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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
Meningeal Rosai-Dorfman disease mimicking meningioma.
Forest F, N'guyen AT, Fesselet J, Metellus P, Bouvier C, de Paula AM, Roche PH, Figarella-Branger D
Ann Hematol 2014 Jun;93(6):937-40. Epub 2013 Dec 24 doi: 10.1007/s00277-013-1994-8. PMID: 24366336
Inflammatory myofibroblastic tumor of the central nervous system and its relationship to inflammatory pseudotumor.
Swain RS, Tihan T, Horvai AE, Di Vizio D, Loda M, Burger PC, Scheithauer BW, Kim GE
Hum Pathol 2008 Mar;39(3):410-9. doi: 10.1016/j.humpath.2007.07.012. PMID: 18261625

Diagnosis

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
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
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
Meningeal Rosai-Dorfman disease mimicking meningioma.
Forest F, N'guyen AT, Fesselet J, Metellus P, Bouvier C, de Paula AM, Roche PH, Figarella-Branger D
Ann Hematol 2014 Jun;93(6):937-40. Epub 2013 Dec 24 doi: 10.1007/s00277-013-1994-8. PMID: 24366336
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

Treatment of intracranial inflammatory myofibroblastic tumor with PD-L1 inhibitor and novel oncolytic adenovirus Ad-TD-nsIL12: a case report and literature review.
Qian X, Ning W, Dunmall LC, Qu Y, Wang Y, Zhang H
Front Immunol 2024;15:1427554. Epub 2024 Jul 24 doi: 10.3389/fimmu.2024.1427554. PMID: 39114662Free PMC Article
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
Granulomatosis with polyangiitis presenting as a renal tumor.
Ward A, Konya C, Mark EJ, Rosen S
Am J Surg Pathol 2014 Oct;38(10):1444-8. doi: 10.1097/PAS.0000000000000294. PMID: 25025447
IgG4-related inflammatory pseudotumor of the central nervous system responsive to mycophenolate mofetil.
Moss HE, Mejico LJ, de la Roza G, Coyne TM, Galetta SL, Liu GT
J Neurol Sci 2012 Jul 15;318(1-2):31-5. Epub 2012 Apr 28 doi: 10.1016/j.jns.2012.04.010. PMID: 22546342Free PMC Article
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

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
Pandora's box: eosinophilic granuloma at the cerebellopontine angle-should we open it?
Öğrenci A, Batçık OE, Ekşi MŞ, Koban O
Childs Nerv Syst 2016 Aug;32(8):1513-6. Epub 2015 Dec 12 doi: 10.1007/s00381-015-2982-1. PMID: 26661575
Recurrent meningeal inflammatory myofibroblastic tumor: a case report and literature review.
Pascual-Gallego M, Yus-Fuertes M, Jorquera M, Gonzalez-Mat A, Ortega L, Martinez-Martinez A, Zimman H
Neurol India 2013 Nov-Dec;61(6):644-9. doi: 10.4103/0028-3886.125273. PMID: 24441334
Posterior fossa intracranial inflammatory pseudotumor: a case report and literature review.
Lin YJ, Yang TM, Lin JW, Song MZ, Lee TC
Surg Neurol 2009 Dec;72(6):712-6; discussion 716. Epub 2009 Jul 15 doi: 10.1016/j.surneu.2009.01.029. PMID: 19608242

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
Intracranial inflammatory pseudotumor: case report and review of the literature.
Yavuzer D, Dalbayrak S, Oz B, Yilmaz M, Akansel G
Clin Neuropathol 2010 May-Jun;29(3):151-5. doi: 10.5414/npp29151. PMID: 20423689
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

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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