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1&#8211;3</a>&#41;&#46;</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><elsevierMultimedia ident="fig0010"></elsevierMultimedia><elsevierMultimedia ident="fig0015"></elsevierMultimedia><p id="par0040" class="elsevierStylePara elsevierViewall">Given that the tumor could progress and result in subsequent clinical deterioration&#44; we explained the therapeutic possibilities to the patient&#44; and the decision was made to intervene surgically&#46;<a class="elsevierStyleCrossRef" href="#bib0085"><span class="elsevierStyleSup">7</span></a></p><p id="par0050" class="elsevierStylePara elsevierViewall">In the operating room&#44; we observed a red mass totally adhered to the roots of the <span class="elsevierStyleItalic">filum terminale</span>&#46; Clips were placed both on the artery and the vein&#46; There was no marked modification in the size or the bleeding of the mass&#46; Under intraoperative neurophysiological monitoring&#44; the resection was highly complex because of the constant tendency of the mass to bleed<a class="elsevierStyleCrossRef" href="#bib0090"><span class="elsevierStyleSup">8</span></a> and its adherence to the nerve roots&#46; We resected only 90&#37; of the tumor because of the lability of the roots that were most closely in contact with the lesion&#46; As a postoperative neurological sequela&#44; the patient developed certain urinary incontinence&#44; but the low back pain that radiated to her extremities had disappeared&#46; Biopsy confirmed the diagnosis of hemangioblastoma&#59; the study of the extension of the disease ruled out the existence of other hemangioblastomas&#46;</p><p id="par0060" class="elsevierStylePara elsevierViewall">The patient remained asymptomatic until the fourth year of follow-up&#44; when she experienced a recurrence&#44; with lumbosciatic pain in her right leg&#44; and lumbosacral MRI revealed tumor progression&#46; Because of the neurological risk of further surgery&#44; the decision was made to embolize the lesion via right pedicle of L4&#44; which resulted in its marked devascularization&#46; After the procedure&#44; the patient remained asymptomatic until the clinical signs reappeared 3 months later&#46; Magnetic resonance imaging showed that the lesion continued to progress&#44; and the decision was made to reoperate&#46; This procedure achieved very limited resection due to adherence of the tumor and scar tissue to the L5 and S1 nerve roots&#44; making it necessary to interrupt the intervention due to the risk of significant neurological sequelae&#46;</p><p id="par0070" class="elsevierStylePara elsevierViewall">After the failure of the surgical approach&#44; it was decided to initiate focal radiotherapy<a class="elsevierStyleCrossRefs" href="#bib0095"><span class="elsevierStyleSup">9&#44;10</span></a> with 54<span class="elsevierStyleHsp" style=""></span>Gy over 27 sessions&#46; The patient showed a good clinical response and there was a reduction in the size of the tumor&#46;</p><p id="par0080" class="elsevierStylePara elsevierViewall">To date&#44; she continues to undergo clinical monitoring every 6 months and annual radiological evaluation&#44; and is asymptomatic and the lesion has not grown &#40;<a class="elsevierStyleCrossRefs" href="#fig0020">Figs&#46; 4 and 5</a>&#41;&#46;</p><elsevierMultimedia ident="fig0020"></elsevierMultimedia><elsevierMultimedia ident="fig0025"></elsevierMultimedia></span><span id="sec0015" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0015">Discussion</span><p id="par0085" class="elsevierStylePara elsevierViewall">Hemangioblastomas are slow-growing tumors&#46; Sporadic lesions are usually solitary and are often in regions associated with greater neurological compromise&#59; they typically affect young men &#40;40 years&#41; and are rare both in older persons and in children&#46; In individuals with VHL disease&#44; the usual age at the time of diagnosis is approximately 29 years&#46;</p><p id="par0095" class="elsevierStylePara elsevierViewall">The clinical manifestations are those derived from the location of the space-occupying lesion&#46; The treatment of these patients should be chosen with this in mind due to the potential sequelae that can result from a surgical intervention at the site of the tumor&#59; surgery should be an option reserved for those individuals who are symptomatic&#46;</p><p id="par0105" class="elsevierStylePara elsevierViewall">Although it is true that the lesions can often be completely removed&#44; it must be taken into account that the recurrence rate ranges between 16&#37; and 30&#37; and that of operative mortality from 8&#37; to 15&#37;&#44; whereas the 5-year survival is 90&#37;&#46;</p><p id="par0115" class="elsevierStylePara elsevierViewall">In this case&#44; the sequelae of urinary incontinence had been discussed and was acceptable to the patient&#44; who had scaled the entire analgesic ladder in the unsuccessful attempt to treat her severe low back pain&#46;</p><p id="par0125" class="elsevierStylePara elsevierViewall">Both surgical management and that based on radiation have a role&#44; and must be selected in accordance with the characteristics of the lesion and the patient&#44; although there are no randomized clinical trials that compare or determine the time demanded by the different therapeutic actions&#46; There is increasing evidence that supports the role of stereotaxic surgery and radiotherapy in selected patients with multiple tumors and those of difficult surgical access and in postoperative recurrence&#46;</p></span><span