- Case report
- Open Access
Primary Inflammatory demyelinating pseudotumor in the left frontal lobe with meningeal involvement presenting as malignant neoplasms
© Ma et al. 2016
- Received: 2 July 2015
- Accepted: 20 October 2015
- Published: 18 February 2016
Tumor-like demyelinating lesion is a rare form of IP, often indistinguishable from brain tumors on CT or MR imaging.
We report here a case of pathologically confirmed tumor-like demyelinating lesions. The clinical manifestations, image feature and pathology of this case are also discussed together with a review of the relevant literature.
The correct diagnosis of tumor-like demyelinating lesion mainly depends on the postsurgical histopathologic examinations. We suggest complete resection of the intracranial lesion and involved dura, followed by administration of short term oral prednisolone therapy will be good for preventing recurrence.
- Inflammatory pseudotumors
- Demyelinating lesion
- Steroid therapy
Inflammatory pseudotumor (IP) belongs to heterogenous disease with unclear pathogenesis and nomenclature. It was first described in the lung as a lesion of plasma cell granuloma in 1973 by Bahadori and Liebow . Intracranial involvement of IP was not reported until 1980 . The central nervous system is extremely rare to be invaded primarily, only fewer than 80 cases have been described [3–5]. The occurrence of this lesion in intracranial location is still infrequent. Tumor-like demyelinating lesion is a rare form of IP, which is hard to be distinguished from brain tumors by CT or MR imaging . Here, we report a case of pathologically confirmed tumor-like demyelinating lesion. The clinical manifestations, image features and pathology of this case are also discussed together with a review of the relevant literature.
The patient was in good condition with no neurologic deficits after surgical intervention. Low-dose steroid treatment with oral administration of methylprednisolone (40 mg, bid) was continued as well. A follow-up enhanced CT scans four weeks after surgery showed the mass lesion was absence and surrounding edema was much smaller. Repeat MR imaging three months after the corticosteroid therapy revealed the absence of gadolinium enhancement and a decrease in the size of edema region (Fig. 1g, h, i). There was no recurrence after 6 months.
IP of the CNS is a pathologic term that describes a reactive, inflammatory nonmalignant phenomenon that clinically and radiographically mimics a neoplasm in the brain. It was originally described as a nonneoplastic process and reported under different names including: inflammatory myofibroblastic tumor, plasma cell granuloma, xanthomatous pseudotumor, pseudosarcomatous, fibromyxoid tumor and inflammatory myofibrohistiocytic proliferation in the literature subsequently [7, 8]. IP primarily affecting the central nervous system is rare. In most cases, the etiology of IP often is uncertain although several subset lesions are inflammatory and associated with a variety of infectious agents and may be caused by autoimmune disorders [9, 10]. In addition, demyelinating diseases presented with tumor-like lesions, such as multiple sclerosis, acute disseminated encephalomyelitis and myelinoclastic diffuse sclerosis had also been reported in the literature [11, 12]. The image features of IP may be as below: First, most IP exhibited some degree of enhancement. Second, some IP contained some degree of infiltrative features such as bone erosion and invasion of surrounding tissues. As reported by Ginat et al, calcifications were indentified in the dural IP. Pathological examination can find inflammatory cells, such as lymphocytes, plasma cells, macrohpages, but evidence of mitosis, anplasia or necrosis.
Our case, by contrast, presented with no history of virus or bacteria infection. The mass lesion was located in the left frontal lobe, showed heterogeneous enhancement on MR images and infiltrative to the surrounding brain parenchyma with mass effect and severe brain edema. It resembled an atypical brain tumor in appearance of radiology and did not have a preference for periventricular area which is predilection for demyelinating diseases. Therefore, it did not quite match the diagnostic criteria of demyelinating lesion such as multiple sclerosis, acute disseminated encephalomyelitis or myelinoclastic diffuse sclerosis. As far as we know, such a case was conventionally termed tumor-like demyelinating lesions or monofocal acute inflammatory demyelination in the previous reports . Actually, the present case was similar to the published cases with tumor-like demyelinating lesions and accompanied by meningeal involvement which was evidently demonstrated on MR images. We speculate that the same radiologic appearance may contribute to the serviceability of image diagnosis for tumor-like demyelinating lesion .
