Einige neuere Übersichtsartikel (Medulloblastom)

[Ulrich]

Hier einige neuere Übersichtsartikel (reviews) zum Thema Medulloblastom. Sie wurden über Medline recherchiert. Suchbegriffe >medulloblastoma review< (aber ohne die Anführungszeichen). Bei Interesse müßte man sich den betreffenden Artikel in der nächstgelegenen Universitätsbibliothek kopieren. Hier finden Sie nur die Zusammenfassungen (abstracts).

Quelle: Cancer. 2005 Jan 7; [Epub ahead of print]
Zitat: Three-year recurrence-free survival in a patient with recurrent medulloblastoma after resection, high-dose chemotherapy, and intrathecal Yttrium-90-labeled DOTA(0)-D-Phe(1)-Tyr(3)-octreotide radiopeptide brachytherapy.
Beutler D, Avoledo P, Reubi JC, Macke HR, Muller-Brand J, Merlo A, Kuhne T.
Department of Oncology/Hematology, University Children´s Hospital Basel, Basel, Switzerland.
BACKGROUND:
Most medulloblastomas express high levels of somatostatin type 2 receptors (sst2). DOTA(0)-D-Phe(1)-Tyr(3)-octreotide (DOTATOC) specifically binds sst2 in the low nanomolar range. The cytotoxic effect is mediated by the chelated, beta-emitting, metallic radionuclide Yttrium 90 ((90)Y). The authors applied this innovative treatment option in a boy age 8 years who presented with a recurrent medulloblastoma of the cauda equina: a prognostically poor condition. Targeted radiotherapy was administered to treat minimal sst2-expressing tumor remnants, which persisted despite conventional and high-dose chemotherapy and intercurrent resection of the lesion.
METHODS:
A medulloblastoma arising from the floor of the fourth ventricle had been removed surgically; then, the patient was treated with standard adjuvant chemotherapy and craniospinal irradiation according to the prospective HIT ´91 protocol. Complete remission was achieved for 20 months, when a drop metastasis of the cauda equina manifested with sensorimotor lumbosacral deficits and urinary incontinence. After four cycles of neoadjuvant chemotherapy (which consisted of combined ifosfamide, carboplatinum and etoposide), two cycles of high-dose chemotherapy and autologous stem cell transplantation were performed; in between, the responding residual tumor within the lumbosacral nerve fibers was microscopically removed. Thereafter, an Indium-111-DOTATOC test injection indicated sst2-expressing tumor remnants within the cauda equina. Con- sequently, 4 cycles of [(90)Y]-DOTATOC (4 x 562.5 megabecquerels) were injected directly into the cerebrospinal fluid in monthly intervals.
RESULTS:
The consolidating intrathecal brachytherapy using [(90)Y]-DOTATOC was tolerated well. A complete remission was achieved for a 3-year period. The only remaining deficit was urinary incontinence.
CONCLUSIONS;
Intrathecal administration of targeted radiopeptide brachytherapy in combination with conventional and high-dose chemotherapy and surgical removal represents a promising new option to treat recurrent medulloblastoma and should be explored further.
Cancer 2005. (c) 2005 American Cancer Society.

Quelle: Crit Rev Oncol Hematol. 2004 May;50(2):121-8.
Zitat: Review of the prognostic factors in medulloblastoma of children and adults.
Brandes AA, Paris MK.
Medical Oncology Department, University Hospital, Via Gattamelata 64, 35100 Padova, Italy. aabrandes@unipd.it

Medulloblastoma (MB) is rare in adults, accounting for 1% of all primary tumours of the central nervous system (CNS). Based on the assumption that the disease pattern in adults is similar to that in children, adults with medulloblastoma are treated using paediatric protocols. Thanks to progress made in recent years, long-term survival is now possible, with overall ranging from 50 to 60% at 5 years and 40 to 50% at 10 years. However, effective therapy may have devastating long-term side effects, including neuro-psychic and neuro-endocrine sequelae and cognitive dysfunction, especially in young adults. Great interest has been expressed in new biological and molecular prognostic factors, which, combined with clinical variables, may allow a more satisfactory stratification of patients.

Quelle: Lancet Oncol. 2004 Apr;5(4):209-18.
Zitat: Medulloblastoma: signalling a change in treatment.
Gilbertson RJ.
Department of Developmental Neurobiology, St Jude Children's Research Hospital, Memphis, TN 38105, USA. Richard.Gilbertson@stjude.org

Medulloblastoma is the most common malignant brain tumour that occurs during childhood. Multimodality treatment regimens have substantially improved survival in this disease; however, the tumour is incurable in about a third of patients with medulloblastoma, and current treatment has a detrimental effect on long-term survivors. Drugs that target cell-signalling pathways provide an alternative to conventional cytotoxic approaches to treatment of cancer. Several pathways have been implicated in medulloblastoma formation, and knowledge of these is now being used to develop new ways of treating children with medulloblastoma.

