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    "textoCompleto" => "<span class="elsevierStyleSections"><span id="sec0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0030">Introduction</span><p id="par0005" class="elsevierStylePara elsevierViewall">Inflammatory pseudotumour is a relatively rare benign process in the CNS&#44; with an incidence of 4&#8211;8&#37; of inflammatory nervous system lesions&#46;<a class="elsevierStyleCrossRef" href="#bib0005"><span class="elsevierStyleSup">1</span></a> Its clinical presentation and radiological morphology mimics a meningioma&#46;<a class="elsevierStyleCrossRef" href="#bib0005"><span class="elsevierStyleSup">1</span></a></p><p id="par0010" class="elsevierStylePara elsevierViewall">Surgery is the treatment of choice&#44; although its management has currently not yet been established&#46;</p><p id="par0015" class="elsevierStylePara elsevierViewall">The term inflammatory myofibroblastic tumour &#40;IMT&#41; was originally used as a synonym of IP&#46; However&#44; recent studies have documented that the two processes have a different aetiology&#44; behaviour and prognosis&#46;<a class="elsevierStyleCrossRef" href="#bib0010"><span class="elsevierStyleSup">2</span></a></p><p id="par0020" class="elsevierStylePara elsevierViewall">The aim of this article is to help establish&#44; via a case report&#44; some key concepts that may help characterise this disease&#46;</p></span><span id="sec0010" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0035">Case report</span><p id="par0025" class="elsevierStylePara elsevierViewall">We present the case of a 31-year-old Senegalese man&#44; with a history of epilepsy secondary to TBI&#44; for whom we have no prior medical reports&#44; who presented with new epileptic seizures&#46; Initial routine tests &#40;full blood count&#44; viral serology&#44; urine test&#44; ECG&#44; chest X-ray and abdominal ultrasound&#41; were performed and these were all normal&#46; MRI and CT scans of the head&#44; with and without contrast&#44; were then completed&#44; which showed a solid&#44; homogeneously enhancing lesion&#44; measuring 13<span class="elsevierStyleHsp" style=""></span>&#215;<span class="elsevierStyleHsp" style=""></span>3&#46;6<span class="elsevierStyleHsp" style=""></span>&#215;<span class="elsevierStyleHsp" style=""></span>4<span class="elsevierStyleHsp" style=""></span>cm&#44; in the anterior fossa &#40;<a class="elsevierStyleCrossRef" href="#fig0005">Fig&#46; 1</a>&#41; with mass effect next to two post-traumatic frontal porencephalic cavities&#46; The initial clinical and radiological diagnosis was a planum sphenoidale meningioma and therefore surgical resection was determined to be the best course of treatment&#46;</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><p id="par0030" class="elsevierStylePara elsevierViewall">Following a bifrontal craniotomy and cranialisation of both frontal sinuses&#44; an extraparenchymal tumour&#44; which was fibrous in consistency&#44; yellow in colour and adhered to the meninges&#44; was accessed &#40;<a class="elsevierStyleCrossRef" href="#fig0010">Fig&#46; 2</a>&#41;&#46;</p><elsevierMultimedia ident="fig0010"></elsevierMultimedia><p id="par0035" class="elsevierStylePara elsevierViewall">Microscopically&#44; the lesion was a fibrous mass with numerous inflammatory aggregates formed by plasma cells with accompanying Russell bodies &#40;<a class="elsevierStyleCrossRef" href="#fig0015">Fig&#46; 3</a>A and B&#41;&#44; lymphocytes&#44; eosinophils and a few neutrophils&#46; The stroma was predominantly hypocellular&#44; with spindle cells interspersed with a sclerotic collagen bundles &#40;<a class="elsevierStyleCrossRef" href="#fig0015">Fig&#46; 3</a>C&#41; and occasional psammomatous calcifications &#40;<a class="elsevierStyleCrossRef" href="#fig0015">Fig&#46; 3</a>D&#41;&#46; No meningothelial cells&#44; foci of necrosis&#44; mitotic figures or other findings suggestive of malignancy were observed&#46; Immunohistochemistry analysis showed concomitant positive immunoreactivity for CD138 and epithelial membrane antigen &#40;EMA&#41; in the plasma population&#44; with no cells that were positive for EMA only suggestive of meningothelial cells&#46; The &#955; and &#954; light-chain study showed a polyclonal lymphoplasmacytic population with no light-chain restriction and the IgG4&#47;IgG ratio was 5&#8211;10&#37;&#44; depending on the area&#46; Myofibroblastic proliferation showed heterogeneous positivity for smooth muscle actin&#46; Glial fibrillary acidic protein &#40;GFAP&#41;&#44; ALK&#44; PAS and Grocott stains were negative&#46;</p><elsevierMultimedia