Tumor-like demyelinating lesion is a rare form of intracranial IP and the correct diagnosis mainly depends on the postsurgical histopathologic examinations due to the rarity . On the other hand, immunohistochemical staining plays an important role in the differential diagnosis. Usually, CD34 positivity was considered as a typical distinguishment between the neoplastic and nonneoplastic groups. In this case, positive immunostaining confirmed the presence of lymphocytes (by CD3, CD20 and CD79), macrophages or plasma cells (by CD163, CD68), and meanwhile, negative immunostaining for S-100, GFAP, vimentin and EMA eliminated the diagnosis of neoplasm resembled such as plasmacytoma, lymphoma, lymphoplasma cell-rich meningioma, and histiocytosis. A final diagnosis of primary IP of tumor-like demyelinating lesion was made based on the histopathological features, immunohistochemical studies and radiographical manifestations.
For the treatment options of primary intracranial IP of tumor-like demyelinating lesion, there remains no consensus regarding schedules in the literature . As is known to all, despite their aggressive appearance, they may respond well to steroid treatment. So, we suggest complete resection of the intracranial lesion and involved dura, if possible, followed by administration of short term oral prednisolone therapy will be good for preventing recurrence.
Open AccessThis article is distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated.
- Bahadori M, Liebow AA. Plasma cell granulomas of the lung. Cancer. 1973;31(1):191–208.View ArticlePubMedGoogle Scholar
- West SG, Pittman DL, Coggin JT. Intracranial plasma cell granuloma. Cancer. 1980;46(2):330–5.View ArticlePubMedGoogle Scholar
- Häusler M, Schaade L, Ramaekers VT, Doenges M, Heimann G, Sellhaus B. Inflammatory pseudotumors of the central nervous system: report of 3 cases and a literature review. Hum Pathol. 2003;34(1):253–62.View ArticlePubMedGoogle Scholar
- Saxena A, Sinha S, Tatke M. Intracranial plasma cell granuloma - a case report and review of the literature. Br J Neurosurg. 2000;14(5):492–5.View ArticlePubMedGoogle Scholar
- Gandhi RH, Li L, Qian J, Kuo YH. Intraventricular inflammatory pseudotumor: Report of two cases and review of the literature. Neuropathology. 2011;31(4):446–54.View ArticlePubMedGoogle Scholar
- Ginat DT, Bokhari A, Bhatt S, Dogra V. Inflammatory pseudotumors of the head and neck in pathology-proven cases. J Neuroradiology. 2012;39(2):110–5.View ArticleGoogle Scholar
- Lui PC, Fan YS, Wong SS, Chan AN, Wong G, Chau TK, et al. Inflammatory pseudotumors of the central nervous system. Hum Pathol. 2009;40(11):1611–7.View ArticlePubMedGoogle Scholar
- Badalian-Very G, Vergilio JA, Degar BA, Rodriguez-Galindo C, Rollins BJ. Recent advances in the understanding of Langerhans cell histiocytosis. Br J Haematology. 2012;156(2):163–72.View ArticleGoogle Scholar
- Saab ST, Hornick JL, Fletcher CD, Olson SJ, Coffin CM. IgG4 plasma cells in inflammatory myofibroblastic tumor. Mod Pathol. 2011;24(2):606–12.View ArticlePubMedGoogle Scholar
- Gomez FP, Steelman AJ, Young CR, Welsh CJ. Hormone and immune system interactions in demyelinating disease. Horm Behav. 2013;63(2):315–21.View ArticlePubMedGoogle Scholar
- Caroli E, Salvati M, Ferrante L. Tumor-like multiple sclerosis: Report of four cases and literature review. Tumori. 2006;92(6):559–62.PubMedGoogle Scholar
- Turatti M, Gajofatto A, Bianchi MR, Ferrari S, Monaco S, Benedetti MD. Benign course of tumour-like multiple sclerosis. Report of five cases and literature review. J Neurol Sci. 2013;324(2):156–62.View ArticlePubMedGoogle Scholar
- Kimura N, Kumamoto T, Hanaoka T, Hasama Y, Nakamura K, Okazaki T. Monofocal large inflammatory demyelinating lesion, mimicking brain glioma. Clin Neurol Neurosurg. 2009;111(10):296–9.View ArticlePubMedGoogle Scholar
- Puntambekar P, Santhakumar S, Kupsky WJ, Tselis A, Mittal S. Primary intracranial plasma cell granulomas presenting as malignant neoplasms. J Neurooncol. 2012;106(7):327–37.View ArticlePubMedGoogle Scholar
- Vedeler CA, Farbu E, Mellgren SI. Chronic inflammatory demyelinating polyneuropathy. Acta Neurol Scand. 2013;127(Suppl 196):48–51.View ArticleGoogle Scholar
- Flannery T, Al-Sabah F, Bhangu J, Alderazi Y, Brett F, Pidgeon C. Treatment of subtotally resected intracranial plasma cell granuloma with steroids: a case report. Br J Neurosurg. 2007;21(5):501–3.View ArticlePubMedGoogle Scholar