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BRAINLIFE NEWSLETTER
Volume 3, Supplement 20 - 25 December 2004

Volume 3
Archive


1: J Pediatr Hematol Oncol. 2004 Nov;26(11):756-60.
 
Carboplatinum and vincristine chemotherapy for central nervous system gliofibroma: case report and review of the literature.

Suarez CR, Raj AB, Bertolone SJ, Coventry S.

Department of Pediatrics, Section of Hematology Oncology, University of Louisville School of Medicine, Louisville, Kentucky, USA. csuarez@psm.edu

Gliofibromas are bimorphic tumors of the central nervous system. Although they are composed of astrocytic and fibroblastic elements, their histogenesis is not clear. An attempt has been made to classify the tumors as low- or high-grade based on morphology and proliferative labeling index, but the clinical behavior and the optimal therapeutic strategies remain unknown. Although they are considered benign, the authors' review shows a 23% mortality rate. The authors report the successful use of carboplatinum and vincristine as treatment of this disease.

Publication Types:
  • Case Reports
  • Review
  • Review, Tutorial

PMID: 15543013 [PubMed - indexed for MEDLINE]

 
2: J Clin Oncol. 2004 Nov 15;22(22):4551-60.
 
White matter lesions detected by magnetic resonance imaging after radiotherapy and high-dose chemotherapy in children with medulloblastoma or primitive neuroectodermal tumor.

Fouladi M, Chintagumpala M, Laningham FH, Ashley D, Kellie SJ, Langston JW, McCluggage CW, Woo S, Kocak M, Krull K, Kun LE, Mulhern RK, Gajjar A.

Department of Hematology-Oncology, St Jude Children's Research Hospital, Memphis, TN 38105-2794, USA. maryam.fouladi@stjude.org

PURPOSE: White matter lesions (WMLs) have been described as a delayed effect of cranial irradiation in children with brain tumors, or a transient subacute effect characterized by an intralesional or perilesional reaction. We report the occurrence of subacute WMLs detected by magnetic resonance imaging (MRI) in children treated for medulloblastoma or primitive neuroectodermal tumor (PNET) and document the associated clinical, radiologic, and neurocognitive findings. PATIENTS AND METHODS: Among 134 patients with medulloblastoma or supratentorial PNET treated prospectively with risk-adjusted craniospinal irradiation and conformal boost to the tumor bed, followed by four high-dose chemotherapy (HDC) cycles with stem-cell rescue, 22 developed WMLs on T1-weighted imaging with and without contrast and/or T2-weighted imaging on MRI. Patients had > or = 12 months of follow-up. Neurocognitive assessments included intelligence quotient (IQ) tests and tests of academic achievement. RESULTS: Twenty-two patients developed WMLs at a median of 7.8 months after starting therapy (range, 1.9 to 13.0 months). Lesions were predominantly in the pons (n = 8) and cerebellum (n = 6). Sixteen patients (73%) had WML resolution at a median of 6.2 months (range, 1.68 to 23.5 months) after onset; two patients developed necrosis and atrophy. Three developed persistent neurologic deficits. Cumulative incidence of WMLs at 1 year was 15% +/- 3%. Patients with WMLs had a significant decline in estimated IQ (-2.5 per year; P = .03) and math (-4.5 per year; P = .003) scores. CONCLUSION: WMLs in medulloblastoma or PNET patients treated with conformal radiotherapy and HDC are typically transient and asymptomatic, and may mimic early tumor recurrence. A minority of patients with WMLs develop permanent neurologic deficits and imaging changes. Overall, the presence of WMLs is associated with greater neurocognitive decline.

Publication Types:
  • Clinical Trial
  • Multicenter Study

PMID: 15542806 [PubMed - indexed for MEDLINE]

 
3: Arthritis Rheum. 2004 Nov;50(11):3616-8.
 
First reported pediatric case of systemic lupus erythematosus associated with prolactinoma.

Reuman PD.

