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Central Nervous System Inflammatory Myofibroblastic Tumor

MedGen UID:
233555
•Concept ID:
C1334183
•
Neoplastic Process

Definition

A multinodular intermediate fibroblastic neoplasm affecting the central nervous system. It is characterized by the presence of spindle-shaped fibroblasts and myofibroblasts, and a chronic inflammatory infiltrate composed of eosinophils, lymphocytes and plasma cells. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVCentral Nervous System Inflammatory Myofibroblastic Tumor

Professional guidelines

PubMed

Management Strategies of Plasma Cell Granuloma Involving the Central Nervous System: A Systematic Review of the Literature.
Sreenivasan S, Agarwal N, Bharath Raju, Kandregula S, Narayan V, Chen CC, Sharma M
World Neurosurg 2023 Dec;180:194-202.e11. Epub 2023 Sep 13 doi: 10.1016/j.wneu.2023.09.026. PMID: 37708970

Recent clinical studies

Etiology

Distinct fibroblast subpopulations associated with bone, brain or intrapulmonary metastasis in advanced non-small-cell lung cancer.
Xu K, Wang H, Zou YX, Zhang HH, Wang YN, Ren XR, Wang HQ, Xu YH, Li JJ, Tang H, He C, Wei S, Tian T, Li LL, Zhou H, Xu LJ, Fang JW, Guo C, Yang JX, Zhou YY, Zhang ZH, Pan YY
Clin Transl Med 2024 Mar;14(3):e1605. doi: 10.1002/ctm2.1605. PMID: 38445456Free PMC Article
Inflammatory myofibroblastic tumors: A short series with an emphasis on the diagnostic and therapeutic challenges.
Nishadham V, Rao S, Saravanan A, Kulanthaivelu K, Vengalil S, Venkatappa HA, Valasani RK, Bardhan M, Pruti N, Nalini A, Mahadevan A
Clin Neuropathol 2023 May-Jun;42(3):100-111. doi: 10.5414/NP301540. PMID: 37051870
Benign skull and subdural lesions in patients with prior medulloblastoma therapy.
Kaneva K, Wadhwani N, DiPatri AJ Jr, Palasis S, Goldman S, Aw-Zoretic J
Childs Nerv Syst 2021 Feb;37(2):359-366. Epub 2020 Sep 2 doi: 10.1007/s00381-020-04874-2. PMID: 32876801
Differentiating Primary Central Nervous System Lymphomas From Glioblastomas and Inflammatory Demyelinating Pseudotumor Using Relative Minimum Apparent Diffusion Coefficients.
Wen JB, Huang WY, Xu WX, Wu G, Geng DY, Yin B
J Comput Assist Tomogr 2017 Nov/Dec;41(6):904-909. doi: 10.1097/RCT.0000000000000636. PMID: 28708728
Inflammatory pseudotumor of the choroid plexus.
Bramwit M, Kalina P, Rustia-Villa M
AJNR Am J Neuroradiol 1997 Aug;18(7):1307-9. PMID: 9282860Free PMC Article

Diagnosis

Inflammatory myofibroblastic tumor of the lung involving the central nervous system in pediatric patients.
Ramos JG, Ochoa A, Cicutti S, Del Rio R, Lubienieki F
Childs Nerv Syst 2023 May;39(5):1137-1145. Epub 2023 Apr 5 doi: 10.1007/s00381-023-05925-0. PMID: 37017800
Brain pseudotumour secondary to Behçet's disease.
Mousa AE, Okasha M, Isaacs JD, Price DA, Bhatnagar P, Joshi A, Surash S
Ann R Coll Surg Engl 2019 Nov;101(8):e164-e168. Epub 2019 Sep 20 doi: 10.1308/rcsann.2019.0134. PMID: 31537109Free PMC Article
A novel KIF5B-ALK fusion in a child with an atypical central nervous system inflammatory myofibroblastic tumour.
Maruggi M, Malicki DM, Levy ML, Crawford JR
BMJ Case Rep 2018 Oct 21;2018 doi: 10.1136/bcr-2018-226431. PMID: 30344149Free PMC Article
IgG4-related inflammatory pseudotumors mimicking multiple meningiomas.
Nishino T, Toda J, Nakatsuka T, Kimura T, Inaoka T, Terada H
Jpn J Radiol 2013 Jun;31(6):405-7. Epub 2013 Mar 1 doi: 10.1007/s11604-013-0191-y. PMID: 23456546
Inflammatory pseudotumor.
Narla LD, Newman B, Spottswood SS, Narla S, Kolli R
Radiographics 2003 May-Jun;23(3):719-29. doi: 10.1148/rg.233025073. PMID: 12740472