ident="fig0015"></elsevierMultimedia><p id="par0040" class="elsevierStylePara elsevierViewall">On collating all the data&#44; the lesion was definitely diagnosed as IP&#46;</p><p id="par0045" class="elsevierStylePara elsevierViewall">The patient&#39;s postoperative progress was satisfactory&#46; A postoperative MRI of the head showed no lesion remnants and therefore adjuvant therapy was ruled out&#46; One year after surgery&#44; and after close monitoring&#44; the patient presents no symptoms&#44; clinical or radiological signs of tumour recurrence&#46;</p></span><span id="sec0015" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0040">Discussion</span><p id="par0050" class="elsevierStylePara elsevierViewall">IP is a rare&#44; benign inflammatory lesion of unknown cause that has traditionally been grouped with IMT&#46;<a class="elsevierStyleCrossRef" href="#bib0015"><span class="elsevierStyleSup">3</span></a> However&#44; due to its behaviour and histopathological&#44; immunohistochemical and cytogenetic features&#44; it is currently considered an independent process&#46;<a class="elsevierStyleCrossRefs" href="#bib0010"><span class="elsevierStyleSup">2&#44;4</span></a> For this reason&#44; there are very few published cases of true IP&#46;</p><p id="par0055" class="elsevierStylePara elsevierViewall">Histologically&#44; both IP and IMT show a proliferation of fibroblastic&#44; myofibroblastic and inflammatory cells&#46; IP is characterised by IgG4-positive cells&#46; Up to 70&#37; of IMT cells present translocation of the ALK gene on the short arm of chromosome 2 &#40;2p23&#41;&#46; This mutation is considered an exclusion criterion for IP&#46; With regards to behaviour&#44; IMT has an aggressive progression and is considered a neoplastic process due to its capacity to spread and produce metastasis&#46;<a class="elsevierStyleCrossRef" href="#bib0020"><span class="elsevierStyleSup">4</span></a></p><p id="par0060" class="elsevierStylePara elsevierViewall">In 1980&#44; West et al&#46; published the first case in an intracranial location&#46; The largest published study on inflammatory lesions&#44; including IMT and IP&#44; suggested that these tumours account for 4&#37; of all primary CNS malignancies&#44; with a slightly higher rate in females and a higher frequency in adults&#46;<a class="elsevierStyleCrossRef" href="#bib0025"><span class="elsevierStyleSup">5</span></a> However&#44; these data are notably different from data produced by other studies&#46; More specifically&#44; the most common sites of IP are the lung and eye&#44;<a class="elsevierStyleCrossRef" href="#bib0030"><span class="elsevierStyleSup">6</span></a> while an intracranial IP is extremely rare&#46;<a class="elsevierStyleCrossRef" href="#bib0035"><span class="elsevierStyleSup">7</span></a> Usually&#44; as in our case&#44; it presents as a single&#44; supratentorial&#44; extra-parenchymatous lesion<a class="elsevierStyleCrossRefs" href="#bib0025"><span class="elsevierStyleSup">5&#44;8</span></a> located in the skull base in the anterior cranial fossa&#46;<a class="elsevierStyleCrossRefs" href="#bib0010"><span class="elsevierStyleSup">2&#44;3</span></a> In spinal cases&#44; it is reported more in the cervicodorsal region &#40;2&#37;&#41;&#46;<a class="elsevierStyleCrossRefs" href="#bib0015"><span class="elsevierStyleSup">3&#44;8</span></a></p><p id="par0065" class="elsevierStylePara elsevierViewall">The most common initial symptom is headache &#40;28&#46;9&#37;&#41;&#44; which is associated with dural thickening and intracranial hypertension&#44;<a class="elsevierStyleCrossRefs" href="#bib0030"><span class="elsevierStyleSup">6&#44;7&#44;9</span></a> followed by focal neurological deficits &#40;23&#46;7&#37;&#41; and epileptic seizures &#40;15&#44;8&#37;&#41;&#46; Up to 20&#37; of cases have systemic symptoms<a class="elsevierStyleCrossRef" href="#bib0040"><span class="elsevierStyleSup">8</span></a> and in a significant percentage of cases&#44; the lesion is discovered by accident&#46;<a class="elsevierStyleCrossRef" href="#bib0045"><span class="elsevierStyleSup">9</span></a></p><p id="par0070" class="elsevierStylePara elsevierViewall">The most widely accepted aetiological hypothesis is that it is an IgG4-related inflammatory autoimmune reaction to a stimulus&#44; with a tendency to develop fibrotic tumours with IgG4-rich lymphoplasmacytic cells&#44; although the serum concentration of IgG4 is not always elevated in blood&#46; To diagnose this disease&#44; an IgG4&#47;IgG ratio of &#62;40&#37; in the lesion is essential<a class="elsevierStyleCrossRefs" href="#bib0050"><span