Pediatric Rheumatology and Clinical Research, Children's Hospital Medical Center, One Perkins Square, Akron, OH 44308, USA. PReuman@CHMCA.org

There is much interest in the possibility that prolactin influences disease activity in systemic lupus erythematosus (SLE). We present the first reported pediatric case of prolactinoma associated with SLE, in a 13-year-old white female. The diagnosis of SLE was based on the presence of arthritis, antinuclear antibodies, and double-stranded DNA, and a chest radiograph showing pleural fluid. The diagnosis of pituitary prolactinoma was based on the histologic features and the presence of amenorrhea, galactorrhea, and an elevated serum prolactin level. Neurosurgical resection and medical therapy with bromocriptine mesylate were independently associated with decreased prolactin levels, loss of arthritis, and reduced levels of inflammatory mediators.

Publication Types:
  • Case Reports

PMID: 15529390 [PubMed - indexed for MEDLINE]

 
4: J Neurooncol. 2004 Aug-Sep;69(1-3):139-50.

Pediatric surgical neuro-oncology: current best care practices and strategies.

Rutka JT, Kuo JS.

The Arthur and Sonia Labatt Brain Tumour Research Centre and Division of Neurosurgery, The Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada. james.rutka@sickkids.ca

Significant advances have been made in the diagnosis and treatment of childhood brain tumors. Gross total surgical resection combined with appropriate adjuvant therapies can achieve a high rate of disease control for low grade gliomas, ependymomas and medulloblastomas. High grade gliomas, tumors involving the optic apparatus or diencepahalic structures, diffuse brainstem lesions, and recurrent or metastatic disease still pose considerable therapeutic challenges. We review the current treatment strategies of the three most common types of pediatric brain tumors: gliomas, medulloblastomas and ependymomas, and discuss current and future diagnostic and therapeutic modalities.

Publication Types:
  • Review
  • Review, Tutorial

PMID: 15527086 [PubMed - indexed for MEDLINE]

 
5: J Neurosurg. 2004 Oct;101(4):607-12.

Postoperative evaluation of microsurgical resection for cavernous malformations of the brainstem.

Kikuta K, Nozaki K, Takahashi JA, Miyamoto S, Kikuchi H, Hashimoto N.

Department of Neurosurgery, Kyoto University Graduate School of Medicine, Kyoto, Japan. kikuta@kuhp.kyoto-u.ac.jp

OBJECT: The aim of this study was to propose criteria to determine whether complete resection of cavernous malformations in the brainstem had been achieved. METHODS: The authors retrospectively analyzed data in 10 patients harboring a single cavernous malformation who had presented with hemorrhagic symptoms and had been followed up for longer than 2 years postsurgery. The study population consisted of five male and five female patients ranging in age from 13 to 57 years (mean 36.8 years). When preoperative magnetic resonance (MR) images demonstrated the lesion as a homogeneous hyperintense mass, the surgery was defined as complete or incomplete based on intraoperative findings. When preoperative MR images revealed other findings, complete resection was determined according to whether postoperative MR imaging results demonstrated lesions distinct from the peripheral hemosiderin rim. Among the 13 operations in this series, nine resulted in complete resection and were associated with no postoperative clinical relapse of hemorrhage, whereas four operations resulted in incomplete resection and were correlated with postoperative recurrent hemorrhage. The seven patients in whom the outcome of the initial operation was complete demonstrated good neurological recovery in the long-term follow-up period, whereas the three patients in whom the outcome of the initial surgery was judged to be incomplete showed inadequate neurological recovery due to recurrent hemorrhage. CONCLUSIONS: The criteria proposed in this study to evaluate surgical treatment may be a reliable means of predicting the recurrence of hemorrhage postoperatively.

PMID: 15481714 [PubMed - indexed for MEDLINE]

 
6: J Clin Endocrinol Metab. 2004 Oct;89(10):4999-5002.
 
Adult heights attained by children with hypothalamic/chiasmatic glioma treated with growth hormone.

Kim RJ, Janss A, Shanis D, Homan S, Moshang T Jr.

Division of Endocrinology, Department of Pediatrics, University of Pennsylvania School of Medicine and Children's Hospital of Philadelphia, Philadelphia, Pennsylvania 19104, USA.

