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Demyelinating Pseudotumor Cerebri: A Case Report

2003; King Faisal Specialist Hospital and Research Centre; Volume: 23; Issue: 5 Linguagem: Inglês

10.5144/0256-4947.2003.291

0975-4466

Abdülkadir Reis, Ertuğrul Çakır, Ümit Çobanoğlu, Haydar Usul, Kayhan Kuzeyli̇,

Cerebral Venous Sinus Thrombosis

Case ReportsDemyelinating Pseudotumor Cerebri: A Case Report Abdulkadir Reis, MD Ertugrul Cakir, MD Umit Cobanoglu, MS Haydar Usul, and MD Kayhan KuzeyliMD Abdulkadir Reis Correspondence to: Dr. A. Reis, KTU Medical Faculty, Department of Pathology, 61080 Trabzon, Turkey From the Department of Pathology and Neurosurgery, Karadeniz Technical University School of Medicine, Turke Search for more papers by this author , Ertugrul Cakir From the Department of Pathology and Neurosurgery, Karadeniz Technical University School of Medicine, Turke Search for more papers by this author , Umit Cobanoglu From the Department of Pathology and Neurosurgery, Karadeniz Technical University School of Medicine, Turke Search for more papers by this author , Haydar Usul From the Department of Pathology and Neurosurgery, Karadeniz Technical University School of Medicine, Turke Search for more papers by this author , and Kayhan Kuzeyli From the Department of Pathology and Neurosurgery, Karadeniz Technical University School of Medicine, Turke Search for more papers by this author Published Online::1 Sep 2003https://doi.org/10.5144/0256-4947.2003.291SectionsPDF ToolsAdd to favoritesDownload citationTrack citations ShareShare onFacebookTwitterLinked InRedditEmail AboutIntroductionDemyelinating pseudotumors are non-neoplastic lesions mostly misinterpreted on biopsy as gliomas, diffuse fibrilliary astrocytomas or rarely oligodendrogliomas.1 Affected tissues show diffuse infiltration by foamy, lipid laden macrophages, reactive astocytosis of variable intensity, and perivascular aggregates of small lymphocytes and occasional plasma cells.1-3Clinical presentations as well as radiological and histopathological findings in biopsies from patients with multiple sclerosis (MS) or other demyelinating disorders of the central nervous system (CNS) are sometimes misleading, resulting in an erroneous diagnosis of brain or spinal cord tumor.2 The idiopathic demyelinating disease of the CNS, of which MS is by far the most common, are usually regarded as “medical” disorders diagnosed on clinical grounds or at autopsy.1Beginning in the late 1970s and early 1980s, several reports had been published of large demyelinating lesions that simulated brain tumors clinically and radiologically.4-9CASE REPORTA 42-year-old patient admitted with ataxia and seizure. He had a history of ataxia for two years and he had seizures for three months. There was no history of preceding viral infection or immunization. The patient’s physical examination and medical history were unremarkable. Neurological examination disclosed tetraparesia, which was prominent at the lower extremities. The patient had 4/5 motor strength at the upper extremities and 2/5 motor strength at the lower extremities. Deep tendon reflexes were increased at all extremities. Babinski and Hoffman signs were positive bilaterally. Whole blood count and biochemical analysis of the serum were within normal limits. Cerebrospinal fluid examination was not performed. Magnetic resonance imaging (MRI) showed hypointense lesions in the right basal ganglia, right capsula interna, and the right temporal, parietal and bilateral frontal lobes (Figure la). T2 weighted images showed hyperintense lesions in deep and subcortical white matter. The lesion showed heterogeneous contrast enhancement (Figure lb).Figure 1. (A) Contrast enhanced T1 -weighted coronal MR image showing a mass lesion invading the deep and subcortical white matter, and the corpus callosum. The lesion shows prominent contrast enhancement. Note the little mass effect of the lesion when coDownload FigureA right parietal craniotomy was performed and several biopsies from the lesion obtained. The histopathological examination was consistent with demyelinating pseudotumor cerebri. No pathological signal intensity was detected on spinal MRI scans. The patient died four months after the biopsy due to pulmonary infection.After fixation in 10% buffered formalin and paraffin embedding, 5 to 6 micrometer thick sections were stained with hematoxylin and eosin (H&E). H&E stained sections revealed reactive hyperthrophied astrocytes with large eosinophilic cytoplasm, some multinucleated and infiltrates of foamy cells (lipid-filled macrophages) and perivascular lymphoplasmocytic infiltrates (Figure 2). Glial fibrillary acidic protein (GFAP) for astrocytes and alpha-1 antichymotripsine (Al ACT) for macrophages were used for immunohistochemical study. Positive immunoreactivity by GFAP in reactive hyperplastic astrocytes were found (Figure 3a), and macrophages were stained in A1ACT (Figure 3b).Figure 2. Glial