Quelle: Radiographics. 2003 Nov-Dec;23(6):1613-37.
Zitat: From the archives of the AFIP: medulloblastoma: a comprehensive review with radiologic-pathologic correlation.
Koeller KK, Rushing EJ.
Department of Radiologic Pathology, Armed Forces Institute of Pathology, 14th St at Alaska Ave, Bldg 54, Washington, DC 20306-6000, USA. koeller@afip.osd.mil

Medulloblastoma is the most common pediatric central nervous system malignancy and the most common primary tumor of the posterior fossa in children. This highly malignant neoplasm occurs more frequently in males and usually before 10 years of age. Clinical symptoms and signs are generally brief, typically less than 3 months in duration, and reflect the strong predilection of this tumor to arise within the cerebellum, most often in the vermis. Although much less common, the disease may also occur in adults, usually in the 3rd and 4th decades of life. Surgical resection, radiation therapy, and chemotherapy have substantially lowered the mortality associated with this tumor, with 5-year survival rates now commonly well above 50%. Still, both dissemination at the time of diagnosis and recurrence remain obstacles in achieving a cure. The tumor has characteristic hyperattenuation on unenhanced computed tomographic scans that reflects the high nuclear-cytoplasmic ratio seen at histologic analysis. The tumor typically appears heterogeneous on images, findings that are related to cyst formation, hemorrhage, and calcification and that are even more pronounced with magnetic resonance (MR) imaging. Evidence of leptomeningeal metastatic spread is present in 33% of all cases at the time of diagnosis and is well evaluated with contrast-enhanced MR imaging of the brain and the spine. Although controversial, postoperative surveillance with MR imaging is performed at most institutions in the hope of facilitating a better outcome. With continued research, treatment of these common neoplasms should improve, perhaps even achieving a cure in the future.

Quelle: Expert Rev Anticancer Ther. 2003 Oct;3(5):615-20.
Zitat: Medulloblastomas: do molecular and biologic markers indicate different prognoses and treatments?
Brandes AA, Paris MK, Basso U.
Department of Medical Oncology, University Hospital, Padova, Italy. aabrandes@unipd.it

The origin and histological classification of embryonal tumors, which make up the largest group of malignant pediatric brain malignancies, are two controversial issues. However, in recent years, progress has been made in our understanding of the molecular genetic abnormalities that govern their onset and/or progression. Several of these abnormalities appear to involve alterations in the signaling system controlling normal cerebellar development. An improved understanding of both the biology and clinical relevance of these molecular defects is probably the only means by which neuro-oncologists will improve the management of these tumors, thus reducing disease- and treatment-related morbidity and mortality.

Quelle: Curr Treat Options Oncol. 2003 Dec;4(6):499-508.
Zitat: Medulloblastomas and central nervous system primitive neuroectodermal tumors.
McLean TW.
Department of Pediatrics, Wake Forest University School of Medicine, Medical Center Boulevard, Winston-Salem, NC 27157, USA. tmclean@wfubmc.edu

Significant advances in the treatment of medulloblastoma and primitive neuroectodermal tumors have been made in the past three decades. Maximal surgical resection is a mainstay of therapy. However, unlike many other central nervous system neoplasms, medulloblastoma and primitive neuroectodermal tumors are radiation and chemotherapy responsive. Despite this response, the prognosis for patients with these tumors remains variable and is relatively poor in infants and patients with metastatic disease. These tumors most commonly arise in children, thus most clinical trials emphasize the reduction of long-term sequelae, in addition to improving survival. All newly diagnosed patients who are eligible should be offered participation in a clinical trial. If a patient is ineligible or declines consent/assent for a clinical trial, the best current treatment approach is surgical resection, followed by radiation therapy (except for children younger than 3 years) with weekly vincristine. For high-risk patients, 36 Gy of craniospinal irradiation should be delivered plus a boost of 19.8 Gy to the posterior fossa/primary tumor bed and sites of bulk metastatic disease. For average-risk patients, the craniospinal irradiation dose may be lowered to 23.4 Gy plus 32.4 Gy to the posterior fossa/tumor bed. After radiation therapy, intensive multimodal chemotherapy should be used for all patients.

Quelle: Cancer. 2003 Sep 1;98(5):1014-20.
Zitat: Evaluation of dietetic intervention in children with medulloblastoma or supratentorial primitive neuroectodermal tumors.
Bakish J, Hargrave D, Tariq N, Laperriere N, Rutka JT, Bouffet E.
Division of Haematology/Oncology, The Hospital for Sick Children, 555 University Avenue, Toronto, Ontario M5G 1X8, Canada.