Hypothalamic/chiasmatic gliomas (H/CG) in children are commonly accompanied by endocrine dysfunction due to mass effects of the tumor itself or as a consequence of tumor therapy, with GH deficiency (GHD) being the most common disorder. We report the height outcomes of GH-treated H/CG patients with GHD. We reviewed the records of 14 GHD patients with H/CG who were treated with human GH. A comparison group of non-GH-treated H/CG patients was also identified. Heights were expressed as sd scores (SDS). For GH-treated patients, the mean initial height was -0.7 +/- 0.3 (+/-se). Their mean final height was -0.3 +/- 0.3. The mean change in height SDS for the GH-treated group was +0.4. The mean initial and final height SDS for the non-GHD patients were 0.6 (se = 0.4) and 0.0 (se = 0.4), respectively. The mean change in height SDS was -0.6. The GHD patients had significantly lower initial height SDS compared with the non-GHD patients (P = 0.01) and had a significantly greater change in their height SDS (P = 0.04). GH treatment for H/CG patients restores much of their growth potential and improves adult height to within normal limits.

PMID: 15472197 [PubMed - indexed for MEDLINE]
http://jcem.endojournals.org/cgi/reprint/89/10/4999

 
7: J Clin Pathol. 2004 Oct;57(10):1111-3.
 
Expression of thyroid transcription factor 1 in primary brain tumours.

Zamecnik J, Chanova M, Kodet R.

Department of Pathology and Molecular Medicine, Charles University, Second Medical Faculty, 150 06 Prague, Czech Republic. josef.zamecnik@lfmotol.cuni.cz

BACKGROUND: Thyroid transcription factor 1 (TTF-1) is expressed in a proportion of carcinomas derived from follicular thyroid cells and respiratory epithelium. Immunohistochemical detection of this protein was shown previously to be a helpful aid in tumour diagnosis, specifically in deciding whether a tumour is primary to the lung/thyroid gland or metastatic. Recently, TTF-1 expression was also observed in certain areas of postnatal brain. AIM/METHOD: To investigate the expression of TTF-1 protein in a spectrum of 73 primary brain tumours including astrocytomas, glioblastomas, ependymomas, oligodendrogliomas, medulloblastomas, and gangliogliomas of different sites. RESULTS: All the tumours were negative for TTF-1 except for two ependymomas of the third ventricle. CONCLUSIONS: The expression of TTF-1 in brain tumours appears to be site specific rather than associated with tumour dedifferentiation. The presented expression of TTF-1 protein in certain primary brain tumours should be taken into consideration when interpreting the immunohistochemical staining of brain tumours of uncertain primary site.

PMID: 15452173 [PubMed - indexed for MEDLINE]

 
8: Cancer. 2004 Nov 1;101(9):2116-25.
 
Parent proxy-reported health-related quality of life and fatigue in pediatric patients diagnosed with brain tumors and acute lymphoblastic leukemia.

Meeske K, Katz ER, Palmer SN, Burwinkle T, Varni JW.

Childrens Center for Cancer and Blood Diseases, Childrens Hospital Los Angeles, Los Angeles, CA 90027, USA. kmeeske@chla.usc.edu