class="elsevierStyleSup">10&#44;11</span></a> &#40;in our case&#44; this ratio was less than 10-15&#37;&#41;&#44; with at least one of the main histopathological features in the tumour&#58; lymphoplasmacytic infiltration&#44; fibrosis and&#47;or obliterative phlebitis&#46;<a class="elsevierStyleCrossRef" href="#bib0060"><span class="elsevierStyleSup">12</span></a> It is related to other autoimmune diseases &#40;transverse myelitis&#44; Sjogren&#8217;s syndrome&#44; lupus&#44; Behcet&#8217;s syndrome&#44; sarcoidosis and myasthenia gravis&#41;&#44; demyelinating diseases &#40;neuromyelitis optica and acute demyelinating encephalomyelitis&#41;&#44; chronic infections&#44; caused by viruses &#40;EBV&#44; varicella zoster&#44; JCV&#44; HV-6&#44; HV-8&#41; and fungi&#44; parasites and tuberculosis&#44; and is finally a disproportionate reaction to a repair process following a traumatic brain injury&#44; as occurred in our case&#46;<a class="elsevierStyleCrossRefs" href="#bib0005"><span class="elsevierStyleSup">1&#44;2&#44;5&#44;10</span></a></p><p id="par0075" class="elsevierStylePara elsevierViewall">On a MRI scan of the brain&#44; IP appears as an iso-hypointense lesion compared to the parenchyma on T1 and T2 sequences&#44; with adhesions and&#47;or dural thickening&#46; When contrast is administered&#44; the lesion shows homogeneous and intense enhancement&#44; and if there is also tissue invasion or cortical venous congestion&#44; the parenchyma may appear hyperintense on T2 and FLAIR sequences&#46;<a class="elsevierStyleCrossRef" href="#bib0045"><span class="elsevierStyleSup">9</span></a> On performing a spectroscopy&#44; IP shows lactate peaks directly related to the degree of inflammation&#44; hypoxia and choline peaks with increased cellularity&#46;<a class="elsevierStyleCrossRef" href="#bib0040"><span class="elsevierStyleSup">8</span></a></p><p id="par0080" class="elsevierStylePara elsevierViewall">The main clinical and radiological differential diagnosis is meningioma&#46;<a class="elsevierStyleCrossRef" href="#bib0050"><span class="elsevierStyleSup">10</span></a></p><p id="par0085" class="elsevierStylePara elsevierViewall">The definitive diagnosis of IP is identified by the pathology study&#46; Histologically&#44; IP is a fibrosclerotic mass with several heterogeneous inflammatory foci&#46;<a class="elsevierStyleCrossRef" href="#bib0040"><span class="elsevierStyleSup">8</span></a> Based on these histological features and the intracranial site of our case&#44; the differential diagnosis would mainly include lymphoplasmacyte-rich meningioma&#44; inflammatory myofibroblastic tumour and a lymphoproliferative syndrome&#46; Firstly&#44; our case was particularly difficult to interpret because we observed EMA positivity in the tumour cells&#46; This immunomarker is conventionally used to diagnose meningioma&#46; However&#44; it is non-specific and positive immunoreactivity for EMA has been described in numerous epithelial tumours&#44; and even in reactive lymphoplasmacytic cells&#46;<a class="elsevierStyleCrossRef" href="#bib0065"><span class="elsevierStyleSup">13</span></a> Considering that no meningothelial cells were detected using hematoxylin and eosin staining and EMA-positive cells were also positive for CD138&#44; the plasmacytic nature of these cells was confirmed and the possibility of a meningioma was ruled out&#46; However&#44; inflammatory myofibroblastic tumour tends to paint a similar histological picture and is characterised by ALK gene &#40;2p23&#41; rearrangement and positivity for ALK by immunohistochemistry&#46;<a class="elsevierStyleCrossRefs" href="#bib0040"><span class="elsevierStyleSup">8&#44;13</span></a> Our case&#44; however&#44; was ALK negative&#44; supporting the diagnosis of an IP&#46; Finally&#44; a plasmacytoma or lymphoma was ruled out because there were very few lymphoplasmacytic cells for a lymphoproliferative malignancy and the cells were polyclonal due to not showing kappa or lambda light-chain restriction&#46;</p><p id="par0090" class="elsevierStylePara elsevierViewall">There is no therapeutic protocol&#44; although complete surgical resection is the treatment of choice&#44; provided that the characteristics of the lesion and the patient&#39;s health allow it&#46; The use of corticosteroids is especially recommended in cases of incomplete resection or lesions with an autoimmune&#47;inflammatory origin<a class="elsevierStyleCrossRef" href="#bib0070"><span class="elsevierStyleSup">14</span></a> in which a clear clinical and radiological improvement has been documented&#46; Other uncertain adjuvant therapies include radiotherapy&#44; immunotherapy and