Autopsy and Case Reports
Autopsy and Case Reports
Clinical Case Report and Review

Intracranial Inflammatory Myofibroblastic Tumor: A Review of 49 cases

Deepika Phogat; S.G.S Datta; Mukul Bajpai; Swayam Tara; Sunil Kumar Ganti

Downloads: 0
Views: 59


ABSTRACT: Inflammatory Myofibroblastic Tumor (IMT) is a rare pathologic entity that was first described in 1973. This lesion is most commonly found in the lungs, but other organs’ involvement has also been reported. Intracranial location of Inflammatory Myofibroblastic Tumor is rare, and the first case was reported in 1980. An intriguing fact about the intracranial IMT is its resemblance with meningioma on clinical presentation and neuroimaging. We came across a case of intracranial Inflammatory Myofibroblastic Tumor (IIMT) in a 27-year-old male who presented with recurrent episodes of seizures and was diagnosed as meningioma on neuroimaging. The lesion did not subside with medical management and kept on progressing in size. The patient had to undergo surgery, and diagnosis of Inflammatory Myofibroblastic Tumor was ascertained on histopathology. This ‘surprise’ diagnosis prompted us to review the literature on all cases of IIMTs reported to date to better understand the entity and its implications. In this review article, we present our observations regarding various studied parameters, including patient profile, clinical presentation, site of involvement, focality of the lesion, special associations, and lines of management of the 49 published cases of IIMTs.


Granuloma, Plasma Cell, Meningioma, Seizures, Magnetic Resonance Imaging, Meningeal Neoplasms


1 Bahadori M, Liebow A. Inflammatory Myofibroblastic Tumors of the lung. Cancer. 1973;31(1):191-208.<191::AID-CNCR2820310127>3.0.CO;2-D. PMid:4683037.

2 West S, Pittman D, Coggin J. Intracranial Inflammatory Myofibroblastic Tumor. Cancer. 1980;46(2):330-5.<330::AID-CNCR2820460220>3.0.CO;2-M. PMid:7388775.

3 Kılınç M, Ertürk İÖ, Uysal H, Birler K, Evrenkaya T, Akkalyoncu B. Multiple Inflammatory Myofibroblastic Tumor of the central nervous system: A unique case with brain and spinal cord involvement. Case report and review of literature. Spinal Cord. 2002;40(4):203-6. PMid:11965561.

4 Güdük M, Yener U, Sav A, Pamir MN. Intracranial multifocal Inflammatory Myofibroblastic Tumor: a case with multiple operations without recurrence of surgically removed lesions. Acta Neurochir (Wien). 2016;158(4):721-3. PMid:26852033.

5 Figarella-Branger D, Gambarelli D, Perez-Castillo M, Garbe L, Grisoli F. Primary Intracerebral Inflammatory Myofibroblastic Tumor: A Light, Immunocytochemical, and ultrastructural study of one case. Neurosurgery. 1990;27(1):142-7. PMid:2198484.

6 Fukunaga A, Yoshida K, Otani M, et al. Inflammatory Myofibroblastic Tumor extending from the extracranial to the intracranial space associated with Epstein-Barr virus infection — Case report. Neurol Med Chir (Tokyo). 1998;38(5):292-6. PMid:9640966.

7 Cannella D, Prezyna A, Kapp J. Primary intracranial plasma-cell granuloma. J Neurosurg. 1988;69(5):785-8. PMid:3183738.

8 Gangemi M, Maiuri F, Giamundo A, Donati P, De Chiara A. Intracranial Inflammatory Myofibroblastic Tumor. Neurosurgery. 1989;24(4):591-95.

9 Maeda Y, Tani E, Nakano M, Matsumoto T. Plasma-cell granuloma of the fourth ventricle. J Neurosurg. 1984;60(6):1291-6. PMid:6374065.

10 Gochman GA, Duffy K, Crandall PH, Vinters HV. Inflammatory Myofibroblastic Tumor of the brain. Surg Neurol. 1990;33(5):347-52. PMid:2330536.

11 Makino K, Murakami M, Kitano I, Ushio Y. Primary intracranial plasma-cell granuloma: A case report and review of the literature. Surg Neurol. 1995;43(4):374-8. PMid:7792709.

12 Le Marc’hadour F, Fransen P, Labat-Moleur F, Passagia JG, Pasquier B. Intracranial Inflammatory Myofibroblastic Tumor: A report of four cases. Surg Neurol. 1994;42(6):481-8. PMid:7825102.

13 Breidahl W, Robbins P, Ives F, Wong G. Intracranial Inflammatory Myofibroblastic Tumor. Neuroradiology. 1996;38(S1):S86-9. PMid:8811690.

14 Dettmeyer R, Varchmin-Schultheiß K, Madea B. Intracranial Inflammatory Myofibroblastic Tumor and Homocystinuria. Pathol Res Pract. 1998;194(3):205-8. PMid:9587941.

15 Saxena A, Sinha S, Tatke M. Intracranial Inflammatory Myofibroblastic Tumor ‘; a case report and review of the literature. Br J Neurosurg. 2000;14(5):492-5. PMid:11198780.

16 Tekkök I, Ventureyra E, Jimenez C. Intracranial Inflammatory Myofibroblastic Tumor. Brain Tumor Pathol. 2000;17(3):97-103. PMid:11310925.

17 Murakami K, Muraishi K, Ikeda H, Yoshimoto T. Inflammatory Myofibroblastic Tumor of the pituitary gland. Surg Neurol. 2001;56(4):247-51. PMid:11738674.