id="sec0020" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0020">Ethical Disclosures</span><span id="sec0025" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0025">Protection of human and animal subjects</span><p id="par0130" class="elsevierStylePara elsevierViewall">The authors declare that no experiments were performed on humans or animals for this study&#46;</p></span><span id="sec0030" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0030">Confidentiality of data</span><p id="par0135" class="elsevierStylePara elsevierViewall">The authors declare that they have followed the protocols of their work center on the publication of patient data&#46;</p></span><span id="sec0035" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0035">Right to privacy and informed consent</span><p id="par0140" class="elsevierStylePara elsevierViewall">The authors declare that no patient data appear in this article&#46;</p></span></span><span id="sec0040" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0040">Conflicts of Interest</span><p id="par0145" class="elsevierStylePara elsevierViewall">The authors declare they have no conflicts of interest&#46;</p></span></span>"
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Images in Clinical Rheumatology
Atypical Onset of Sciatica in a Patient With a Filum Terminale Hemangioblastoma
Ciatalgia de presentación atípica en paciente con hemangioblastoma de filum terminale
Tarek Carlos Salman-Montea,
Corresponding author
, Francisco Castro-Domingueza, Gloria Villalbab, Jaume Capelladesc, Jordi Monforta
a Servicio de Reumatología, Hospital del Mar, Parc de Salut Mar, IMIM, Barcelona, Spain
b Servicio de Neurocirugía, Hospital del Mar, Parc de Salut Mar, Barcelona, Spain
c Sección de Neurorradiología, Servicio de Radiología, Hospital del Mar, Parc de Salut Mar, Barcelona, Spain
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a modification of the pia mater&#46; It is about 20<span class="elsevierStyleHsp" style=""></span>cm long and originates at the vertex of the conus medullaris at the base of the coccyx&#44; providing longitudinal support to the spinal cord&#46;</p></span><span id="sec0010" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0010">Case Report</span><p id="par0010" class="elsevierStylePara elsevierViewall">We present the case of a woman who&#44; at the time of the diagnosis&#44; was 70 years of age and had a history of hemithyroidectomy for a thyroid nodule&#44; as well as acromegaly due to a growth hormone and prolactin-producing pituitary adenoma&#46; This lesion was removed using a transsphenoidal approach and her postoperative healing was satisfactory&#46; She also had osteopenia and osteoarthritis of both knees&#44; with total replacement of right knee&#46;</p><p id="par0020" class="elsevierStylePara elsevierViewall">She came to the rheumatology department for the first time after a history of several months of low back pain that was progressively more disabling&#44; and was treated with radiation of both lower limbs&#46; Maneuvers to evaluate radicular pain were negative and there was no motor or sensory deficit&#59; however&#44; the patient experienced paresthesia&#46;</p><p id="par0030" class="elsevierStylePara elsevierViewall">The patient underwent magnetic resonance imaging &#40;MRI&#41; of the low back&#44; which revealed an intradural space-occupying lesion in the conus medullaris&#46; It was hypervascularized and receiving its supply of blood from a rosary-like vessel&#44;<a class="elsevierStyleCrossRef" href="#bib0070"><span class="elsevierStyleSup">4</span></a> that extended from T10&#44; occupying nearly the entire spinal canal to the level of L5&#46; Spinal angiography confirmed the presence of a hypervascularized tumor irrigated by a medium-sized artery and a large&#44; tortuous anterior spinal artery that was not amenable to embolization<a class="elsevierStyleCrossRefs" href="#bib0075"><span class="elsevierStyleSup">5&#44;6</span></a> &#40;<a class="elsevierStyleCrossRefs" href="#fig0005">Figs&#46; 1&#8211;3</a>&#41;&#46;</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><elsevierMultimedia ident="fig0010"></elsevierMultimedia><elsevierMultimedia ident="fig0015"></elsevierMultimedia><p id="par0040" class="elsevierStylePara elsevierViewall">Given that the tumor could progress and result in subsequent clinical deterioration&#44; we explained the therapeutic possibilities to the patient&#44; and the decision was made to intervene surgically&#46;<a class="elsevierStyleCrossRef" href="#bib0085"><span class="elsevierStyleSup">7</span></a></p><p id="par0050" class="elsevierStylePara elsevierViewall">In the operating room&#44; we observed a red mass totally adhered to the roots of the <span class="elsevierStyleItalic">filum terminale</span>&#46; Clips were placed both on the artery and the vein&#46; There was no marked modification in the size or the bleeding of the mass&#46; Under intraoperative neurophysiological monitoring&#44; the resection was highly complex because of the constant tendency of the mass to bleed<a class="elsevierStyleCrossRef" href="#bib0090"><span class="elsevierStyleSup">8</span></a> and its adherence to the nerve roots&#46; We resected only 90&#37; of the tumor because of the lability of the roots that were most closely in contact with the lesion&#46; As a postoperative neurological sequela&#44; the patient developed certain urinary incontinence&#44; but the low back pain that radiated to her extremities had disappeared&#46; Biopsy confirmed the diagnosis of hemangioblastoma&#59; the study of the extension of the disease ruled out the existence of other hemangioblastomas&#46;</p><p