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Original article
Primary malignant melanoma of the central nervous system: A diagnostic challenge
Melanoma maligno primario del sistema nervioso central: un reto diagnóstico
Javier Quillo-Olvera
Corresponding author
a_i_o_n@hotmail.com

Corresponding author at: Doctores 102 Col. El Marqués, 76047 Querétaro, Querétaro, México. Tel.: +52 01 44 2223 6904.
, Juan Salvador Uribe-Olalde, Leopoldo Alberto Alcántara-Gómez, Jorge Dax Rejón-Pérez, Héctor Guillermo Palomera-Gómez
Servicio de Neurocirugía, Hospital Regional Valentín Gómez Farías, Instituto de Seguridad y Servicios Sociales de los Trabajadores del Estado, Zapopan, Jalisco, Mexico
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primary malignant melanoma of the central nervous system is even more unusual&#58; it accounts for 1&#37; of all cases of melanoma<a class="elsevierStyleCrossRefs" href="#bib0155"><span class="elsevierStyleSup">3&#44;4</span></a> and 0&#46;7&#37; of all primary tumours of the central nervous system&#46;<a class="elsevierStyleCrossRef" href="#bib0165"><span class="elsevierStyleSup">5</span></a> Its incidence is specifically 0&#46;005 cases per 100&#44;000 individuals&#46;<a class="elsevierStyleCrossRef" href="#bib0170"><span class="elsevierStyleSup">6</span></a> It presents in an age range from 35 to 50&#46;<a class="elsevierStyleCrossRef" href="#bib0175"><span class="elsevierStyleSup">7</span></a> Bhandari et al&#46;<a class="elsevierStyleCrossRef" href="#bib0150"><span class="elsevierStyleSup">2</span></a> state that it was Virchow who&#44; in 1859&#44; described the first case of primary diffuse intracranial melanoma&#44; and Oogle reported in 1899 the first case of primary solitary intracranial melanoma&#46;<a class="elsevierStyleCrossRefs" href="#bib0175"><span class="elsevierStyleSup">7&#44;8</span></a> These tumours arise from melanocytes present in the leptomeninges in the cerebral convexity&#44; in the skull base&#44; posterior fossa&#44; cervical spinal canal&#44; the pia mater covering vessels&#44; reticular formation of the bridge and spinal cord&#44; black substance and <span class="elsevierStyleItalic">locus coeruleus</span>&#46;<a class="elsevierStyleCrossRef" href="#bib0175"><span class="elsevierStyleSup">7</span></a> The most common sites within the central nervous system are&#58; lobar &#40;53&#46;1&#37;&#41;&#44; posterior fossa &#40;17&#46;3&#37;&#41; and pineal region &#40;13&#46;6&#37;&#41;&#46;<a class="elsevierStyleCrossRef" href="#bib0150"><span class="elsevierStyleSup">2</span></a> The diagnosis of these tumours is difficult&#59; it has to be carried out by exclusion&#44; with primary melanocytic tumours of the central nervous system&#44; primary tumours of the central nervous system with a certain degree of melanisation&#44; and mainly with metastases from primary extracranial melanoma&#44; the most common neoplasm causing cerebral metastases after lung cancer and breast cancer&#46;<a class="elsevierStyleCrossRefs" href="#bib0180"><span class="elsevierStyleSup">8&#44;9</span></a> Although the prognosis of patients with primary malignant melanoma of the central nervous system is discouraging&#44; especially in cases with leptomeningeal dissemination&#44;<a class="elsevierStyleCrossRefs" href="#bib0170"><span class="elsevierStyleSup">6&#44;10</span></a> and this being the most direct prognosis factor&#44;<a class="elsevierStyleCrossRef" href="#bib0195"><span class="elsevierStyleSup">11</span></a> it seems highly dependent on a full tumour resection&#46;<a class="elsevierStyleCrossRefs" href="#bib0190"><span class="elsevierStyleSup">10&#44;12</span></a></p><p id="par0010" class="elsevierStylePara elsevierViewall">A current review of PubMed literature on primary malignant melanoma of the central nervous system was carried out&#46; Due to its low incidence&#44; report cases and small series of cases were