BACKGROUND: Pediatric patients with brain tumors (BT) are often excluded from health-related quality of life (HRQOL) studies even though they experience more severe disease and treatment-related sequelae than children with other types of cancer. Parent proxy assessments of HRQOL allow for greater inclusion of children who are developmentally immature, physically ill, or cognitively impaired. METHODS: Parents of children ages 2-18 years who were diagnosed at Childrens Hospital Los Angeles and Children's Hospital San Diego with BT (n = 86) or acute lymphoblastic leukemia (ALL; n = 170) evaluated their children's HRQOL over the previous week using the parent-proxy versions of the Pediatric Quality of Life Inventory (PedsQL) 4.0 Generic Core scales, the PedsQL 3.0 Acute Cancer Module, and the PedsQL Multidimensional Fatigue scales. Multiple regression analyses were used to determine the independent effect of the child's diagnosis on HRQOL. Separate analyses were conducted for patients receiving treatment, patients who had not received treatment for < 12 months, and patients who had not received treatment for > or = 12 months. RESULTS: Patients with BT exhibited more problems than patients with ALL in the physical, social, psychosocial, school, cognitive, and fatigue domains of HRQOL. The Core Physical Health, Core Psychosocial Health, and Fatigue Total scores for patients with BT demonstrated peak improvements for children who had not received treatment for < 12 months and sharp declines for children who had not received treatment for > or = 12 months. The Core Physical Health and Fatigue Total scores for patients with ALL were highest (better HRQOL) for those who had not received treatment for > or = 12 months. CONCLUSIONS: Pediatric patients and survivors of BT experienced more fatigue and HRQOL problems than patients with ALL, and HRQOL differed by treatment status.

PMID: 15389475 [PubMed - indexed for MEDLINE]

 
9: Oncogene. 2004 Sep 20;23(43):7267-73.
 
Cancer stem cells in nervous system tumors.

Singh SK, Clarke ID, Hide T, Dirks PB.

The Arthur and Sonia Labatt Brain Tumor Research Centre, The Hospital for Sick Children, Toronto, Canada.

Most current research on human brain tumors is focused on the molecular and cellular analysis of the bulk tumor mass. However, evidence in leukemia and more recently in solid tumors such as breast cancer suggests that the tumor cell population is heterogeneous with respect to proliferation and differentiation. Recently, several groups have described the existence of a cancer stem cell population in human brain tumors of different phenotypes from both children and adults. The finding of brain tumor stem cells (BTSCs) has been made by applying the principles for cell culture and analysis of normal neural stem cells (NSCs) to brain tumor cell populations and by identification of cell surface markers that allow for isolation of distinct tumor cell populations that can then be studied in vitro and in vivo. A population of brain tumor cells can be enriched for BTSCs by cell sorting of dissociated suspensions of tumor cells for the NSC marker CD133. These CD133+ cells, which also expressed the NSC marker nestin, but not differentiated neural lineage markers, represent a minority fraction of the entire brain tumor cell population, and exclusively generate clonal tumor spheres in suspension culture and exhibit increased self-renewal capacity. BTSCs can be induced to differentiate in vitro into tumor cells that phenotypically resembled the tumor from the patient. Here, we discuss the evidence for and implications of the discovery of a cancer stem cell in human brain tumors. The identification of a BTSC provides a powerful tool to investigate the tumorigenic process in the central nervous system and to develop therapies targeted to the BTSC. Specific genetic and molecular analyses of the BTSC will further our understanding of the mechanisms of brain tumor growth, reinforcing parallels between normal neurogenesis and brain tumorigenesis.

Publication Types:
  • Review
  • Review, Tutorial

PMID: 15378086 [PubMed - indexed for MEDLINE]

 
10: Cancer. 2004 Sep 15;101(6):1445-54.
 
Medullomyoblastoma: a radiographic and clinicopathologic analysis of six cases and review of the literature.

Helton KJ, Fouladi M, Boop FA, Perry A, Dalton J, Kun L, Fuller C.

Department of Radiological Sciences, St. Jude Children's Research Hospital, Memphis, TN 38105-2794, USA. kathleen.helton@stjude.org

