOSE: To assess the efficacy and safety of arterial embolotherapy in patients with massive duodenal hemorrhage. MATERIALS AND METHODS: Between January 1999 and June 2002, 25 patients (age: 34-81, mean 58, male: 19, female: 6) underwent arterial embolization for duodenal hemorrhage after failed endoscopic therapy. The hemorrhage originated from duodenal ulcer in sixteen patients, from cancer with duodenal invasion in five patients, from endoscopic sphincterectomy in two patients, and from pseudoaneurysm complicating acute pancreatitis in two patients. Hemorrhage was detected at endoscopy and an attempt was made to treat it endoscopically in all patients, but failed in each case. At angiography, direct bleeding signs such as contrast extravasation or pseudoaneurysm were demonstrated in nineteen patients. In the six patients without angiographic evidence of bleeding, blind embolization of the gastroduodenal artery was performed based on the endoscopic examination. Microcoil and gelfoam particles were used as embolic agents. RESULTS:Hemostasis was achieved immediately after embolotherapy in 21 patients (84%). Bleeding recurred in 4 patients (16%), and of these cases, one was successfully treated purely by endoscopic means, a second was reembolized three times due to bleeding from the collateral vessels of the tumor and the two others were treated by surgery. After the procedure, six patients died (24%). The causes of death were disseminated intravascular coagulopathy, multiorgan failure, sepsis and acute renal failure. The underlying diseases of the deceased patients were cancers with duodenal invasion (n=4) and abdominal aortic aneurysm with ischemic colitis (n=1). CONCLUSION: Transarterial embolotherapy in the case of massive duodenal hemorrhage is a safe and effective procedure. Even in the absence of angiographic evidence of bleeding, blind embolization of the gastroduodenal artery is effective for patients in the surgically high risk group.

A Case of Acute Necrotizing Encephalopathy with a Thalamic Hemorrhage

Acute necrotizing encephalopathy predominantly affects young children and infants living in Japan and Taiwan, and is characterised by acute encephalopathy with seizures and decreased level of consciousness. The Hallmark of the disease is diffuse and symmetrical CNS lesions of both thalami, brainstem tegmentum, cerebral periventricular white matter and cerebellar medula. The clinical, radiological and pathological features of this disease, a disease entity established recently, is proposed by Masashi Mizuguchi et al in 1995. The aetiology is unknown but infectious or parainfectious process seems likely. The diagnosis can be made without difficulty on the basis of the combination of a typical clinical figures and characteristic radiologic findings. There is no specific therapy or prevention. The prognosis was poor in the 1980s but has improved recently. We experienced a case of 6-month-old female infant with acute necrotizing encephalopathy and a thalamic hemorrhage. We report this case with a review of the related literatures.

Changes in Total Cerebral Blood Flow with Aging, Parenchymal Volume Changes, and Vascular Abnormalities: a Two-dimensional Phase-Contrast MRI Study

PURPOSE: To evaluate changes in total cerebral blood flow (tCBF) with aging, parenchymal volume changes and vascular abnormalities, using 2 dimensional (D) phase-contrast magnetic resonance imaging (PC MRI). MATERIALS AND METHODS: Routine brain MRI including T2 weighted image, time-of-flight (TOF) MR Angiography (MRA) and 2D PC MRI were performed in 73 individuals, including 12 volunteers. Normal subjects (12 volunteers, and 21 individuals with normal MRI and normal MRA) were classified into groups according to age (18 -29, 30 -49 and 50 -66 years). For the group with abnormalities in brain MRIs, cerebral parenchymal volume changes were scored according to the T2 weighted images, and atherosclerotic changes were scored according to the MRA findings. Abnormal groups were classified into 4 groups: (i) mild reduction in volume, (ii) marked reduction in volume by parenchymal volume and atherosclerotic changes, and (iii) increased volume and (iv) Moya-moya disease. Volumetric flow was measured at the internal carotid artery (ICA) and vertebral artery bilaterally using the velocity-flow diagrams from PC MRI, and combined 4 vessel flows and tCBF were compared among all the groups. RESULTS: The age-specific distribution of tCBFs in normal subjects were as follows: 12.0 +/- 2.1 ml/sec in 18 -29 years group, 11.8 +/- 1.9 ml/sec in 30 -49 years group, 10.9 +/-2.2 ml/sec in 50 -66 years group. The distribution of tCBFs in the different subsets of the abnormal population were as follows: 9.5 +/-2.5 ml/sec in the group with mild reduction in volume, 7.6 +/-2.0 ml/sec in the group with marked reduction in volume, and 7.3 +/-1.2 ml/sec and 7.0 +/- 1.1 ml/sec in the increased parenchymal volume and Moya-moya disease groups respectively. CONCLUSION: Total cerebral blood flow decreases with increasing age with a concomitant reduction in parenchymal volumes and increasing atherosclerotic changes. It is also reduced in the presence of increased parenchymal volume and Moya-moya disease. 2D PC MRI can be used as a tool to evaluate tCBF with aging and in the presence of various conditions that can affect parenchymal volume and cerebral vasculature.