id="par0060" class="elsevierStylePara elsevierViewall">The patient remained asymptomatic until the fourth year of follow-up&#44; when she experienced a recurrence&#44; with lumbosciatic pain in her right leg&#44; and lumbosacral MRI revealed tumor progression&#46; Because of the neurological risk of further surgery&#44; the decision was made to embolize the lesion via right pedicle of L4&#44; which resulted in its marked devascularization&#46; After the procedure&#44; the patient remained asymptomatic until the clinical signs reappeared 3 months later&#46; Magnetic resonance imaging showed that the lesion continued to progress&#44; and the decision was made to reoperate&#46; This procedure achieved very limited resection due to adherence of the tumor and scar tissue to the L5 and S1 nerve roots&#44; making it necessary to interrupt the intervention due to the risk of significant neurological sequelae&#46;</p><p id="par0070" class="elsevierStylePara elsevierViewall">After the failure of the surgical approach&#44; it was decided to initiate focal radiotherapy<a class="elsevierStyleCrossRefs" href="#bib0095"><span class="elsevierStyleSup">9&#44;10</span></a> with 54<span class="elsevierStyleHsp" style=""></span>Gy over 27 sessions&#46; The patient showed a good clinical response and there was a reduction in the size of the tumor&#46;</p><p id="par0080" class="elsevierStylePara elsevierViewall">To date&#44; she continues to undergo clinical monitoring every 6 months and annual radiological evaluation&#44; and is asymptomatic and the lesion has not grown &#40;<a class="elsevierStyleCrossRefs" href="#fig0020">Figs&#46; 4 and 5</a>&#41;&#46;</p><elsevierMultimedia ident="fig0020"></elsevierMultimedia><elsevierMultimedia ident="fig0025"></elsevierMultimedia></span><span id="sec0015" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0015">Discussion</span><p id="par0085" class="elsevierStylePara elsevierViewall">Hemangioblastomas are slow-growing tumors&#46; Sporadic lesions are usually solitary and are often in regions associated with greater neurological compromise&#59; they typically affect young men &#40;40 years&#41; and are rare both in older persons and in children&#46; In individuals with VHL disease&#44; the usual age at the time of diagnosis is approximately 29 years&#46;</p><p id="par0095" class="elsevierStylePara elsevierViewall">The clinical manifestations are those derived from the location of the space-occupying lesion&#46; The treatment of these patients should be chosen with this in mind due to the potential sequelae that can result from a surgical intervention at the site of the tumor&#59; surgery should be an option reserved for those individuals who are symptomatic&#46;</p><p id="par0105" class="elsevierStylePara elsevierViewall">Although it is true that the lesions can often be completely removed&#44; it must be taken into account that the recurrence rate ranges between 16&#37; and 30&#37; and that of operative mortality from 8&#37; to 15&#37;&#44; whereas the 5-year survival is 90&#37;&#46;</p><p id="par0115" class="elsevierStylePara elsevierViewall">In this case&#44; the sequelae of urinary incontinence had been discussed and was acceptable to the patient&#44; who had scaled the entire analgesic ladder in the unsuccessful attempt to treat her severe low back pain&#46;</p><p id="par0125" class="elsevierStylePara elsevierViewall">Both surgical management and that based on radiation have a role&#44; and must be selected in accordance with the characteristics of the lesion and the patient&#44; although there are no randomized clinical trials that compare or determine the time demanded by the different therapeutic actions&#46; There is increasing evidence that supports the role of stereotaxic surgery and radiotherapy in selected patients with multiple tumors and those of difficult surgical access and in postoperative recurrence&#46;</p></span><span id="sec0020" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0020">Ethical Disclosures</span><span id="sec0025" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0025">Protection of human and animal subjects</span><p id="par0130" class="elsevierStylePara elsevierViewall">The authors declare that no experiments were performed on humans or animals for this study&#46;</p></span><span id="sec0030" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0030">Confidentiality of data</span><p id="par0135" class="elsevierStylePara elsevierViewall">The authors declare that they have followed the protocols of their work center on the publication of patient data&#46;</p></span><span id="sec0035" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0035">Right to privacy and informed consent</span><p id="par0140" class="elsevierStylePara elsevierViewall">The authors declare that no patient data appear in this article&#46;</p></span></span><span id="sec0040" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0040">Conflicts of Interest</span><p id="par0145" class="elsevierStylePara elsevierViewall">The authors declare they have no conflicts of interest&#46;</p></span></span>"
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                      "titulo" => "Haemangioblastoma"
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                        0 => array:2 [
                          "etal" => false
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                            0 => "K&#46;D&#46; Aldape"
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Article information
ISSN: 21735743
Original language: English
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