found&#46; Also&#44; it must be considered that in Mexico there is only one case of primary malignant melanoma of the central nervous system&#44; reported in 2008 by Avil&#233;s-Aguilar et al&#46;&#44;<a class="elsevierStyleCrossRef" href="#bib0205"><span class="elsevierStyleSup">13</span></a> confirming the rare incidence of this tumour&#46; In this article we document and present the radiological characteristics&#44; macroscopic&#44; histological and immunohistochemistry findings&#44; and discuss the potential aetiological genotype&#44; current management options and the prognosis of this infrequent entity&#46; An illustrative case treated in our neurosurgery service is presented&#46;</p></span><span id="sec0010" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0060">Clinical case</span><p id="par0015" class="elsevierStylePara elsevierViewall">This clinical case is of a female patient&#44; 51 years of age&#44; with no relevant history for her current condition&#46; The symptoms described upon admission had one month of progression&#44; with holocranial headache&#44; mostly in the morning&#46; Upon admission the patient mentioned intense holocranial headache&#44; which ceased momentaneously with NSAID and was worsened with the Valsalva manoeuvre&#46; She had difficulty in moving her left arm and leg&#44; leading to ambulatory problems&#46; In the physical examination we found&#58; proper alertness&#44; bradifrenia&#44; 3&#47;5 strength decrease in left arm and leg&#44; and generalised hypertonia and hyperreflexia in the four limbs&#46; Bilateral papillary oedema was observed in the ophthalmoscopy&#46; The simple cranium axial tomography&#44; carried out upon admission &#40;<a class="elsevierStyleCrossRef" href="#fig0005">Fig&#46; 1</a>&#41;&#44; showed a hyperdense&#44; well-defined image&#44; right temporoparietal&#44; apparently intraventricular&#44; with collapse of the right lateral ventricle&#44; mainly the atrium&#44; and severe expansive effect&#46; The lesion presented heterogeneous areas of density&#46; It extends up to the right parietal lobe&#46; Its medial limits are the right fornix callosum region&#44; extending along the occipital horn&#46; Compression of the callosum body is evident&#44; as well as the fornix cross-sectional area on the right side&#46; The left ventricular system is perceived as permeable&#44; with no compensatory dilation&#46; A magnetic resonance imaging study was also performed&#46; In the axial T1 weighted image &#40;<a class="elsevierStyleCrossRef" href="#fig0010">Fig&#46; 2</a>&#41;&#44; the craneodorsal extension of the tumour was observed&#59; the tumour was intraaxial and isointense in relation to the cerebral parenchyma&#44; and intratumoural hyperintense areas&#44; with suspected intratumoural microhaemorrages&#46; The expansive effect over the body of the fornix&#44; the fornix cross-sectional area&#44; the bulb of the corpus callosum and the splenium of the corpus callosum on the right side was evident&#44; in addition to the complete collapse of the body and atrium of the right ventricular system and apparent occupation of the ipsilateral occipital horn&#46; In the T1 weighted image with contrast material&#44; the same hyperintense lesion was observed&#44; homogeneously enhanced by the gadolinium&#46; In the axial T2 weighted image &#40;<a class="elsevierStyleCrossRef" href="#fig0015">Fig&#46; 3</a>&#41; a hypointense lesion was observed&#46;</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><elsevierMultimedia ident="fig0010"></elsevierMultimedia><elsevierMultimedia ident="fig0015"></elsevierMultimedia><p id="par0020" class="elsevierStylePara elsevierViewall">In her first hospital stay&#44; a loop diuretic was administered&#44; steroid&#44; anticonvulsant and semi-Fowler&#39;s position&#46; Also&#44; an elective surgical treatment was proposed&#46; The presumptive diagnosis was meningioma&#44; and a differential