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BRAINLIFE NEWSLETTER
Volume 4, Number 14 - 22 March 2005

Volume 4
Archive


1: Ann Neurol. 2005 Jan;57(1):136-9.
 
Capillary physiology and drug delivery in central nervous system lymphomas.

Warnke PC, Timmer J, Ostertag CB, Kopitzki K.

Department of Neurological Science, University of Liverpool, Clinical Sciences Centre for Research and Education, Lower Lane, Fazakerley, Liverpool L9 7LJ, Merseyside, United Kingdom. warnke@liverpool.ac.uk

To evaluate whether the chemosensitivity of primary central nervous system lymphomas to water-soluble drugs could result from improved drug delivery, we quantitatively assessed pharmacokinetic factors in seven patients. The capillary permeability surface product was found to be significantly increased in central nervous system lymphomas compared with glioblastoma multiforme, medulloblastomas, and metastases. Tumoral blood flow was significantly greater than in normal white matter. Our results suggest favorable pharmacokinetics to water- and lipid-soluble drugs in primary central nervous system lymphomas.

PMID: 15622544 [PubMed - indexed for MEDLINE]


 
2: Arch Pathol Lab Med. 2004 Oct;128(10):1161-4.
 
Microdissection genotyping of mixed glial and primitive neuroectodermal central nervous system neoplasm.

Mohan D, Rao GR, Swalsky PA, Bakker A, Martinez AJ, Finkelstein SD.

Department of Pathology, University of Pittsburgh Medical Center, Pittsburgh, Pa, USA. Deepak.Mohan@cshs.org

A 22-year-old man with previous radiation treatment for childhood astrocytoma underwent resection of a right parietooccipital lesion. Histopathology revealed a malignant neoplasm with areas of astrocytic and primitive neuroectodermal components. To resolve the relationship and cellular origin, representative tissue was microdissected from several targets, obtaining a balanced mixture of each element. Nonneoplastic brain parenchyma was separately microdissected to determine polymorphic marker informativeness and to serve as an internal negative control. Despite the relatively small quantity of tissue removed for each microdissection target, sufficient material was available for reliable, balanced, polymerase chain reaction-format genotyping encompassing a panel of tumor suppressor genes and genetic loci associated with these forms of neoplasia. The findings revealed distinct discordant genotypic profiles for each of the neoplastic components. The efficacy of the approach used for molecular analysis of this complex neoplasm and the implication of the genotypic findings are discussed.

Publication Types:
  • Case Reports

PMID: 15387704 [PubMed - indexed for MEDLINE]


 
3: Cancer. 2005 Mar 15; [Epub ahead of print]
 
Medulloblastoma in the second decade of life: A specific group with respect to toxicity and management.

Tabori U, Sung L, Hukin J, Laperriere N, Crooks B, Carret AS, Silva M, Odame I, Mpofu C, Strother D, Wilson B, Samson Y, Bouffet E.

Pediatric Brain Tumor Program, The Hospital for Sick Children, Toronto, Ontario, Canada.

BACKGROUND: Most reported data of chemoradiotherapy protocols for the treatment of medulloblastoma describe children who were treated in the first decade of life. To consider the feasibility of this approach in adolescents, the authors studied their clinical course with specific emphasis on toxicity, tolerability, and prognosis. METHODS: In this retrospective study, the authors examined the toxicity profiles and outcomes of children age 10-20 years with medulloblastoma who were treated at centers throughout Canada between 1986 and 2003. Detailed toxicity data from 2 chemotherapy protocols were collected for teenagers and were compared with data from a group of control patients age 5-10 years. RESULTS: In total, 72 teenagers were analyzed. Grade >/= 2 ototoxicity and neurotoxicity occurred in 45% and 71% of chemotherapy-treated patients, respectively. Grade 3-4 hematotoxicty occurred in 95% of patients. Toxicity resulted in delay of treatment for 73% of patients and dose modification in 75% of patients, including protocol discontinuation in 25% of patients. Weight loss > 10% was encountered in 73% of patients and required intervention in 45% of patients. Teenagers had significantly more hematotoxicity and neurotoxicity compared with controls on both chemotherapeutic protocols. Ototoxicity was similar in both age groups. Toxicity resulted in significantly more treatment delays and dose modifications in teenager patients compared with controls. The 5-year overall and event-free survival rates (+/- standard deviation) were 78% +/- 6% and 70% +/- 6%, respectively. The mean time (+/- standard deviation) to disease recurrence was 3.2 +/- 2.2 years. CONCLUSIONS: The increased toxicity rate and high incidence of treatment modifications in this study suggested that current pediatric protocols may require modifications for teenagers with medulloblastoma. The results highlighted several issues that should be addressed in future prospective trials. Cancer 2005. (c) 2005 American Cancer Society.