chemotherapy&#44;<a class="elsevierStyleCrossRefs" href="#bib0070"><span class="elsevierStyleSup">14&#44;15</span></a> which are reserved for refractory cases&#46;<a class="elsevierStyleCrossRef" href="#bib0045"><span class="elsevierStyleSup">9</span></a> Tumour remnants are associated with a higher risk of progression&#44; which in up to 40&#37;<a class="elsevierStyleCrossRef" href="#bib0075"><span class="elsevierStyleSup">15</span></a> of cases occurs within the first two years&#46;<a class="elsevierStyleCrossRef" href="#bib0060"><span class="elsevierStyleSup">12</span></a> With this type of tumour&#44; metastases are rare&#46;<a class="elsevierStyleCrossRefs" href="#bib0045"><span class="elsevierStyleSup">9&#44;10</span></a> In our case&#44; the lesion was completely resected without complications and the patient&#8217;s epuileptic seizures disappeared&#46; At present&#44; the patient still has no signs of recurrence and watchful waiting is preferred&#46;</p></span><span id="sec0020" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0045">Conclusion</span><p id="par0095" class="elsevierStylePara elsevierViewall">IP has a radiological appearance that is almost indistinguishable from meningioma&#46; Therefore&#44; meningioma must be included in the differential diagnosis and pre-surgical planning for this type of lesion&#46;</p><p id="par0100" class="elsevierStylePara elsevierViewall">IMT and IP are different processes in terms of their behaviour and histopathological features&#44; and for correct diagnosis&#44; a clear understanding of their differences is required&#46; Due to the rarity of this process and the limited amount of published literature available&#44; it is necessary to develop unified diagnostic criteria and management protocol&#46;</p><p id="par0105" class="elsevierStylePara elsevierViewall">IP secondary to a repair reaction following a traumatic injury must also be considered as a cause&#46;</p></span></span>"
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            2 => "Immunoglobulin G4"
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        "titulo" => "Abstract"
        "resumen" => "<span id="abst0005" class="elsevierStyleSection elsevierViewall"><p id="spar0020" class="elsevierStyleSimplePara elsevierViewall">Inflammatory pseudotumor is a rare lesion&#44; especially at the level of the central nervous system&#46; Its etiology is unknown and the most accepted hypothesis is that it is the consequence of an exaggerated immune response&#46; We present the clinical case of a young Senegalese male&#44; with a history of epilepsy secondary to severe cranioencephalic trauma in childhood&#44; who presented with new epileptic seizures&#46; Imaging tests showed a lesion in the anterior fossa intimately attached to the meninges&#44; so the initial diagnosis was meningioma&#46; A bifrontal craniotomy and microsurgical excision were performed&#46; The definitive anatomopathological analysis concluded that the lesion is an inflammatory pseudotumor whose origin is secondary to a disproportionate reparative process after cranioencephalic trauma&#46;</p></span>"
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        "resumen" => "<span id="abst0010" class="elsevierStyleSection elsevierViewall"><p id="spar0025" class="elsevierStyleSimplePara elsevierViewall">El pseudotumor inflamatorio es una lesi&#243;n poco frecuente&#44; especialmente a nivel del sistema nervioso central&#46; Su etiolog&#237;a es desconocida y la hip&#243;tesis m&#225;s aceptada es que es consecuencia de una respuesta inmunitaria exagerada&#46; Presentamos el caso cl&#237;nico de un var&#243;n joven senegal&#233;s&#44; con antecedentes de epilepsia secundaria a traumatismo craneoencef&#225;lico severo en la infancia&#44; que present&#243; nuevas crisis epil&#233;pticas&#46; Las pruebas de imagen mostraron una lesi&#243;n en la fosa anterior &#237;ntimamente adherida a las meninges&#44; por lo que el diagn&#243;stico inicial fue de meningioma&#46; Se realiz&#243; una craneotom&#237;a bifrontal y ex&#233;resis microquir&#250;rgica&#46; El an&#225;lisis anatomopatol&#243;gico definitivo concluy&#243; que la lesi&#243;n es un pseudotumor inflamatorio cuyo origen es secundario a un proceso reparativo desproporcionado tras el traumatismo craneoencef&#225;lico&#46;</p></span>"
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          "en" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">Pre-surgical T1-weighted MRI with gadolinium contrast&#46; A&#41; Sagittal image shows a homogeneous&#44; hyperintense lesion in the anterior cranial fossa and a porencephalic cavity with mass effect on the frontal horn of the right lateral ventricle&#46; B&#41; Axial image shows hyperostotic crista galli and the lesion with moderate peri-lesional oedema&#46; C&#41; Coronal image&#44; invasion of superior ethmoid sinuses by the lesion&#44; which has a dural tail&#46;</p>"