BACKGROUND: Medullomyoblastoma (MMB) is a rare cerebellar embryonal neoplasm that occurs almost exclusively in children. It is biphasic by microscopy, containing myoblastic and primitive neuroectodermal components. METHODS: The authors conducted a retrospective review of the radiographic and pathologic characteristics, treatment, and clinical outcomes of six children with MMB who were treated at St. Jude Children's Research Hospital (Memphis, TN) between 1984 and 2003. Fluorescence in situ hybridization (FISH) data were available for four children. A literature review also was conducted and focused on imaging and pathologic findings. RESULTS: The median age at diagnosis was 4.5 years (range, 0.83-7.5 years). Radiographically, all tumors were cerebellar and exhibited variable enhancement, and 50% of tumors had necrotic foci. Three tumors contained discrete, magnetic resonance imaging (MRI) T2-weighted-hypointense/computed tomography (CT)-hyperdense enhancing regions and separate hyperintense/hypodense nonenhancing regions, which correlated microscopically with geographic islands of primitive neuroectodermal and rhabdomyoblastic cells. Large cell/anaplastic (five tumors), nodular/desmoplastic (two tumors), and classic (two tumors) medulloblastoma histologies were encountered either alone (five tumors) or in combination with each other (two tumors). All 4 tumors that were tested exhibited alterations in chromosome 17 or c-myc amplification. All patients underwent macroscopic total resection and subsequently received chemotherapy and craniospinal (five patients) or local conformal (one patient) radiotherapy. At a median follow-up of 92 months (range, 23-187 months), 3 patients remain alive with no evidence of disease, 2 patients have died of disease, and 1 patient has died of secondary acute lymphocytic leukemia. CONCLUSIONS: The results of the current study demonstrated the frequent correlation of biphasic nodularity (as determined by MRI or CT) with discrete rhabdomyoblastic and primitive neuroectodermal islands (as revealed by microscopy) in MMB. These results also support the view that MMB and medulloblastoma may have common tumorigenic origins, given their similar histologic and molecular features. Copyright 2004 American Cancer Society.

Publication Types:
  • Review

PMID: 15368333 [PubMed - indexed for MEDLINE]

 
11: J Neurosurg. 2004 Sep;101(3):536-40.

Endotracheal tube electrodes to map and monitor activities of the vagus nerve intraoperatively. Technical note.

Mikuni N, Satow T, Taki J, Nishida N, Enatsu R, Hashimoto N.

Department of Neurosurgery, Kyoto University Graduate School of Medicine, Kyoto, Japan. mikunin@kuhp.kyoto-u.ac.jp

Difficulty swallowing due to damage of the vagus nerve is one of the most devastating complications of surgery in and around the medulla oblongata; therefore, intraoperative anatomical and functional evaluation of this nerve is crucial. The authors applied endotracheal tube surface electrodes to record electromyography (EMG) activity from vocal cords innervated by the vagus nerve. The vagal nucleus or rootlet was electrically stimulated during surgery and vocalis muscle EMG activities were displayed by auditory and visual signals. This technique was used successfully to identify the vagus motor nerve and evaluate its integrity during surgery. The advantages of this method compared with the use of needle electrodes include safe simple electrode placement and stable recording during surgery. In cases involving a pontine cavernoma pressing the nucleus or a jugular foramen tumor encircling the rootlet, this method would be particularly valuable. Additional studies with a larger number of patients are needed to estimate the significance of this method as a means of functional monitoring to predict clinical function.

PMID: 15352615 [PubMed - indexed for MEDLINE]

 
12: Inf Process Med Imaging. 2003 Jul;18:270-81.

Knowledge-driven automated extraction of the human cerebral ventricular system from MR images.

Xia Y, Hu Q, Aziz A, Nowinski WL.

Biomedical Imaging Lab, Institute for Infocomm Research, 21 Heng Mui Keng Terrace, Singapore 119613. yanxia@i2r.a-star.edu.sg

This work presents an efficient and automated method to extract the human cerebral ventricular system from MRI driven by anatomic knowledge. The ventricular system is divided into six three-dimensional regions; six ROIs are defined based on the anatomy and literature studies regarding variability of the cerebral ventricular system. The distribution histogram of radiological properties is calculated in each ROI, and the intensity thresholds for extracting each region are automatically determined. Intensity inhomogeneities are accounted for by adjusting intensity threshold to match local situation. The extracting method is based on region-growing and anatomical knowledge, and is designed to include all ventricular parts, even if they appear unconnected on the image. The ventricle extraction method was implemented on the Window platform using C++, and was validated qualitatively on 30 MRI studies with variable parameters.

Publication Types:
  • Clinical Trial
  • Controlled Clinical Trial
  • Validation Studies

PMID: 15344464 [PubMed - indexed for MEDLINE]

 
13: Cancer Chemother Biol Response Modif. 2003;21:655-81.