PMID: 15770645 [PubMed - as supplied by publisher]


 
4: Cancer Res. 2005 Feb 1;65(3):787-96.
 
Suppression of melanotroph carcinogenesis leads to accelerated progression of pituitary anterior lobe tumors and medullary thyroid carcinomas in Rb+/- mice.

Zhou Z, Flesken-Nikitin A, Levine CG, Shmidt EN, Eng JP, Nikitina EY, Spencer DM, Nikitin AY.

Department of Biomedical Sciences, Cornell University, Ithaca, NY 14853-6401, USA.

Mice with a single copy of the retinoblastoma gene (Rb(+/-)) develop a syndrome of multiple neuroendocrine neoplasia. They usually succumb to fast-growing, Rb-deficient melanotroph tumors of the pituitary intermediate lobe, which are extremely rare in humans. Thus, full assessment of Rb role in other, more relevant to human pathology, neoplasms is complicated. To prevent melanotroph neoplasia while preserving spontaneous carcinogenesis in other types of cells, we have prepared transgenic mice in which 770-bp fragment of pro-opiomelanocortin promoter directs expression of the human RB gene to melanotrophs (TgPOMC-RB). In three independent lines, transgenic mice crossed to Rb(+/-) background are devoid of melanotroph tumors but develop the usual spectrum of other neoplasms. Interestingly, abrogation of melanotroph carcinogenesis results in accelerated progression of pituitary anterior lobe tumors and medullary thyroid carcinomas. A combination of immunologic tests, cell culture studies, and tumorigenicity assays indicates that alpha-melanocyte-stimulating hormone, which is overproduced by melanotroph tumors, attenuates neoplastic progression by decreasing cell proliferation and inducing apoptosis. Taken together, we show that cell lineage-specific complementation of Rb function can be successfully used for refining available models of stochastic carcinogenesis and identify alpha-melanocyte-stimulating hormone as a potential attenuating factor during progression of neuroendocrine neoplasms.

PMID: 15705875 [PubMed - indexed for MEDLINE]


 
5: J Clin Oncol. 2005 Mar 20;23(9):2114-6.
 
Survival after brain metastases from breast cancer in the trastuzumab era.

Kirsch DG, Ledezma CJ, Mathews CS, Bhan AK, Ancukiewicz M, Hochberg FH, Loeffler JS.

PMID: 15774813 [PubMed - in process]


 
6: J Neurol Neurosurg Psychiatry. 2005 Apr;76(4):562-8.
 
Facets and determinants of quality of life in patients with recurrent high grade glioma.

Giovagnoli AR, Silvani A, Colombo E, Boiardi A.

Department of Neurology and Neuropathology, Carlo Besta National Neurological Institute, Via Celoria 11, 20133 Milan, Italy. rgiovagnoli@istituto-besta.it.