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          "en" => "<p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">Intraoperative imaging&#46; Retraction of the frontal lobes to expose a solid&#44; fibrous lesion adhered to the base of the anterior cranial fossa&#46;</p>"
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          "en" => "<p id="spar0015" class="elsevierStyleSimplePara elsevierViewall">Pathology&#46; Microscopic images of the lesion with hematoxylin and eosin staining&#46; A&#41; Inflammatory aggregates in a dense collagen stroma &#40;&#215;10&#41;&#46; B&#41; Plasma cells&#44; lymphocytes&#44; neutrophils and eosinophils forming an inflammatory aggregate with Russell bodies &#40;arrow&#44; &#215;40&#41;&#46; C&#41; Hypocellular tumour with thick collagen bundles &#40;&#215;20&#41;&#46; D&#41; Psammoma body surrounded by inflammatory cells&#46; E&#41; CD138-positive plasma cells &#40;&#215;40&#41;&#46; F&#41; Comparative images of the population of plasma cells with a IgG4&#47;IgG ratio that is much less than 40&#37; &#40;&#215;40&#41;&#46;</p>"
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                      "titulo" => "Inflammatory pseudotumor&#58; a rare intracranial lesion"
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                          "etal" => false
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                      "titulo" => "Inflammatory pseudotumor simulating a jugular foramen meningioma&#58; case report&#44; technical video&#44; and literature review"
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                        0 => array:2 [
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Journal Information
Vol. 35. Issue 3.
Pages 164-168 (May - June 2024)
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Vol. 35. Issue 3.
Pages 164-168 (May - June 2024)
Case Report
Inflammatory pseudotumor, the great mimicker: A case report
Pseudotumor inflamatorio, el gran imitador: a propósito de un caso
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María José Castelló Ruiza,
Corresponding author
mariajosecastelloruiz@gmail.com

Corresponding author.
, Ascensión Contreras Jimeneza, Iñigo Gorrostiaga Altunab, Jose Masegosa Gonzaleza
a Departamento de Neurocirugía, Hospital Torrecárdenas, Almería, Spain
b Departamento de Anatomía Patológica, Hospital Torrecárdenas, Almería, Spain
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Abstract

Inflammatory pseudotumor is a rare lesion, especially at the level of the central nervous system. Its etiology is unknown and the most accepted hypothesis is that it is the consequence of an exaggerated immune response. We present the clinical case of a young Senegalese male, with a history of epilepsy secondary to severe cranioencephalic trauma in childhood, who presented with new epileptic seizures. Imaging tests showed a lesion in the anterior fossa intimately attached to the meninges, so the initial diagnosis was meningioma. A bifrontal craniotomy and microsurgical excision were performed. The definitive anatomopathological analysis concluded that the lesion is an inflammatory pseudotumor whose origin is secondary to a disproportionate reparative process after cranioencephalic trauma.

Keywords:
Pseudotumor
Inflammatory
Immunoglobulin G4
Idiopathic
Myofibroblastic
Abbreviations:
CNS
IP
IMT
TBI
Resumen

El pseudotumor inflamatorio es una lesión poco frecuente, especialmente a nivel del sistema nervioso central. Su etiología es desconocida y la hipótesis más aceptada es que es consecuencia de una respuesta inmunitaria exagerada. Presentamos el caso clínico de un varón joven senegalés, con antecedentes de epilepsia secundaria a traumatismo craneoencefálico severo en la infancia, que presentó nuevas crisis epilépticas. Las pruebas de imagen mostraron una lesión en la fosa anterior íntimamente adherida a las meninges, por lo que el diagnóstico inicial fue de meningioma. Se realizó una craneotomía bifrontal y exéresis microquirúrgica. El análisis anatomopatológico definitivo concluyó que la lesión es un pseudotumor inflamatorio cuyo origen es secundario a un proceso reparativo desproporcionado tras el traumatismo craneoencefálico.

Palabras clave:
Pseudotumor
Inflamatorio
Inmunoglobulina G4
Idiopático
Miofibroblástico

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