Brain tumors.

Pueschel JK, Ashby LS, Shapiro WR.

Barrow Neurological Institute, Department of Neurology, Phoenix, Arizona 85013, USA.

Publication Types:
  • Review
  • Review, Tutorial

PMID: 15338768 [PubMed - indexed for MEDLINE]

 
14: Int J Radiat Oncol Biol Phys. 2004 Sep 1;60(1):204-13.
 
Patterns of failure in supratentorial primitive neuroectodermal tumors treated in Children's Cancer Group Study 921, a phase III combined modality study.

Hong TS, Mehta MP, Boyett JM, Donahue B, Rorke LB, Yao MS, Zeltzer PM.

Department of Human Oncology, University of Wisconsin School of Medicine, Madison, WI, USA.

PURPOSE: To analyze the patterns of failure in patients with supratentorial primitive neuroectodermal tumors (ST-PNETs) treated with combined modality therapy in a large, randomized, multi-institutional study. METHODS AND MATERIALS: A total of 44 prospectively staged patients with ST-PNET confirmed by central pathology review were treated in the Children's Cancer Group Study 921, which compared two chemoradiotherapy regimens. The patterns of initial sites of failure were analyzed. These were compared with the failure patterns of 188 children with posterior fossa (PF) PNETs treated in the same protocol. RESULTS: The major determinant for progression-free survival was the initial metastatic stage. The 3-year progression-free survival for M0 patients was 53% +/- 8.5% compared with 14% +/- 9.4% for M+ patients. The cumulative 5-year relapse incidence was 71.4% +/- 21% for M+ patients compared with 47.5% +/- 8.6% for M0 patients. The overall failure rate for both M0 and M+ ST-PNETs was greater than that for PF-PNETs (47.5% +/- 8.6% vs. 29.3% +/- 4.7% for M0 and 71.4% +/- 21% vs. 48.4% +/- 5.5% for M+). Failure at the primary site, either as the sole site or as a component of initial failure, was also seen more frequently in ST-PNETs than in PF-PNETs. For M0 patients, the 5-year local failure rate as a component of initial failure was 42.0% +/- 8.5% for ST-PNETs compared with 17.7% +/- 3.9% for PF-PNETs. For patients with primary tumors either in the ST or PF, the 5-year spinal axis failure rate as a component of initial failure was not significantly different statistically when compared by M stage. For M+ patients, the 5-year spinal axis failure rate as a component of initial failure was 42.9% +/- 22.8% for ST-PNETs and 34.6% +/- 5.2% for PF-PNETs. CONCLUSION: Despite aggressive combined modality therapy, ST-PNETs had high rates of failure, with M+ patients faring especially poorly. Both local and spinal failure rates remained high, indicating the need to maximize both local and regional/systemic therapies. Overall, these patients fared worse than those with high-risk PF-PNETs in terms of progression-free survival and failure rates.

Publication Types:
  • Clinical Trial
  • Clinical Trial, Phase III
  • Multicenter Study
  • Randomized Controlled Trial

PMID: 15337557 [PubMed - indexed for MEDLINE]

 
15: Cancer. 2004 Sep 1;101(5):1036-42.
 
Analysis of interleukin-13 receptor alpha2 expression in human pediatric brain tumors.

Kawakami M, Kawakami K, Takahashi S, Abe M, Puri RK.

Laboratory of Molecular Tumor Biology, Division of Cellular and Gene Therapies, Center for Biologics Evaluation and Research, Food and Drug Administration, Bethesda, MD 20892, USA.