OBJECTIVES: To assess patients with recurrent high grade brain glioma with the aim of evaluating facets of quality of life (QOL) and their association with mood, cognition, and physical performance. METHODS: Ninety four glioma patients (four groups with different duration of glioma recurrence) were compared with 24 patients with other chronic neurological diseases and 48 healthy subjects. The Functional Living Index-Cancer (FLIC) provided QOL self evaluations, and standardised scales and neuropsychological tests assessed physical performance, mood, and cognition. RESULTS: In glioma patients, factor analysis of the FLIC items documented five domains: Psychological well being, Role/sociability, Inner experience of disease, Isolation/sharing, and Nausea. Higher FLIC total scores were related to better cognition, physical performances, and mood, and lower grading; poorer Psychological well being and worse Inner experience of disease to depressed mood; minor Role/sociability to worse cognitive and physical performances and higher grading; worse Nausea to longer disease duration. Compared with healthy subjects, all glioma groups were cognitively impaired and more anxious, and two groups with short duration of recurrence were also more depressed. Patients with chronic neurological diseases showed worse mood and cognitive abilities compared with healthy subjects, but performed attention tests better than glioma patients. Glioma and chronic disease patients showed similar FLIC scores and autonomy. CONCLUSIONS: These results show that QOL of recurrent high grade glioma patients is multifaceted and determined by multiple factors. Disease severity does not necessarily eliminate the possibility of expressing personal feelings and opinions which could provide criteria for clinical decision making and psychological support.

PMID: 15774446 [PubMed - in process]


 
7: J Neurol Neurosurg Psychiatry. 2005 Apr;76(4):555-561.
 
Views of bereaved relatives about quality of survival after radiotherapy for malignant cerebral glioma.

Davies E, Clarke C.

Thames Cancer Registry, Guy's, King's, and St Thomas's School of Medicine, 1st Floor, Capital House, 42 Weston Street, London SE1 3QD, UK. elizabethdavies@doctors.org.uk.

OBJECTIVE: To explore the views of bereaved relatives about quality of survival after radiotherapy for malignant cerebral glioma. DESIGN: Semistructured interviews with the bereaved relatives of 56 previously studied patients with glioma. SETTING: Patients treated at six London hospitals from 1990 to 1992 surviving between one and 46 months (median, eight). SUBJECTS: Fifty six relatives (44 spouses, 12 others) seen four to six months after bereavement and 20 again at 13 months. MAIN OUTCOME MEASURES: Views about quality of life and satisfaction with radiotherapy. RESULTS: Relatives described quality of life as "good or acceptable" when patients carried on some normal activities or enjoyed social relationships. They described restricted and dependent states, constant deterioration, or loss of social interaction as giving "poor or unacceptable" quality of life. Length of time lived in such states also appeared important. Relatives' views of good or acceptable quality of life were independently related to low initial cognitive or personality change or low distress in the patient after diagnosis, and to their subsequent survival free from physical disability for at least one month. Satisfaction with radiotherapy was related to low initial distress, some degree of surgical resection, and overall length of survival longer than six months. CONCLUSIONS: Carefully exploring the views of bereaved relatives can bring a useful perspective to difficult treatment decisions. Their values support including disability and distress in quality of life measures, but cast doubt on the QALY-type approach of using full years of survival or time free from disability to judge whether treatments are worthwhile.

PMID: 15774445 [PubMed - as supplied by publisher]


 
8: J Neurol Neurosurg Psychiatry. 2005 Mar;76(3):415-9.
 
The time course of visual field recovery following transphenoidal surgery for pituitary adenomas: predictive factors for a good outcome.

Gnanalingham KK, Bhattacharjee S, Pennington R, Ng J, Mendoza N.

Brindhaven, II Deena Close, Queens Drive, London W3 0HR, UK. kannagnana@doctors.org.uk

