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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
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
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
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

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
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
Intradural extramedullary spinal inflammatory myofibroblastic tumor: case report and literature review.
Zemmoura I, Hamlat A, Morandi X
Eur Spine J 2011 Jul;20 Suppl 2(Suppl 2):S330-5. Epub 2011 Apr 6 doi: 10.1007/s00586-011-1783-9. PMID: 21465290Free PMC Article
Inflammatory myofibroblastic tumor of the cerebellar hemisphere--case report.
Kato K, Moteki Y, Nakagawa M, Kadoyama S, Ujiie H
Neurol Med Chir (Tokyo) 2011;51(1):79-81. doi: 10.2176/nmc.51.79. PMID: 21273753
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
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
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

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
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
Inflammatory myofibroblastic tumor involving lung and brain in a 10-year-old boy: a case report.
Sharma S, Sankhyan N, Kalra V, Garg A, Gupta SD, Agarwala S, Das P
J Child Neurol 2009 Oct;24(10):1302-6. doi: 10.1177/0883073809341270. PMID: 19805826
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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