Introduction: Meningeal solitary fibrous tumour is a relatively recent pathological entity that has rarely been described in children. With radiological techniques, it cannot be distinguished from meningiomas, and the diagnosis has to be confirmed histologically. Case report: We discuss the possible histogenesis of this tumour and the need for recognizing this lesion as a separate entity. We report the case of a 12-year-old boy who developed a meningeal solitary fibrous tumour; the main clinical symptoms were progressive headaches for a long period and recent transient hemiparesis. Conclusion: This child presents an uneventful evolution without additional therapy 3.5years after surger

Agazzi, Siviero

de Ribaupierre, Sandrine

Meagher-Villemure, Kathleen

Rilliet, Benedict

RERO DOC Digital Library

Childs Nerv Syst (2006) 22: 619–622DOI 10.1007/s00381-005-0042-y CASE REPORTSandrine de RibaupierreKathleen Meagher-VillemureSiviero AgazziBenedict RillietReceived: 7 July 2005Revised: 22 July 2005Published online: 14 January 2006# Springer-Verlag 2006Meningeal solitary fibrous tumour in a childAbstract Introduction: Meningealsolitary fibrous tumour is a relativelyrecent pathological entity that hasrarely been described in children.With radiological techniques, it can-not be distinguished from meningio-mas, and the diagnosis has to beconfirmed histologically. Casereport: We discuss the possible his-togenesis of this tumour and the needfor recognizing this lesion as a sepa-rate entity. We report the case of a12-year-old boy who developed ameningeal solitary fibrous tumour; themain clinical symptoms were pro-gressive headaches for a long periodand recent transient hemiparesis.Conclusion: This child presents anuneventful evolution without addi-tional therapy 3.5 years after surgery.Keywords Solitary fibrous tumour .Pediatric neurosurgeryIntroductionMeningeal solitary fibrous tumour is a relatively recentpathological entity that can occur in every organ of thebody. The first description of a meningeal location wasrecorded in 1996 by Carneiro et al. [1]. To our knowledge,already 64 cases found in the central nervous system havebeen reported but only two presentations within childhood[2–4]. The diagnosis of this tumour is mainly done byhistological examination of the surgical material, as theradiological investigation does not reveal any specificcriteria to differentiate it from a meningioma [5]. We reportthe case of a 12-year-old boy who developed such a tumourin the meninges.Materials and methodsA 12-year-old boy known to have bilateral frontalmigraines for a few years progressively developed atransient right hemiparesis associated with these migraines.The physical examination was normal except for hyperre-flexia with clonus of the lower extremities. An EEGperformed was normal. A cerebral MRI was motivatedafter the child presented a stronger episode includingdysarthria and a transient facial paresis that lasted for 2 h.The radiological examination revealed an extra-axial leftparietal mass in continuity with the falx, the superiorsagittal sinus and the dural convexity (Fig. 1). The lesionwas surrounded by a significant perilesional oedema. Thecerebral CT scan did not reveal any other feature, inparticular, no calcification. A spinal MRI was interpretedwithin normal limit.At surgery, a parietal craniotomy was performed. Itrevealed an extra-axial mass attached to the falx, wellseparated from the parenchyma anteriorly but stronglyadherent to the brain posteriorly. Total resection wasachieved. The surgical material was processed for routinehistopathological study, immunohistochemistry, electronmicroscopy and cytometry.The postoperative course was uneventful, and the childreturned home 6 days later in a good neurological status.S. de Ribaupierre (*) . S. Agazzi .B. RillietNeurosurgery Department,Centre Hospitalier UniversitaireVaudois (CHUV),Rue du Bugnon,1011 Lausanne, Switzerlande-mail: s_derib@hotmail.comK. Meagher-VillemureInstitut Universitaire de Pathologie,Centre HospitalierUniversitaire Vaudois,Lausanne, SwitzerlandThe child is still doing well 3.5 years later, with no furthersymptom and no recurrence seen in his latest MRI.PathologyThe main surgical material received for histological exam-ination consisted of a piece of roundish beige tissue, partlycovered on one side by dura. It measures 2.5×2 cm and had amultilobulated configuration (Fig. 2a). On cross-section, thetissue was firm and appeared fascicular. On frozen section,the tumour was reported as compatible with a meningioma.On study of the permanent section, however, the tumourrevealed a more complex type of growth. It was made by aproliferation of small cells with regular oval nuclei and