OBJECTIVE: To report the quantitative assessment of visual fields (VF) in patients with pituitary macroadenomas, and the time course and predictive factors for recovery of vision. METHODS: Retrospective study of 41 patients with pituitary adenomas and visual disturbance. Patients underwent pre- and postoperative VF assessment at one week, three to six months, one year, two years, and five years using the Humphrey field analyser, which gives a quantitative measure of VF in each quadrant. RESULTS: 36/41 patients (88%) presented with a visual disturbance. Mean (SEM) duration of symptoms was 94 (50) weeks (range 0.5 to 1500); 12 (29%) had optic atrophy at presentation. Impairment of VF was greatest in the upper temporal quadrant, followed by the lower temporal. VF recovery was progressive and apparent even at the five year follow up (p<0.001). Overall, VF returned to normal in 35% of eyes, improved in 60%, and remained unchanged in 5%. Patients whose VF returned to normal had a shorter duration of symptoms (16 (5) v 137 (56) weeks; p<0.05), better preoperative visual acuity (p<0.05), and a smaller degree of impairment in preoperative lateral quadrant VF (p<0.01) than those whose VF only improved. On multivariate analysis, the only predictive factor for VF recovery was the degree of impairment in VF preoperatively. CONCLUSIONS: Transphenoidal surgery for pituitary macroadenoma results in a progressive recovery of VF in 95% of patients. The extent of the VF recovery is mainly dependent on the preoperative VF deficit, which emphasises the need for early intervention in these patients.

PMID: 15716538 [PubMed - indexed for MEDLINE]


 
9: J Neurol Neurosurg Psychiatry. 2005 Mar;76(3):390-4.
 
Lower urinary tract function in patients with pituitary adenoma compressing hypothalamus.

Yamamoto T, Sakakibara R, Uchiyama T, Liu Z, Ito T, Yamanishi T, Hattori T.

Neurology Department, Chiba University, 1-8-1 Inohana Chuo-ku, Chiba 260-8670, Japan.

BACKGROUND: The micturition reflex is under the tonic influence of suprapontine structures including the anteromedial frontal cortex, basal ganglia, and hypothalamus. However, there have been few reports about the role of the hypothalamus on the lower urinary tract (LUT) function in humans. OBJECTIVE: To investigate LUT function in patients with pituitary adenomas. METHODS: Urodynamic studies were carried out in three patients with LUT symptoms who had pituitary adenomas extending upwards to the hypothalamus. RESULTS: All three male patients (age 28 to 62 years) developed LUT symptoms (urinary urgency and frequency (3); urinary incontinence (3); voiding difficulty and retention (2)) along with weight loss, psychiatric symptoms, unsteady gait, and/or visual disturbances. One had the syndrome of inappropriate secretion of antidiuretic hormone, but none had diabetes insipidus. Two had resection of the tumour and subsequent radiation therapy, but LUT dysfunction persisted. The third patient had partial resection of the tumour to ameliorate hydrocephalus. Urodynamic studies showed detrusor overactivity during the storage phase in all patients; during the voiding phase there was underactive detrusor in two and non-relaxing sphincter in one. CONCLUSIONS: Hypothalamic lesions can cause severe LUT dysfunction in both the storage and voiding phases of micturition. This may reflect the crucial role of the hypothalamus in regulating micturition in humans.

Publication Types:
  • Case Reports

PMID: 15716534 [PubMed - indexed for MEDLINE]


 
10: J Neurosurg. 2005 Feb;102(2):402-3; author reply 403.

Comment on:
Spectroscopy and navigation.

Chernov MF, Muragaki Y, Ochiai T, Maruyama T, Izawa M, Hayashi M, Ono Y, Kubo O, Hori T.

Publication Types:
  • Comment
  • Letter

PMID: 15739576 [PubMed - indexed for MEDLINE]


 
11: J Neurosurg. 2005 Feb;102(2):386-8.

Tension pneumoventricle after placement of a ventriculoperitoneal shunt: a novel treatment strategy. Case report.

Perrin RG, Bernstein M.

Division of Neurosurgery, Toronto Western Hospital, Toronto, Ontario, Canada.

A case of pneumoventricle following insertion of a ventriculoperitoneal shunt for hydrocephalus is described. The pneumoventricle was associated with significant morbidity, which improved with treatment. The authors propose a rational treatment for this condition, which, as far as they know, has not previously been described. They also discuss potential mechanisms involved in the pathogenesis of pneumoventricle.

Publication Types:
  • Case Reports

PMID: 15739571 [PubMed - indexed for MEDLINE]


 
12: J Neurosurg. 2005 Feb;102(2):376-81.