Therapy

Rapid Response to Lorlatinib in a Patient With TFG-ROS1 Fusion Positive Inflammatory Myofibroblastic Tumor of the Chest Wall Metastatic to the Brain and Refractory to First and Second Generation ROS1 Inhibitors.
Carcamo B, Bista R, Wilson H, Reddy P, Pacheco J
J Pediatr Hematol Oncol 2021 Jul 1;43(5):e718-e722. doi: 10.1097/MPH.0000000000002185. PMID: 34157012
Primary intracranial plasma cell granuloma responsive to rituximab.
Schneider C, Henning TD, Fink GR, Schroeter M, Lehmann HC
Neurology 2014 Sep 16;83(12):1119-20. Epub 2014 Aug 8 doi: 10.1212/WNL.0000000000000799. PMID: 25107881
Clinical and cytogenetic analysis of an intracranial inflammatory myofibroblastic tumor induced by a ventriculoperitoneal shunt.
de Oliveira RS, Amato MC, Brassesco MS, Valera ET, Jucá CE, Neder L, Tone LG, Machado HR
J Neurosurg Pediatr 2009 Oct;4(4):372-7. doi: 10.3171/2009.5.PEDS0958. PMID: 19795970
Inflammatory pseudotumors of the central nervous system.
Lui PC, Fan YS, Wong SS, Chan AN, Wong G, Chau TK, Tse GM, Cheng Y, Poon WS, Ng HK
Hum Pathol 2009 Nov;40(11):1611-7. Epub 2009 Aug 5 doi: 10.1016/j.humpath.2009.04.016. PMID: 19656549
Inflammatory pseudotumor associated with HIV, JCV, and immune reconstitution syndrome.
Gonzalez-Duarte A, Sullivan S, Sips GJ, Naidich T, Kleinman G, Murray J, Morgello S, Germano I, Mullen M, Simpson D
Neurology 2009 Jan 20;72(3):289-90. doi: 10.1212/01.wnl.0000339488.32588.0f. PMID: 19153378Free PMC Article

Prognosis

Inflammatory myofibroblastic tumor of the lung involving the central nervous system in pediatric patients.
Ramos JG, Ochoa A, Cicutti S, Del Rio R, Lubienieki F
Childs Nerv Syst 2023 May;39(5):1137-1145. Epub 2023 Apr 5 doi: 10.1007/s00381-023-05925-0. PMID: 37017800
DCTN1-ALK gene fusion in inflammatory myofibroblastic tumor (IMT) of the CNS.
Vidrine DW, Berry JF, Garbuzov A, Falcon C, Tubbs RS, Bui CJ
Childs Nerv Syst 2021 Jul;37(7):2147-2151. Epub 2021 May 20 doi: 10.1007/s00381-021-05219-3. PMID: 34014367
Clinical and cytogenetic analysis of an intracranial inflammatory myofibroblastic tumor induced by a ventriculoperitoneal shunt.
de Oliveira RS, Amato MC, Brassesco MS, Valera ET, Jucá CE, Neder L, Tone LG, Machado HR
J Neurosurg Pediatr 2009 Oct;4(4):372-7. doi: 10.3171/2009.5.PEDS0958. PMID: 19795970
Inflammatory pseudotumor of the choroid plexus.
Bramwit M, Kalina P, Rustia-Villa M
AJNR Am J Neuroradiol 1997 Aug;18(7):1307-9. PMID: 9282860Free PMC Article
Atypical monoclonal plasma cell hyperplasia: its identity and treatment. Case report.
Hsiang JN, Ng HK, Poon WS
J Neurosurg 1996 Oct;85(4):697-700. doi: 10.3171/jns.1996.85.4.0697. PMID: 8814179

Clinical prediction guides

Distinct fibroblast subpopulations associated with bone, brain or intrapulmonary metastasis in advanced non-small-cell lung cancer.
Xu K, Wang H, Zou YX, Zhang HH, Wang YN, Ren XR, Wang HQ, Xu YH, Li JJ, Tang H, He C, Wei S, Tian T, Li LL, Zhou H, Xu LJ, Fang JW, Guo C, Yang JX, Zhou YY, Zhang ZH, Pan YY
Clin Transl Med 2024 Mar;14(3):e1605. doi: 10.1002/ctm2.1605. PMID: 38445456Free PMC Article
Advanced MRI features of intraventricular inflammatory myofibroblastic tumor: a case report.
Tang XL, Huang WJ, Ma Q, Yi KM
BMC Neurol 2022 Dec 2;22(1):448. doi: 10.1186/s12883-022-02993-8. PMID: 36460992Free PMC Article
Intracranial Inflammatory Myofibroblastic Tumor with Negative Expression of Anaplastic Lymphoma Kinase: A Case Report and Review of the Literature.
Wang X, Chen Y, Wu X, Zhang H
World Neurosurg 2019 May;125:117-122. Epub 2019 Feb 5 doi: 10.1016/j.wneu.2019.01.155. PMID: 30735872
Inflammatory pseudotumors of the central nervous system: report of 3 cases and a literature review.
Häusler M, Schaade L, Ramaekers VT, Doenges M, Heimann G, Sellhaus B
Hum Pathol 2003 Mar;34(3):253-62. doi: 10.1053/hupa.2003.35. PMID: 12673560
Plasma cell granuloma extending from the extracranial to the intracranial space associated with Epstein-Barr virus infection.
Fukunaga A, Yoshida K, Otani M, Ogawa Y, Horiguchi T, Ishihara M, Toya S, Kawase T
Neurol Med Chir (Tokyo) 1998 May;38(5):292-6. doi: 10.2176/nmc.38.292. PMID: 9640966

Recent systematic reviews

Management Strategies of Plasma Cell Granuloma Involving the Central Nervous System: A Systematic Review of the Literature.
Sreenivasan S, Agarwal N, Bharath Raju, Kandregula S, Narayan V, Chen CC, Sharma M
World Neurosurg 2023 Dec;180:194-202.e11. Epub 2023 Sep 13 doi: 10.1016/j.wneu.2023.09.026. PMID: 37708970

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