ABSTRACT
PURPOSE: Inflammatory myofibroblastic tumor (IMT) is a rare neoplastic tumor type of intermediate biological potential, only recently distinguished from the non-neoplastic category of inflammatory pseudotumor (IP). The literature describes very few cases of IMTs arising in the central nervous system (CNS), and the distinguishing clinical, pathological, and molecular features of IMT-CNS are not well understood. Our purpose is to publish a case of an IMT-CNS with a novel DCTN1-ALK gene fusion, furthering in the literature's characterization of a rare tumor type. METHODS: Review of the literature included a PubMed Database search of articles found by the following searches: "Inflammatory myofibroblastic tumor;" "Inflammatory myofibroblastic tumor central nervous system;" "ALK gene fusion;" and "DCTN1-ALK gene fusion." Inclusion of articles discovered by these search terms was determined through critical appraisal of article relevance, number of citations, cross-citation within articles of interest, and rare findings with conflicting conclusions in an effort to reduce publication bias. RESULTS: We present a case of IMT-CNS with several distinctive molecular features including a DCTN1-ALK gene fusion, the first of its kind described in an intracranial IMT. CONCLUSION: IMT is an infrequent tumor type and its presentation within the CNS is exceedingly rare. The paucity of cases, along with the ambiguity of terminology in the literature, has stunted accurate clinical, pathological, and molecular characterization of IMT-CNS. Our case report improves the characterization of the recently appreciated category of IMT-CNS so that connections between phenotype and prognosis, and between genotype and treatment, can eventually be made.
Subject(s)
Granuloma, Plasma Cell , Anaplastic Lymphoma Kinase , Central Nervous System , Dynactin Complex , Gene Fusion , Granuloma, Plasma Cell/diagnostic imaging , Granuloma, Plasma Cell/genetics , Granuloma, Plasma Cell/surgery , Humans , Receptor Protein-Tyrosine Kinases/geneticsABSTRACT
OBJECTIVE: The present systematic review and meta-analysis analyzes the available clinical literature on post-intracerebral hemorrhage (ICH) cognitive impairment. METHODS: We conducted a systematic review with meta-analysis following PRISMA guidelines. A search of bibliographic databases up to July 31, 2020 yielded 2155 studies. Twenty articles were included in our final qualitative systematic review and 18 articles in quantitative meta-analysis. RESULTS: Based on analysis of data from 18 studies (3270 patients), we found prevalence of post-ICH cognitive impairment to be 46% (confidence interval, 35.9-55.9), with a follow-up duration ranging from 8 days to 4 years. The estimated pooled prevalence of cognitive decline decreased over longitudinal follow-up, from 55% (range, 37.7%-71.15%) within 6 months of ICH to 35% (range, 27%-42.7%) with >6 months to 4 years follow-up after ICH. The modalities used to evaluate cognitive performance after ICH in studies varied widely, ranging from global cognitive measures to domain-specific testing. The cognitive domain most commonly affected included nonverbal IQ, information processing speed, executive function, memory, language, and visuoconstructive abilities. Prognostic factors for poor cognitive performance included severity of cortical atrophy, age, lobar ICH location, and higher number of hemorrhages at baseline. CONCLUSIONS: The prevalence of post-ICH cognitive impairment is high. Despite the heterogeneity among studies, the present study identified cognitive domains most commonly affected and predictors of cognitive impairment after ICH. In future, prospective cohort studies with larger sample sizes and standardized cognitive domains testing could more accurately determine prevalence and prognostic factors of post-ICH cognitive decline.