Multicentric pleomorphic xanthoastrocytoma in a patient with neurofibromatosis type 1. Case report and review of the literature.

Saikali S, Le Strat A, Heckly A, Stock N, Scarabin JM, Hamlat A.

Department of Pathology, Centre Hospitalier Regional et Universitaire Pontchaillou, Rennes, France.

The authors report an unusual case of multicentric pleomorphic xanthoastrocytoma (PXA) in a 36-year-old woman with neurofibromatosis Type 1 (NF1). Both lesions were diagnosed as PXA but demonstrated different neuroimaging features and very different outcomes. Although the occipital lesion was cured surgically, the cerebellar tumor recurred three times and underwent malignant transformation into an anaplastic oligodendroglioma. The authors discuss the causes of PXA and suggest that it could originate from common bipotential precursor cells with two phenotypes.

Publication Types:
  • Case Reports

PMID: 15739569 [PubMed - indexed for MEDLINE]


 
13: J Neurosurg. 2005 Feb;102(2):342-7.

Biological activity of adult cavernous malformations: a study of 56 patients.

Sure U, Freman S, Bozinov O, Benes L, Siegel AM, Bertalanffy H.

Department of Neurosurgery, Philipps University, Marburg, Germany. sure@med.uni-marburg.de

OBJECT: Cerebral cavernous malformations (CCMs) have previously been considered as congenital and biologically static malformations. On the other hand, the potential for growth and de novo generation of CCMs have also been reported. It is therefore important to study the proliferative and neoangiogenetic capacity of these lesions. METHODS: The authors studied the surgical specimens of 56 CCMs (23 deep and 33 superficial) obtained from adult patients. The proliferative activity of the endothelium and the neoangiogenetic capacity of these lesions were considered through immunohistochemical anaylsis of proliferating cell nuclear antigen (PCNA), MIB-1, Flk-1, vascular endothelial growth factor (VEGF), hypoxia-inducible factor (HIF)-1alpha, and endoglin antibodies. Positive immunostaining of endothelial cells occurred in 86% of patients for PCNA and in 38% of the cases for MIB 1. The expression of Flk-1 was observed in the endothelium of 71% of the cases, for VEGF in 41%, for HIF-1 alpha in 48.1%, and for endoglin in 63.6% of the cases. The correlation of immunohistochemical and clinical data indicated that VEGF was expressed in significantly less deep-seated lesions when compared with superficial CCMs. Neither the expression of the proliferative markers nor the expression of the angiogenetic antibodies correlated with patient age at surgery, sex, or the number of recent prior hemorrhagic episodes in the patients. CONCLUSIONS: The CCMs from adult patients are active lesions exhibiting endothelial proliferation and neoangiogenesis. According to the data in this study, neoangiogenesis is more prominent in superficial CCMs than in deep-seated CCMs and is not associated with recent prior hemorrhages.

PMID: 15739564 [PubMed - indexed for MEDLINE]


 
14: J Neurosurg. 2005 Feb;102(2):336-41.

Diffusion tensor analysis of peritumoral edema using lambda chart analysis indicative of the heterogeneity of the microstructure within edema.

Morita K, Matsuzawa H, Fujii Y, Tanaka R, Kwee IL, Nakada T.

Center for Integrated Human Brain Science, Department of Neurosurgery, Brain Research Institute, University of Niigata, Japan.