smallfusiform or fibrillary cytoplasm. Different areas were seen:some with larger cells arranged in sheets in which thickbands of collagen were forming stellate or large spiderconfiguration (Fig. 2b). Other areas were more cellular,arranged in nodular pattern in which numerous small vesselswere bordered by one layer of endothelial cells (Fig. 2c).These areas reflected a hemangiopericytoma pattern. Otherareas had a more myxoid pattern in which the cells werealigned in small rows suggesting a chondroid or a chordoidaspect. There were no significantmitotic figures seen, and nonecrosis was visualized. On immunohistochemistry, thetumour cells were strongly positive for vimentin and desmin,negative for the epithelial markers (C11, EMA) and negativefor S-100 protein and GFAP. The CD34 was focally positivewithin the tumour cells, and the CD99 was strongly positive.The proliferation index seen on Mib-I was minimal. Anultrastructure study of a well-representative zone of thetumour revealed that the cells were of fusiform shape withbipolar processes. The nuclei were slightly cerebriform withpale chromatin. The cytoplasm contained the usual orga-nelles, a well-developed rough endoplasmic reticulum, astrong amount of intermediate filaments and occasionaldense bodies running parallel with the cytoplasmic mem-brane. Few micropinocytic vesicles were seen as well asfocal basement membrane material at the cytoplasmicborder, but no cellular junction and no glial or neuronalfeature could be seen. On static cytometry, the tumour had asharp diploid peak.The histology, immunohistochemistry results and ultra-structure findings excluded the diagnosis of a meningioma,neurofibroma, schwannoma or hemangiopericytoma andwere compatible with the diagnosis of a solitary fibroustumour.DiscussionSolitary fibrous tumours located in the meninges are rare:64 cases have been described in the literature [2, 6, 7] butonly two cases in the pediatric population [3, 4].Radiologically and macroscopically, they are notdistinguishable from meningiomas, but histologically, thedifference between meningiomas and solitary fibroustumour is easily shown by immunohistochemistry [1, 8–10]. Solitary fibrous meningeal tumours are a proliferationof fusiform cells positive for vimentin, desmin and CD34,while meningiomas are positive for S-100 protein, vimen-tin and, especially, epithelial marker like EMA. The casedescribed has the typical pathological features includingthe various patterns seen on histology, particularly thehemangiopericytoma pattern, the dense collagen fibresarrangement and the chondroid appearance focally. Onimmunohistochemistry, the reaction to vimentin, desminand CD99 was strong, but the CD34 was not as diffuselypositive as it is often seen, and no epithelial markers werereactive.The histogenesis of this tumour is not clear. Two theorieshave been elaborated in considering the leptomeningealpresentation.– The first theory is that the growth is a proliferationfrom pluripotential glial cells [11], the argument beingFig. 1 a Sagittal T1-weighted image with gadolinium showing ahomogenous enhancing lesion, with important perilesional oedema.b Axial T2-weighted image demonstrating the importance of theperilesional oedema. c Coronal T1-weighted image with gadolini-um, where the convexity dural attachment can be noted620the focal positivity for gliofibrillary acidic protein(GFAP) in some of the cases. GFAP is an intermediatefilament found in the cytoskeleton of glial cells and istherefore found in astrocytes, ependymal cells, espe-cially in maturing glia. Not all the cases described hada positive reaction to GFAP. This theory is thenconcerning only the cases where the tumour cells mayarise from this plutipotential glial cells. Anotherargument in favour of this theory would be thepositivity to CD34. As Rodriguez et al. stated [3],CD34 is a glycoprotein found in endothelial cells,myeloid progenitor cells and immature mesenchymalcells, and therefore, its positivity would tend toassociate this tumour cells to the pluripotent glial cells.