diagnosis was performed in the location between choroid plexus papilloma&#44; metastases and glioma for ventricular atrium tumours&#44; without taking into consideration primary malignant melanoma of the central nervous system&#46; On the fourth day she presented sudden deterioration of alertness and consciousness&#44; dilated right pupil and 2&#47;5 tetraparesis&#44; with accentuation of the superior motor neuron syndrome she had upon admission&#44; and clinical signs of right uncal herniation were added&#46; Therefore&#44; an emergency decompressive right temporal craniectomy was performed&#46; Pressure was released on the craniotomised temporal fossa&#46; She was admitted into the intensive care unit in the postsurgical stage&#46;</p><p id="par0025" class="elsevierStylePara elsevierViewall">Afterwards&#44; in better clinical condition and having improved the right pupillary diameter&#44; on her first day in the intensive care unit&#44; she went into surgery for a tumoral resection&#46; A posterosuperior craniotomy was performed&#44; as well as a superior intersulcal&#44; right parietal approach&#46; There was total resection of the tumour&#46; The macroscopic characteristics of the tumour were soft neoplastic&#44; aspirable&#44; highly vascularised and bleeding&#44; with vessel clusters&#44; of low quality and difficult coagulation&#44; parenquimatose and leptomeningeal infiltration&#46; During surgery&#44; an intraaxial lobar and extraventricular tumour was confirmed&#44; with severe expansive effect on the right lateral ventricular system&#46; The main characteristic of this tumour is that it was &#8220;black&#8221; in colour &#40;<a class="elsevierStyleCrossRef" href="#fig0020">Fig&#46; 4</a>&#41;&#46;</p><elsevierMultimedia ident="fig0020"></elsevierMultimedia><p id="par0030" class="elsevierStylePara elsevierViewall">Two weeks after the surgery&#44; a simple tomography of the head was taken&#44; where midbrain was observed with no evidence of compression&#44; an area of right temporoparietal encefalomalacia&#44; secondary to the total tumour resection&#46; The right lateral ventricular system with no collapse&#44; permeable right occipital horn and atrium&#44; with no transependimary communication&#44; because the tumour was extraventricular and those structures were not injured during resection&#46; The review of the histopathology sample revealed a highly cellular neoplasia&#44; organised in nests of epithelial cells&#44; with pleomorphic nuclei and a variable amount of cytoplasmic melanin&#44; in addition to prominent nucleoli &#40;<a class="elsevierStyleCrossRefs" href="#fig0025">Figs&#46; 5 and 6</a>&#41;&#44; and positive for HMB45 and S-100 protein&#46; With the postsurgical findings mentioned and the previous immunohistochemistry&#44; a directed anamnesis was performed&#44; regarding the family history of skin neoplasms in the patient&#44; which were denied&#46; The case was formalised by the dermatology and ophthalmology service&#44; and an exhaustive review was carried out to exclude mucotegumentary lesions and primary retinal neoplastic lesions&#46;</p><elsevierMultimedia ident="fig0025"></elsevierMultimedia><elsevierMultimedia ident="fig0030"></elsevierMultimedia><p id="par0035" class="elsevierStylePara elsevierViewall">Having ruled out metastatic tumours of the central nervous system with the use of negative tumour markers and scintigram&#44; the final conclusion was a diagnosis of primary malignant melanoma of the central nervous system&#46; The patient was discharged 9 days after her postsurgical status&#44; the oncology service determined total cerebral radiotherapy with 3&#46;000<span class="elsevierStyleHsp" style=""></span>centigray in 10 fractions&#44; concomitant with temozolamide for 6 weeks&#46; She is currently under oncology treatment&#46;</p></span><span id="sec0015" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0065">Discussion</span><p