OBJECT: Histopathological studies indicate that cerebral edema associated with tumors (peritumoral edema) does not represent a single pathophysiological or clinical entity. In this study the authors investigated peritumoral edema by performing lambda chart analysis (LCA), a noninvasive technique that can be used to make visible and analyze apparent water diffusivity in tissues in vivo, and assessed the utility of LCA in differentiating high-grade gliomas from nonglial tumors. METHODS: The water diffusivity characteristics of peritumoral edema associated with four tumor groups-12 high-grade gliomas, five low-grade gliomas, 11 metastatic tumors, and 15 meningiomas-were assessed in 43 patients by performing magnetic resonance imaging with the aid of a 3-tesla magnetic resonance imaging system. In all tumor groups, peritumoral edema exhibited greater trace values and reduced anisotropy compared with normal white matter. Edema associated with high-grade gliomas had significantly higher trace values than edema associated with the other three tumor groups, although the anisotropic angles of those groups were comparable. CONCLUSIONS: Lambda chart analysis identified two distinct types of peritumoral edema: edema associated with high-grade gliomas and edema associated with low-grade gliomas or nonglial tumors. The apparent water diffusivity was significantly greater in high-grade gliomas, whereas the anisotropy in these lesions was comparable to that of edema in other tumors. These findings indicated that water movement in areas of edema, predominantly in the extracellular spaces, was less restricted in high-grade gliomas, a phenomenon that likely reflected the destruction of the extracellular matrix ultrastructure by malignant cell infiltration and consequently greater water diffusion. Although preliminary, this study indicates that LCA could be used as a clinical tool for differentiating high-grade gliomas and for evaluating the extent of cellular infiltration.

PMID: 15739563 [PubMed - indexed for MEDLINE]


 
15: J Neurosurg. 2005 Feb;102(2):302-10.

Brain surface reformatted images for fast and easy localization of perirolandic lesions.

Hattingen E, Good C, Weidauer S, Herminghaus S, Raab P, Marquardt G, Raabe A, Seifert V, Zanella FE.

Institute of Neuroradiology, Department of Neurosurgery, Johann Wolfgang Goethe University, Frankfurt am Main, Germany. elkejoerg@web.de

OBJECT: The goal of this study was to evaluate a novel form of brain surface representation that allows simple, reliable mapping of the surface neuroanatomy for the preoperative evaluation of the spatial relationship between a focal lesion and the precentral gyrus. METHODS: High-resolution three-dimensional (3D) magnetic resonance (MR) imaging data sets were postprocessed using a curved multiplanar reformatting technique to create brain surface reformatted (BSR) images. These BSR images were reconstructed in less than 5 minutes and demonstrated the entire central sulcus with adjacent surface structures in one view. Two experienced neuroradiologists determined the localization of lesions near the central sulcus in 27 patients on standard MR images in three orthogonal planes and on BSR images. In addition, these observers judged whether the lesions were easy or difficult to localize on standard MR and BSR images, and whether diagnoses based on these methods were certain or doubtful. Anatomical localization based on BSR images was compared with that based on functional MR (fMR) images or intraoperative mapping of motor function. The BSR images yielded a perfect concordance with the fMR images and intraoperative mapping (Cohen kappa 1.0) and optimal diagnostic accuracy in localizing perirolandic lesions (both sensitivity and specificity were 100%). Localization was judged to be easy for 48 of 54 diagnoses based on BSR images compared with 26 of 54 based on standard MR images. Diagnoses were assessed as certain for 52 cases based on BSR images and 34 cases based on standard MR images. CONCLUSIONS: Brain surface reformatted imaging improves the diagnostic accuracy of standard anatomical MR imaging for localizing superficial brain lesions in relation to the precentral gyrus. The complementary use of this technique with standard two-dimensional imaging is supported by the fast and simple postprocessing technique and may provide useful information for preoperative surgical planning.

PMID: 15739559 [PubMed - indexed for MEDLINE]


 
16: J Neurosurg. 2005 Feb;102(2):295-301.

Intraoperative power Doppler ultrasonography with a contrast-enhancing agent for intracranial tumors.

Kanno H, Ozawa Y, Sakata K, Sato H, Tanabe Y, Shimizu N, Yamamoto I.