reaction, foamy cells infiltration and perivascular mononuclear inflammatory infiltration in demyelinating lesion (H&E × 200).Download FigureFigure 3A. Immunohistochemical GFAP positivity in reactive astrocytes (GFAP immunoperoxidase).Download FigureFigure 3B. Immunohistochemical A1ACT positivity in macrophages (A1ACT immunoperoxidase).Download FigureDISCUSSIONThere is a spectrum of various stages in demyelinating diseases. Lesions are usually classified as active or inactive lesions based on the presence or absence of inflammation or macrophage infiltration. Active lesions usually show an enhancing character.3 Active enhancing lesions are more easily misinterpreted as a neoplasm. Frozen sections are especially difficult to analyse in regard to whether they are active or inactive. In our case, the frozen diagnosis was astrocytoma. Regular distribution of the reactive astrocytes and demonstration with oil-red-O staining of macrophages containing neutral lipid are helpful. Several of these cases were initially interpreted as astrocytomas or infectious processes. In a series with 17 cases, Zagzag et al described some histological features that may indicate that a clinically diagnosed “tumor” is not a neoplasm.2 The presence of infiltrating foamy macrophages and a pattern of sheets of well formed, often large, gemiocytic. astrocytes are more suggestive of reactive gliosis than of a neoplasm. The presence of a prominent inflammatory infiltrate, especially a mixture of lymphocytes and plasma cells, favor the diagnosis of demyelination.In our case, these cells were identified as macrophages and not as astrocytes following immunostaining with A1ACT.11 The reactive astrocytes had a suggestive “spidery” appearance on a GFAP immunostain. Gliosis is a known feature of multiple sclerosis.2Demyelinating lesions usually have well defined borders, while tumors do not.2 These clues should alert the pathologist to the possibility of multiple sclerosis or another demyelinating process.Thirty-one patients with large, focal cerebral demyelinating lesions were reported by Kepes.4 The lesions presented clinically and radiologically as brain tumors (gliomas or metastases) or as multiple cysts. The field of demyelinating disease of the CNS encompasses numerous entities, with MS being the most common and best known among them. In that disease, multiple demyelinating lesions separated from each other in space and time, preferentially involve certain areas of the CNS, such as the periventricular white matter, the matter optic system, the cerebellum, the brainstem and the spinal cord.4All three types of diffuse demyelinating diseases described by Schilder have been considered in some leading textbooks as relentlessly progressive childhood diseases,12,13 although clinical improvement has also been reported.14 Progressive multifocal leukoencephalopathy constitutes another form of demyelination that may present with very large foci, but it has a well-established viral cause and a distinct histopathological appearance.Still another group of acquired demyelinating diseases, post-infectious and postvaccination encephalomyelites, are regarded as an immunologically mediated form of myelin destruction manifested by perivenous lesions usually of microscopic size, although some confluence of lesions is possible. In our case, there was no history of preceding viral infection or immunization.ARTICLE REFERENCES:1. Rosai J. Ackerman’s Surgical Pathology., Eight edition. Mosby: St. Louis; 1995. Google Scholar2. Zagzag D, Miller DC, Kleinman GM, Abati A, Donnenfield H, Budzilovich GN. "Demyelinating disease versus tumor in surgical neuropathology. Clues to a correct pathological diagnosis." Am J Surg Pathol.. 1993; 17(6):537-545. Google Scholar3. Nesbit GM, Forbes GS, Scheithauer BW, Okazaki H, Rodriguez M. "Multiple Sclerosis:Histopathologic and/or MR correlation in 37 cases at biopsy and three cases at autopsy." Radiology.. 1991; 180:467-474. Google Scholar4. Kepes JJ. "Large focal tumor-like demyelinating lesions of the brain: intermediate entity between multiple sclerosis and acute disseminated encephalomyelitis? A study of 31 patients." Ann Neurol.. 1993; 33:18-27. Google Scholar5. Van der Velden M, Bors GTAM, Endtz LJ. "Cranial CT in multiple sclerosis showing a mass effect." 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Am J Surg Pathol.. 1992; 16:493-499. Google Scholar12. Merritt HH. A textbook of neurology. 5th ed. Philadelphia: Lea and Febiger; 1973. Google Scholar13. Poirier J, Gray F, Escourolle R. Manual of basic neuropathology. Philadelphia: WB Saunders; 1990:132. Google Scholar14. Ellison PH, Barron KD. "Clinical recovery from Schilder disease." Neurology.. 1979; 29:244-251. Google Scholar Previous article Next article FiguresReferencesRelatedDetails Volume 23, Issue 5September-October 2003 Metrics History Accepted1 January 2003Published online1 September 2003 InformationCopyright © 2003, Annals of Saudi MedicinePDF download