id="par0040" class="elsevierStylePara elsevierViewall">Melanosomic melanin is different from neuromelanin&#44; which is only found on neurons&#46;<a class="elsevierStyleCrossRef" href="#bib0185"><span class="elsevierStyleSup">9</span></a> Leptomeningeal melanocytes are derived from the neural crest&#44; consisting of a population of multipotential cells arising from the lateral margin of the neural tube during early embryo development&#44; on day 22 of the embryogenesis&#44; differentiating in leptomeningeal cells&#44; glial cells&#44; medullar adrenal cells and melanocytes&#46;<a class="elsevierStyleCrossRef" href="#bib0200"><span class="elsevierStyleSup">12</span></a> The higher concentration of melanocytes in the nervous system is in the pia mater&#44; around the spinal cord and the high cervical cord&#46;<a class="elsevierStyleCrossRefs" href="#bib0150"><span class="elsevierStyleSup">2&#44;14</span></a> However&#44; it is possible for the primary melanoma to arise from heterotopic masses of melanocytes in the central nervous system&#46;<a class="elsevierStyleCrossRef" href="#bib0150"><span class="elsevierStyleSup">2</span></a></p><p id="par0045" class="elsevierStylePara elsevierViewall">Several theories have been proposed&#44; such as&#58; &#40;1&#41; Mesodermal theory&#44; stating that the cell pigment arises from the mesoderm and reaches the brain or spine through pial blood vessels&#46; &#40;2&#41; Ectodermal theory&#44; where only epithelial cells can produce pigment&#59; therefore&#44; the primary malignant melanoma of the central nervous system derive from aberrant embrionary ectodermal cells&#46; &#40;3&#41; Neurogenic theory&#58; pigmented cells are derived from the neural crest and may cause tumours&#46;<a class="elsevierStyleCrossRef" href="#bib0155"><span class="elsevierStyleSup">3</span></a> Chromosome abnormalities have also been reported&#44; such as the deletion or rupture in the long and short arm of chromosome 6&#44; with a potential loss in the function of tumour suppressor genes in primary malignant melanoma of the central nervous system&#44;<a class="elsevierStyleCrossRef" href="#bib0215"><span class="elsevierStyleSup">15</span></a> and experimental models have been designed on mice showing the role of oncogenes<a class="elsevierStyleCrossRefs" href="#bib0220"><span class="elsevierStyleSup">16&#44;17</span></a> and the appearance of primary malignant melanoma of the central nervous system on mice when the oncogene NRAS is expressed in melanocytes during embryogenesis through an endogenous NRAS promoter&#46;<a class="elsevierStyleCrossRef" href="#bib0230"><span class="elsevierStyleSup">18</span></a> It is known that this oncogene induces proliferation of melanocytes and congenital melanocytic lesions in humans&#46; The acquisition of somatic mutations in the NRAS oncogene in melanocytes of the central nervous system is a risk factor predisposing to primary melanoma of the central nervous system in children&#46;<a class="elsevierStyleCrossRef" href="#bib0220"><span class="elsevierStyleSup">16</span></a> Other somatic mutations&#44; such as that of the GNAQ gen&#44; at codon 209&#44; are frequent events in primary melanocytic neoplasms of the central nervous system&#44; with relevance in the future immunotherapy of these tumours&#46;<a class="elsevierStyleCrossRef" href="#bib0225"><span class="elsevierStyleSup">17</span></a> Histopathology findings commonly found in the primary malignant melanoma of the central nervous system consist of pigmented tumour cells&#44; cytologically atypical&#44; with invasion to the central nervous system&#44; pleomorphic nuclei&#59; these are highly cellular neoplasms&#44; organised in a syncytial pattern&#44; epithelial&#44; or irregular conglomerates of pigmented cells with leptomeningeal infiltration&#46;<a class="elsevierStyleCrossRef" href="#bib0200"><span class="elsevierStyleSup">12</span></a> However&#44; a