Department of Neurosurgery, Yokohama City University Graduate School of Medicine, Yokohama, Japan. kanno@med.yokohama-cu.ac.jp

OBJECT: The goal of this study was to evaluate intraoperative power Doppler ultrasonography when used with a contrast-enhancing agent for operations on intracranial tumors. METHODS: Forty intracranial tumors were examined using power Doppler ultrasonography with a galactose microparticle-based ultrasonographic contrast-enhancing agent during operations on the brain. The tumors included 37 intracranial neoplasms (14 gliomas, six meningiomas, three hemangioblastomas, two malignant lymphomas, three other primary neoplasms, nine metastatic tumors, and three nonneoplastic lesions). All patients also underwent computerized tomography and magnetic resonance imaging, and all but three of the patients underwent digital subtraction (DS) angiography. Before injection of the ultrasonographic contrast agent, intra- and peritumoral power Doppler flow signals were detected in 32 of the intracranial tumors. After the injection, the signals were enhanced in blood vessels around the tumors and in the tumor parenchyma in 36 tumors. The duration of contrast enhancement continued for 70 to 365 seconds (mean 251.8 +/- 69 seconds) after the injection. Among the tumors, hemangioblastomas displayed particularly strong contrast enhancement. In these intracranial tumors, the echo signals obtained using contrast-enhanced power Doppler ultrasonography correlated with DS angiographic staining. Power Doppler ultrasonograms with the appropriate contrast agent provided better data on the precise real-time position of the tumors and their relationship to adjacent vessels than ultrasonograms obtained before the injection of the contrast agent. CONCLUSIONS: Intraoperative power Doppler ultrasonography performed using a contrast-enhancing agent can facilitate intraoperative real-time navigation and assessment of the intratumoral vasculature and peritumoral vessels, particularly for tumors having abundant vessels such as hemangioblastomas.

PMID: 15739558 [PubMed - indexed for MEDLINE]


 
17: J Neurosurg. 2005 Feb;102(2):189-93.

Surgical outcomes in 118 patients with Rathke cleft cysts.

Aho CJ, Liu C, Zelman V, Couldwell WT, Weiss MH.

Department of Neurosurgery, University of Southern California, Los Angeles, California, USA.

OBJECT: Microscopic Rathke cleft cysts are a common incidental autopsy finding, but some Rathke cleft cysts can become sufficiently large to cause visual impairment, hypothalamic-pituitary dysfunction, and headaches. In this study patients were evaluated pre- and postoperatively to ascertain the clinical significance of surgical intervention on endocrine and visual improvement. Factors correlated with cyst recurrence were also evaluated. METHODS: A retrospective analysis was conducted in 160 patients with Rathke cleft cysts who were treated between 1984 and 1995 and completed at least a 5-year follow-up period. Of these 160 patients, 118 initially exhibited symptoms of visual impairment or endocrine dysfunction, became symptomatic during the follow-up period, or were found to have cyst enlargement. These 118 patients underwent transsphenoidal surgery. Forty-two patients with incidental lesions that demonstrated no growth on magnetic resonance (MR) images were followed up without an operation. Complete resection, as observed on MR images 3 months postoperatively, was obtained in 114 (97%) of 118 patients. Vision improved postoperatively in 57 (98%) of 58 patients. Hypogonadism improved in 11 (18%) of 62 patients, growth hormone deficiency resolved in 14 (18%) of 78 patients, and hypocortisolemia resolved in one (14%) of seven patients. Twenty-two patients (19%) began to exhibit symptoms of diabetes insipidus, which had not been present preoperatively. The total 5-year recurrence rate was 18% (21 of 118 patients), with 12 patients requiring a repeated operation. Surgical and pathological factors that were found to be statistically associated with recurrence were the use of a fat and/or fascial graft for closure (p < 0.01) and the presence of squamous metaplasia in the cyst wall (p < 0.01). The extent of resection of the cyst wall was not associated with an increased rate of recurrence. In 42 (69%) of 61 patients the incidental cysts did not progress on imaging studies or clinically. CONCLUSIONS: This is the largest series of patients with symptomatic Rathke cleft cysts who received operative intervention and participated in the longest postoperative follow up reported in the literature. The high recurrence rate (18%) supports the theory that a relationship exists between a symptomatic Rathke cleft cyst and craniopharyngioma. Improvements in visual and endocrine dysfunction can be expected after surgical decompression of the optic apparatus and the hypothalamic-pituitary axis.

PMID: 15739543 [PubMed - indexed for MEDLINE]
 

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