BACKGROUND: Malnutrition is a common complication of cancer treatment; it can affect energy levels and, as a consequence, quality of life. The goal of the current study was to evaluate the effect of dietetic intervention in a cohort of children treated for medulloblastoma and supratentorial primitive neuroectodermal tumors (PNET) over a 10-year period.
METHODS: A retrospective chart review (1992-2002) of newly diagnosed cases of medulloblastoma/supratentorial PNET was performed. Hospital records were reviewed for data, including demographic characteristics, patient heights and weights, and information on treatment modalities and the use of dietetic intervention. Percent changes in body weight were calculated at time points associated with particular stages of treatment or dietetic intervention.
RESULTS: One hundred three of 112 cases were evaluable. Treatment methods included surgery only (7.8%), surgery + radiotherapy (16.5%), surgery + chemotherapy (14.5%), and surgery + radiotherapy + chemotherapy (61.2%). There was no significant change in patient weight due to surgery (median change in body weight [MCBW], -0.35%) or radiotherapy (MCBW, -0.78%). In contrast, children experienced significant weight loss (MCBW, -4.35%; P < 0.0001) 3 months after starting chemotherapy. A dietician saw 53 of the 103 children in the study cohort. There were 84 dietetic interventions (oral, 36%; parenteral, 27%; enteral, 37%) among these 53 patients. Oral diets did not result in weight gain. Parenteral nutrition was associated with significant weight gain at 1 month (MCBW, +2.7%; P = 0.03), but not at 3 months. The use of enteral feeds resulted in significant weight gain at 1 month (MCBW, +4.8%; P = 0.006) and at 3 months (MCBW, +11.8%; P < 0.0001).
CONCLUSIONS: Current multimodality treatment of intracranial PNET results in significant nutritional morbidity, primarily due to the use of intensive chemotherapy regimens. Dietetic input for pediatric patients with medulloblastoma/PNET is essential, and the implementation of enteral feeding in these children can help to reverse their nutritional morbidity. Copyright 2003 American Cancer Society.

Quelle: J Neurosurg. 2003 Aug;99(2):280-6.
Zitat: Surveillance neuroimaging of intracranial medulloblastoma in children: how effective, how often, and for how long?
Saunders DE, Hayward RD, Phipps KP, Chong WK, Wade AM.
Department of Neuroradiology and Neurosurgery, Great Ormond Street Hospital, London, United Kingdom. SaundD@gosh.nhs.uk

OBJECT: The goal of this paper was to review brain and spine images obtained in children with medulloblastomas to determine the risk factors for tumor recurrence and to assess the impact of surveillance imaging on patient outcomes among patients who remain alive 1 month postsurgery.
METHODS: Imaging studies and clinical data obtained in children with medulloblastomas, who presented between January 1987 and August 1998, were retrospectively reviewed. Images were termed surveillance if they were follow-up studies and symptom prompted if they were obtained to investigate new symptoms. One hundred seven patients (mean age 6 years and 3 months, range 2 months-15 years and 6 months) were entered into the study. Fifty-three children experienced tumor recurrence; 41 had one recurrence, nine had two, and three had three recurrences. Surveillance imaging revealed 10 of the first 53 recurrences and 15 of all 68 recurrences. When the first recurrence was identified by the emergence of symptoms (42 patients), the children tended to survive for a shorter time (hazard ratio 3.72, 95% confidence interval 1.42-9.76, p = 0.008) than children in whom the first recurrence was detected before symptoms occurred (10 patients). The median survival time following symptomatic tumor recurrence was 4 months and that after surveillance-detected tumor recurrence was 17 months. The median increased survival time among patients whose recurrence was asymptomatic and identified by imaging studies was 13 months, more than half the mean time between surveillance imaging sessions. Incomplete tumor resection was associated with a significantly reduced time to recurrence (p = 0.048) and to death (p = 0.002). The number of recurrences that were experienced was associated with a reduced time to death (p < 0.001). CONCLUSIONS: Surveillance imaging is associated with an increase in survival in children with medulloblastomas. More frequent surveillance imaging in children with incomplete tumor excision and recurrent disease may further improve the length of survival.

Quelle: Pediatr Neurosurg. 2003 Jul;39(2):60-7.
Zitat: Medulloblastoma: present concepts of stratification into risk groups.
Packer RJ, Rood BR, MacDonald TJ.
Department of Neurology, Center for Neuroscience and Behavioral Medicine, Children's National Medical Center, Washington, DC 20010, USA. rpacker@cnmc.org

For the past two decades, staging studies have been used to stratify children with medulloblastoma into risk groups. Therapeutic approaches have been based on separation of patients into 'average-risk' and 'poor-risk' categories. The extent of disease at diagnosis has been most reproducibly shown to be of prognostic significance, but age at diagnosis and amount of residual disease after surgery or extent of resection have also been commonly incorporated into stratification schemata. Tumor histology has been variably related to outcome. Biologic markers, especially molecular genetic findings, have not yet been incorporated into risk classifications, but will likely add to the understanding of medulloblastoma and may significantly alter concepts of staging and treatment. Copyright 2003 S. Karger AG, Basel

Quelle: Eur J Paediatr Neurol. 2003;7(2):53-66
Zitat: What's new in neuro-oncology? Recent advances in medulloblastoma.
Ellison DW, Clifford SC, Gajjar A, Gilbertson RJ.
The Northern Institute for Cancer Research, University of Newcastle upon Tyne, The Medical School, Newcastle upon Tyne, UK. d.w.ellison@ncl.ac.uk