discouraging prognosis has been observed when these lesions are large&#44; indurated&#44; with deep invasion&#44; epithelioid cellular pattern and marked mitotic activity&#46;<a class="elsevierStyleCrossRefs" href="#bib0190"><span class="elsevierStyleSup">10&#44;19</span></a></p><p id="par0050" class="elsevierStylePara elsevierViewall">The peak of incidence of primary malignant melanoma of the central nervous system is in the fourth and fifth decade of life&#44; and has a preponderance in males&#46;<a class="elsevierStyleCrossRefs" href="#bib0150"><span class="elsevierStyleSup">2&#44;4</span></a> Presentation symptoms include intracranial hypertension and hydrocephalus &#40;43&#46;2&#37;&#41;&#44; focal neurologic deficit due to cerebral and spinal compression effect &#40;34&#46;6&#37;&#41;&#44; subarachnoid haemorrhage &#40;17&#46;3&#37;&#41; and convulsions &#40;11&#46;1&#37;&#41;&#46; It may be found as solitary or in the context of neurocutaneous melanosis&#44;<a class="elsevierStyleCrossRefs" href="#bib0200"><span class="elsevierStyleSup">12&#44;20</span></a> and associations have been described between primary malignant melanoma and giant congenital nevo&#44; although it is rare&#44;<a class="elsevierStyleCrossRef" href="#bib0245"><span class="elsevierStyleSup">21</span></a> and transoperatory findings between leptomeningeal melanosis and primary melanoma of the central nervous system&#46;<a class="elsevierStyleCrossRef" href="#bib0250"><span class="elsevierStyleSup">22</span></a> The beginning of symptoms may be acute&#44; potentially associated to intratumoural haemorrhages&#44; common in this tumour&#46;<a class="elsevierStyleCrossRef" href="#bib0215"><span class="elsevierStyleSup">15</span></a> 70&#37; of these lesions are black as surgical finding&#46;<a class="elsevierStyleCrossRef" href="#bib0200"><span class="elsevierStyleSup">12</span></a></p><p id="par0055" class="elsevierStylePara elsevierViewall">Typical radiologic characteristics in cranial computed tomography are those of a hyperdense lesion&#44; in images with no contrast material&#46;<a class="elsevierStyleCrossRef" href="#bib0150"><span class="elsevierStyleSup">2</span></a> In the magnetic resonance we observe a hyperintense tumour in T1 and hypointense in T2&#46;<a class="elsevierStyleCrossRef" href="#bib0150"><span class="elsevierStyleSup">2</span></a> This is due to the paramagnetic effect of stable free radicals of melanin&#46;<a class="elsevierStyleCrossRefs" href="#bib0150"><span class="elsevierStyleSup">2&#44;23</span></a> Odd electrons of melanin and protons of water produce a dipole-dipole interaction causing hyperintensity in T1 and hypointensity in T2&#46;<a class="elsevierStyleCrossRef" href="#bib0185"><span class="elsevierStyleSup">9</span></a> However&#44; there are variants in relation to these findings&#58; hypointensity in T1 and hyperintensity in T2&#44; predominantly found in tumours with less than 10&#37; of melanin in its cells&#44;<a class="elsevierStyleCrossRef" href="#bib0255"><span class="elsevierStyleSup">23</span></a> and in tumours with intralesional haemorrhage&#46;<a class="elsevierStyleCrossRef" href="#bib0160"><span class="elsevierStyleSup">4</span></a> The only imaging sign for diagnosis is leptomeningeal dissemination&#46;<a class="elsevierStyleCrossRef" href="#bib0150"><span class="elsevierStyleSup">2</span></a> Other primary tumours must be taken into consideration in the differential diagnosis with the primary malignant melanoma of the central nervous system&#44; such as meningioma&#44; meduloblastoma&#44; astrocytoma&#44; melanotic schwannoma&#44; pituitary tumours&#44; choroid plexum papillomas&#46;<a class="elsevierStyleCrossRef" href="#bib0150"><span class="elsevierStyleSup">2</span></a></p><p id="par0060" class="elsevierStylePara elsevierViewall">There is much controversy if upon diagnosis of a melanoma this is primary of the central nervous