BACKGROUND: Compared with normal brain tissue cells, human malignant glioma cells express higher levels of interleukin-13 receptor (IL-13R). However, whether this receptor is expressed in situ has not been carefully examined. With IL-13R-targeted cytotoxin (IL13-PE38QQR, comprising IL-13 and a mutated form of Pseudomonas exotoxin [PE]) being tested in three Phase I/II clinical trials for the treatment of adult human glioma, and with pediatric studies being planned, the authors set out to analyze pediatric brain tumor tissue specimens for the expression of IL-13R. METHODS: Using in situ hybridization and immunohistochemical staining, the authors examined 58 pediatric brain tumor specimens for expression of the predominant IL-13 binding and internalizing protein (IL-13Ralpha2) chain at the mRNA and protein levels. RESULTS: Overall, approximately 83% of pediatric brain tumor samples expressed IL-13Ralpha2. One hundred percent (11 of 11) high-grade astrocytoma, 79% (26 of 33) low-grade astrocytoma, 67% (4 of 6) medulloblastoma, and 67% (2 of 3) ependymoma samples were positive for IL-13Ralpha2. Among IL-13Ralpha2-positive samples, 88% (42 of 48 samples) had positive expression in > or = 50% of all tumor fields. The results obtained using both assays were consistent with each other. CONCLUSIONS: The current study established that pediatric brain tumor specimens expressed the IL-13Ralpha2 chain. Because the IL-13Ralpha2 chain is a major binding component of the IL-13R complex, these results suggest that the targeting of IL-13R may represent a useful approach for the treatment of pediatric brain tumors. Copyright 2004 American Cancer Society.

PMID: 15329913 [PubMed - indexed for MEDLINE]

 
16: Br J Neurosurg. 2004 Jun;18(3):310-3.

Primary midbrain germinoma.

Ben Amor S, Siddiqui K, Baessa S.

Department of Neurosciences, King Faisal Specialist Hospital & RC, Jeddah, Kingdom of Saudi Arabia. benamor@healthgulf.com

Intracranial germinomas arising primarily in the midbrain are extremely rare and only one case has been reported in the literature. A 15-year-old boy presented with headache, diplopia, unsteadiness and personality changes. Brain MRI showed a heterogeneous lesion in the midbrain. The pineal body region was free. The preoperative diagnosis included brain-stem glioma, metastasis and lymphoma. Stereotactic biopsy was permitted in order to take a specimen and the diagnosis of germinoma was established. The patient responded well to chemotherapy and radiotherapy. Germinoma should be included in the differential diagnosis of midbrain lesions. Preoperative diagnosis is difficult and biopsy is still needed for such lesions.

Publication Types:
  • Case Reports
  • Review
  • Review of Reported Cases

PMID: 15327241 [PubMed - indexed for MEDLINE]

 
17: Br J Neurosurg. 2004 Jun;18(3):284-9.

Primary cerebellar germinomas of the posterior fossa.

Maiuri F, Cappabianca P, Del Basso De Caro M, Esposito F, de Divitiis E.

Department of Neurological Sciences, Section of Neurosurgery, School of Medicine, University Federico II, Naples, Italy. fcmaiuri@unina.it

Primary cerebellar germinomas, in the absence of germ-cell tumours outside the nervous system or elsewhere in the cranial cavity and CSF pathways, are exceptional; only two previous cases have been reported in the literature. Two personal observations are described from our 20-year records of intra-axial posterior fossa tumours. The patients were a 32-year-old man and a 17-year-old woman with a clinical history of posterior fossa tumour, studied by computed tomography. The first patient with slight cerebellar signs had a small right hemispheric cerebellar tumour, and the other had a left cerebellar mass with hydrocephalus and progressive intracranial hypertension. Both were treated by tumour removal and irradiation to the whole posterior fossa. The survival times were 58 and 49 months, respectively. The diagnosis of primary cerebellar germinoma cannot be suspected before pathological confirmation. The clinical, neuroradiological and surgical findings are non-specific and quite similar to those of other malignant cerebellar tumours, such as anaplastic gliomas or metastases. Surgery and radiotherapy ensure adequate tumour control in the early stages; cases of recurrence or disseminated disease may be treated by irradiation and chemotherapy.

Publication Types:
  • Case Reports
  • Review
  • Review of Reported Cases

PMID: 15327234 [PubMed - indexed for MEDLINE]

 
18: J Neurol Neurosurg Psychiatry. 2004 Sep;75(9):1309-13.
 
