Object: The question of whether to obtain routine or selective preoperative imaging of the neuraxis in pediatric patients with cerebellar neoplasms remains a controversial topic. Staging of the neuraxis is generally considered beneficial in patients with neoplasms associated with an elevated risk of leptomeningeal dissemination (LD). When these studies are obtained preoperatively, there is a decrease in the number of false-positive images related to debris in the immediate postoperative period. Additionally, knowledge of the extent of spread has the potential to affect the risk/benefit analysis of aggressive resection. Although the majority of pediatric neurosurgeons surveyed choose to obtain selective preoperative imaging of the neuraxis in cases of cerebellar neoplasms "with findings suggestive of high-grade pathology," an evidence-based protocol in the literature is lacking. The goal of this study was to assess radiological characteristics of tumors with an elevated risk of LD and identify a method to help guide preoperative imaging of the neuraxis.
Methods: The authors first reviewed the literature to gain an appreciation of the risk of LD of pediatric cerebellar neoplasms based on underlying histopathology and/or grade. Available evidence indicates preoperative imaging of the neuraxis in patients with Grade I tumors to be of questionable utility. In contrast, evidence suggested that preoperative imaging of the neuraxis in patients with Grades II-IV neoplasms was clinically warranted. The authors then evaluated an extensive base of neuroradiological literature to identify possible MR imaging and/or CT findings with the potential to differentiate Grade I from higher-grade neoplasms in pediatric patients. They analyzed the preoperative radiological findings in 50 pediatric patients who had undergone craniotomy for resection of cerebellar neoplasms at Vanderbilt Children's Hospital since 2003 with reference to 7 chosen radiological criteria. Logistic regression models were fit using radiological features to determine the best predictors of Grades II-IV tumors. Receiver operating characteristic methods were used to identify diagnostic properties of the best predictors.
Results: The relative T2 signal intensity (RT2SI), an indirect measure of the water content of the solid component of the tumor, was best able to identify neoplasms with an elevated risk of LD. An RT2SI value of 0.71 was selected by the authors as the best operating point on the curve. Of the 31 neoplasms retrospectively designated as hypointense T2-weighted lesions (RT2SI ≤ 0.71), 30 (97%) were Grade II or higher. All medulloblastomas, ependymomas, and high-grade (Grades III and IV) neoplasms were hypointense T2-weighted lesions. Of the 19 T2-weighted hyperintense neoplasms (RT2SI > 0.71), 16 (84%) were Grade I and 3 were Grade II.
Conclusions: Measurement of the RT2SI can help predict Grade II-IV tumors at an elevated risk of leptomeningeal spread and guide staging of the neuraxis. Pediatric patients with cerebellar neoplasms found to have an RT2SI of less than or equal to 0.71 are recommended for neuraxis imaging prior to surgery.
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http://dx.doi.org/10.3171/2010.11.PEDS10312 | DOI Listing |
Childs Nerv Syst
December 2024
Department of Neurosurgery, Medical University of Vienna, Währinger Gürtel 18-20, 1090, Vienna, Austria.
Purpose: We aimed to present our surgical experience and the impact of a solid or cystic morphology of cerebellar pilocytic astrocytoma (cPA) on surgery and the risk for a re-resection.
Methods: We retrospectively analyzed all children operated at our institution between 2009 and 2023 for cPA. Tumours were categorized into 4 groups: (i) cystic PA without cyst wall enhancement, (ii) cystic PA with cyst wall enhancement, (iii) solid tumour, (iv) and solid tumour with central necrosis.
Acta Neuropathol Commun
December 2024
Michigan Center for Translational Pathology, University of Michigan, Ann Arbor, MI, USA.
We identified a rare heterozygous germline loss-of-function variant in the tumor necrosis factor receptor-associated factor 2 (TRAF2) in a young adult patient diagnosed with medulloblastoma. This variant is located within the TRAF-C domain of the E3 ubiquitin ligase protein and is predicted to diminish the binding affinity of TRAF2 to upstream receptors and associated adaptor proteins. Integrative genomics revealed a biallelic loss of TRAF2 via partial copy-neutral loss-of-heterozygosity of 9q in the medulloblastoma genome.
View Article and Find Full Text PDFMedicine (Baltimore)
December 2024
Department of Neurosurgery, The First Affiliated Hospital of Shihezi University, Shihezi, China.
Rationale: This study aims to present a case of cerebellar dysplastic ganglioneuroma, which is commonly referred to as Lhermitte-Duclos disease (LDD). Furthermore, the study aims to provide an extensive review of the essential aspects of LDD, thereby providing essential information for its accurate diagnosis and effective treatment.
Patient Concerns: A 54-year-old woman was admitted with symptoms of headache, facial numbness, and a visible cerebellar mass.
Surg Neurol Int
November 2024
Department of Pathology and Laboratory Medicine, All India Institute of Medical Sciences, Rishikesh, Uttarakhand, India.
Background: Neurocysticercosis (NCC) is one of the leading parasitic infections of the brain. Giant NCC is rare, with only two cases of cerebellar involvement reported till now. In the presence of a host immune response, these giant NCCs can mimic primary central nervous system neoplasms.
View Article and Find Full Text PDFActa Med Acad
August 2024
Medical School, National and Kapodistrian University of Athens, Athens, Greece.
Background: Paraneoplastic Neurological Syndromes (PNS) constitute a heterogeneous cluster of disease manifestations related to various cancers. Small Cell Lung Cancer (SCLC) is strongly related to PNS. This narrative review conducted a survey in the available PubMed literature to highlight the appearance of PNSs in SCLC cases and discuss published research highlights on the subject so that general practitioners can be acquainted with the medical phenomenon present in SCLC patients.
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