18 Lyo I, Suh J, Kwon Y. Intracranial Inflammatory Myofibroblastic Tumor: A Case Report. J Korean Neurosurg Soc. 2001;30(6):774-6.

19 Greiner C, Rickert CH, Möllmann FT, et al. Inflammatory Myofibroblastic Tumor involving the brain and the lung. Acta Neurochir (Wien). 2003;145(12):1127-31. PMid:14663572.

20 Choi CY, Whang CJ, Park SH. Intracranial Inflammatory Myofibroblastic Tumor. J Korean Neurosurg Soc. 2003;33:593-5.

21 Brandsma D, Jansen G, Spliet W, van Nielen K, Taphoorn M. The diagnostic difficulties of meningeal and intracerebral Inflammatory Myofibroblastic Tumors - Presentation of three cases. J Neurol. 2003;250(11):1302-6. PMid:14648145.

22 Murakami M, Hashimoto N, Kimura S, Hosokawa Y, Kakita K. Intracranial Inflammatory Myofibroblastic Tumor with genetic analysis. Acta Neurochir (Wien). 2003;145(3):221-5. PMid:12632119.

23 Buccoliero A, Caldarella A, Santucci M, Ammannati F, Mennonna P, Taddei A. Inflammatory Myofibroblastic Tumor—An enigmatic lesion: Description of an extensive intracranial case and review of the literature. Arch Pathol Lab Med. 2003;127(4):220-3. PMid:12683907.

24 Roche P-H, Figarella-Branger D, Pellet W. Mixed meningeal and brain plasma-cell granuloma: an example of an unusual evolution. Acta Neurochir (Wien). 2004;146(1):69-72. PMid:14740268.

25 Özüm Ü, Özer H, Karadağ Ö, Polat N. Intracranial plasma cell-granuloma with extensive ossification. Br J Neurosurg. 2006;20(3):153-6. PMid:16801048.

26 Nawashiro H, Omura T, Kobayashi H. Cystic Intracranial Inflammatory Myofibroblastic Tumor. J Neurosurg. 2006;105(5):799. PMid:17121152.

27 Sato K, Kubota T, Kitai R, Miyamori I. Meningeal Inflammatory Myofibroblastic Tumor with relapsing polychondritis. J Neurosurg. 2006;104(1):143-6. PMid:16509158.

28 Flannery T, Al-Sabah F, Bhangu J, Alderazi Y, Brett F, Pidgeon C. Treatment of subtotally resected intracranial Inflammatory Myofibroblastic Tumor with steroids: a case report. Br J Neurosurg. 2007;21(5):501-3. PMid:17852108.

29 Kim D-J, Choi Y-S, Song Y-J, Kim K-U. Intracranial Inflammatory Myofibroblastic Tumor. J Korean Neurosurg Soc. 2009;46(2):161. PMid:19763220.

30 Chen D, Liu L, Qiu L. The MRI Misdiagnosis Analysis of Intracranial Inflammatory Myofibroblastic Tumor (4 Cases Report and Literature Review). Journal of Clinical Radiology. 2009;(3):42.

31 Agarwal A, Aronov R, Agarwal K, Lee HK, Zak I, Kish K. Inflammatory Myofibroblastic Tumor of the brain: an unusual case. Eur J Radiol Extra. 2010;74(2):e23-5.

32 Cao X, Luan S, Sun L, Yang B, Shen C, Bao W. Impaired vision associated with a solitary intracranial plasmacytoma. J Clin Neurosci. 2010;17(9):1215-7. PMid:20570518.

33 Brito C, Lopes F, Chimelli L, Gasparetto E. Intracranial cell plasma granuloma. Arq Neuropsiquiatr. 2010;68(1):127-9. PMid:20339667.

34 Puntambekar P, Santhakumar S, Kupsky WJ, Tselis A, Mittal S. Primary intracranial Inflammatory Myofibroblastic Tumors presenting as malignant neoplasms. J Neurooncol. 2011;106(2):327-37. PMid:21786175.

35 Schneider C, Henning T, Fink G, Schroeter M, Lehmann H. Primary intracranial Inflammatory Myofibroblastic Tumor responsive to rituximab. Neurology. 2014;83(12):1119-20. PMid:25107881.

36 Renfrow JJ, Mitchell JW, Goodman M, et al. Relapsing intracranial Inflammatory Myofibroblastic Tumor: A case report. Oncol Lett. 2013;7(2):531-3. PMid:24396482.

37 Forcucci J, Butler-Williams S, Miller N, Lazarchick J. Inflammatory Myofibroblastic Tumor: An n9entity within the spectrum of IgG4-related disease. Ann Clin Lab Sci. 2015;45(3):340-3. PMid:26116600.

38 Bibars W, Singh H, Zbytek B. Inflammatory Myofibroblastic Tumor in Cerebellum: A case report. Am. J. Clin. Pathol. 2016 Sep;146(1 Suppl).

39 Gautam S, Ramesh V, Karthikeyan K, Krishnakumar M. Intracranial inflammatory pseudotumor presenting as an en plaque mass. Neurol India. 2017;65(5):1169-70. PMid:28879929.

40 Shah M, McClain K. Intracranial Inflammatory Myofibroblastic Tumor. J Pediatr Hematol Oncol. 2005;27(11):599-603. PMid:16282891.

Submitted date:

Accepted date:

Publication date:

604b6285a95395300157bdf2 autopsy Articles
Links & Downloads

Autops Case Rep

Share this page
Page Sections