Magnetoencephalography (MEG) predicts focal epileptogenicity in cavernomas.

Stefan H, Scheler G, Hummel C, Walter J, Romstock J, Buchfelder M, Blumcke I.

Department of Neurology, Epilepsy and Neuro-Center, University of Erlangen-Nurnberg, Germany. hermann.stefan@neuro.imed.uni-erlangen.de

OBJECTIVE: The aim of this study was to identify the irritative epileptic zone in patients with cavernomas by means of magnetoencephalography (MEG). METHOD: Among 82 patients operated for epilepsy, whose presurgical evaluation had included MEG, histological assessment of the tissue removed had confirmed cavernomas in eight. These eight patients had epilepsy since 18.6 (SD 12.7) years on average. The monitoring lasted about 2.1 (SD 1.3) hours and a median 20.9 (SD 14.3) spikes per hour were recorded. Spontaneous brain activity was recorded by means of a 74 channel dual unit MEG system (Magnes II, 4-D Neuroimaging) with simultaneous EEG recording (31 scalp electrodes). Spike analysis was performed using different source (moving dipole, current density reconstruction) and head models (spherical shells, BEM). Co-registration of neurophysiological and imaging data (MRI) was based upon anatomical landmarks. RESULTS: In 6/8 patients co-localisation from the cavernoma and epileptic zone was found. In two patients the focus was localised in the parieto-occipital lobe, in three patients in the frontal lobe and in three patients in the temporal lobe. In one case of temporal and one case of frontal lobe focus localisation there was no spatial relationship to the cavernoma. CONCLUSION: In cases of focal seizures due to a single cavernoma, MEG may precisely delineate the epileptogenic tissue bordering the lesion. In patients with multiple cavernomas or dual pathology, MSI may reveal the complexity of the case, and contribute to the decision about further invasive diagnostics and more sophisticated therapeutic measures. MEG is a promising method for prediction of the epileptic zone in cavernoma related epilepsies, and thus it can contribute to decision making about and planning of epilepsy surgery.

Publication Types:
  • Clinical Trial

PMID: 15314122 [PubMed - indexed for MEDLINE]

 
19: Toxicol Appl Pharmacol. 2004 Sep 1;199(2):118-31.
 
Epidemiology of brain tumors in childhood--a review.

Baldwin RT, Preston-Martin S.

Department of Family Medicine, Division of Preventive Medicine, David Geffen School of Medicine, University of California-Los Angeles, Los Angeles, CA 90095, USA.

Malignant brain tumors are the leading cause of cancer death among children and the second most common type of pediatric cancer. Despite several decades of epidemiologic investigation, the etiology of childhood brain tumors (CBT) is still largely unknown. A few genetic syndromes and ionizing radiation are established risk factors. Many environmental exposures and infectious agents have been suspected of playing a role in the development of CBT. This review, based on a search of the medical literature through August 2003, summarizes the epidemiologic evidence to date. The types of exposures discussed include ionizing radiation, N-nitroso compounds (NOC), pesticides, tobacco smoke, electromagnetic frequencies (EMF), infectious agents, medications, and parental occupational exposures. We have chosen to focus on perinatal exposures and review some of the recent evidence indicating that such exposures may play a significant role in the causation of CBT. The scientific community is rapidly learning more about the molecular mechanisms by which carcinogenesis occurs and how the brain develops. We believe that advances in genetic and molecular biologic technology, including improved histologic subtyping of tumors, will be of huge importance in the future of epidemiologic research and will lead to a more comprehensive understanding of CBT etiology. We discuss some of the early findings using these technologies.

Publication Types:
  • Review

PMID: 15313584 [PubMed - indexed for MEDLINE]

 
20: Pediatr Nurs. 2004 May-Jun;30(3):238-41.

The story of Margaret and her family: forced choices, obligations, and hope.

Carnevale FA.

McGill University, Montreal, Quebec, Canada.

Publication Types:
  • Case Reports

PMID: 15311651 [PubMed - indexed for MEDLINE]
 

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