– The second theory suggests that this fibrous meningealtumour is a proliferation from meningothelial cells orperivascular fibrous tissue [6] and therefore of an extra-axial origin. While on immunohistochemistry vimentinis positive, it is also an intermediate filament used as amarker for mesenchymal cells and found as well inimmature astrocytes; this marker is positive both insolitary fibrous tumours and in meningiomas, whileother markers, like epithelial markers such as EMA andCD11, are negative in solitary fibrous meningealtumours but are positive in meningiomas.In our case, CD34 was only slightly positive, whileanother marker, CD99, revealed to be strongly positive.CD99, being a peripheral neuroectodermal marker, wasfound especially in Ewing’s sarcoma/PNETand a reflectionof the 11,22 translocation.Our case, in comparison with the other pediatric casesdescribed in the literature, had a long-standing presenta-tion. This child suffered for a very long period (5 years) ofprogressive episodes of headache and transient hemipare-sis. Other cases described had a shorter history: 1-weekhistory of worsening headache in a 9-year-old child,29 months after chemotherapy and radiotherapy for amixed germ cell tumour of the pineal gland; the second oneis a 14-year-old-girl who presented intense headaches for5 months. The long-term follow-up of these children is notknown, and therefore, prognosis cannot be extrapolated onthe presenting symptoms.In the majority of the reported cases, the naturalbehaviour of the tumour was benign after surgical resec-tion; nevertheless, more aggressive growths have beenreported [12, 13], including one case of recurrence [1] andone of metastatization [14]. Will that reveal to be differentin childhood cases?ConclusionSolitary fibrous meningeal tumours represent a uniquepathological entity that is increasingly recognized with theuse of ancillary techniques in routine histopathologicalexamination. Until a broader body of knowledge isacquired, the only guideline transpiring from the fewFig. 2 aMultilobulated mass ofbeige colour, moderately firmin consistency. b Dense collagenfibres in spider arrangement.H & E, ×40. c Cellularzone with hemangiopericytomapattern. H & E, ×400.d Loose cords or rows of cellswithin a myxoid background.H & E, ×400621cases reported in the literature is a careful radiologicalfollow-up for all patients diagnosed to have such a tumour.It should be kept in mind that although the majority ofcases present a benign postoperative course, aggressivebehaviour, including recurrence and metastatization, hasalso been reported.The occurrence of such a tumour in the pediatric agegroup, although seldom reported, certainly represents avaluable experience in determining the long-term naturalhistory of these lesions.References1. Carneiro SS, Scheithauer BW,Nascimento AG, Hirose T, Davis DH(1996) Solitary fibrous tumor of themeninges: a lesion distinct from fibrousmeningioma. A clinicopathologic andimmunohistochemical study. Am J ClinPathol 106:217–2242. Caroli E, Salvati M, Orlando ER, LenziJ, Santoro A, Giangaspero F (2004)Solitary fibrous tumors of the meninges:report of four cases and literature review.Neurosurg Rev 27:246–2513. Rodriguez L, Lopez J, Marin A,Cardozo D, Molina O, Cardozo J(2000) Solitary fibrous tumor of themeninges. Clin Neuropathol 19:45–484. Slavik T, Bentley RC, Gray L, FuchsHE, McLendon RE (1998) Solitaryfibrous tumor of the meninges occurringafter irradiation of a mixed germ celltumor of the pineal gland. ClinNeuropathol 17:55–605. Guillou L, Fletcher DM, FletcherCDM, Mandahl N (2002) Extrapleuralsolitary fibrous tumour andhaemangiopericytoma. In: FletcherCDM, Unni KK, Mertens F (eds)Pathology and genetics: tumours of softtissue and bone. IARC, Lyon, pp 86–886. Alston SR, Francel PC, Jane JA Jr(1997) Solitary fibrous tumor of thespinal cord. Am J Surg Pathol 21:477–4837. Martin AJ, Fisher C, IgbaseimokumoU, Jarosz JM, Dean AF (2001) Solitaryfibrous tumours of the meninges:case series and literature review.J Neurooncol 54:57–698. Gold JS, Antonescu CR, Hajdu C,Ferrone CR, Hussain M, Lewis JJ et al(2002) Clinicopathologic correlates ofsolitary fibrous tumors. Cancer94:1057–10689. Martin AJ, Summersgill BM, Fisher C,Shipley JM, Dean AF (2002) Chromo-somal imbalances in meningeal solitaryfibrous tumors. Cancer Genet Cytogenet135:160–16410. Suzuki S, Fukui M, Nishio S, Iwaki T(2000) Clinicopathological features ofsolitary fibrous tumor of the meninges:an immunohistochemical reappraisal ofcases previously diagnosed to be fi-brous meningioma or hemangiopericy-toma. Pathol Int 50:808–81711. Mooney JE, Papasozomenos SC (1996)Leptomeningeal fibroma. ClinNeuropathol 15:92–9512. Ahn JY, Shim JY, Yang WI, Kim TS(2001) Meningeal solitary fibroustumor as an unusual cause of ex-pohthalmos: case report and review ofthe literature. Neurosurgery 48:1362–136613. Castilla EA, Prayson RA, Stevens GH,Barnett GH (2002) Brain-invasive sol-itary fibrous tumor of the meninges:report of a case. Int J Surg Pathol10:217–22114. Ng HK, Choi PC, Wong CW, To KF,Poon WS (2000) Metastatic solitaryfibrous tumor of the meninges. Casereport. J Neurosurg 93:490–493622

Meningeal solitary fibrous tumour in a child

http://doc.rero.ch/record/311788/files/381_2005_Article_42.pdf

Meningeal solitary fibrous tumour in a child

Abstract

Similar works

Full text

Available Versions

RERO DOC Digital Library