system or metastatic&#59; however&#44; Hayward<a class="elsevierStyleCrossRef" href="#bib0260"><span class="elsevierStyleSup">24</span></a> proposed in 1976 the following classifications&#58; &#40;1&#41; Primary malignant melanoma of the central nervous system&#46; &#40;2&#41; Secondary malignant melanoma of the central nervous system&#46; &#40;3&#41; Variants of other intracranial tumours with melanin&#46; For the purposes of classifying these tumours&#44; the factors to look for are as follows&#58; &#40;a&#41; with no finding of malignant melanoma outside the central nervous system&#59; &#40;b&#41; cranial or spinal leptomeninges taken by the tumour&#59; &#40;c&#41; intramedullary spinal lesions&#59; &#40;d&#41; hydrocephalus&#59; &#40;e&#41; tumour in the pineal or pituitary gland&#44; and &#40;f&#41; solitary cerebral lesion&#46;<a class="elsevierStyleCrossRef" href="#bib0260"><span class="elsevierStyleSup">24</span></a> These criteria are still relevant and used currently&#44; because the immunohistochemical diagnosis or differential histological diagnosis between the primary and secondary malignant melanoma of the central nervous system is difficult&#46;<a class="elsevierStyleCrossRef" href="#bib0265"><span class="elsevierStyleSup">25</span></a></p><p id="par0065" class="elsevierStylePara elsevierViewall">The final diagnosis has to be performed by a pathologist&#44; taking into consideration the result of immunohistochemical markers used&#44; such as the S100 protein &#40;expressed in cells originated in the neural crest&#41;&#44; HMB45 &#40;detected in melanosomas&#41; and Melan&#46;<a class="elsevierStyleCrossRef" href="#bib0150"><span class="elsevierStyleSup">2</span></a> Generally this tumour is negative for glial fibrillary acidic protein &#40;GFAP&#41; and epithelial membrane antigen &#40;EMA&#41;&#46;<a class="elsevierStyleCrossRef" href="#bib0270"><span class="elsevierStyleSup">26</span></a></p><p id="par0070" class="elsevierStylePara elsevierViewall">Surgical treatment is the main option&#46;<a class="elsevierStyleCrossRef" href="#bib0150"><span class="elsevierStyleSup">2</span></a> The use of combined chemotherapy&#44; such as decarbazine together with 1-&#40;4-amino-2-methyl-5-pyrimidinyl&#41; methyl-3-&#40;2-chloroethyl&#41; 3-nitrosourea hydrochloride &#40;ACNU&#41; and vincristine with OK 432 &#40;picibanil&#41;&#44; has proven promising results in neoplasms of metastatic and primary origin&#46;<a class="elsevierStyleCrossRef" href="#bib0150"><span class="elsevierStyleSup">2</span></a> In 2011 the FDA approved new medication for malignant metastatic melanoma of the central nervous system&#59; a new immunotherapy with ipilimumab and vemurafenib&#44; blocker of mitogen-activated protein &#40;MAP&#41; kinase&#44;<a class="elsevierStyleCrossRef" href="#bib0275"><span class="elsevierStyleSup">27</span></a> which may be used in cerebral primary malignant melanoma&#59; however&#44; there are still no clinical trials or clinical evidence to support its use as a sole therapy&#46; Most authors agree that there is no defined therapy standard&#44; and the efficacy of radiotherapy and chemotherapy on cerebral primary malignant melanoma is still controversial&#46;<a class="elsevierStyleCrossRef" href="#bib0280"><span class="elsevierStyleSup">28</span></a> The prognosis of cerebral primary malignant melanoma is better than that of metastatic malignant melanoma&#44; especially with full resection&#44;<a class="elsevierStyleCrossRef" href="#bib0200"><span class="elsevierStyleSup">12</span></a> since&#44; despite the aggressive multimodal management&#44; in cerebral secondary malignant melanoma a survival of 3&#8211;6 months is reported&#44;<a class="elsevierStyleCrossRef" href="#bib0215"><span class="elsevierStyleSup">15</span></a> compared to that of patients with diagnosis of solitary primary malignant melanoma of the central nervous system&#44; which is 20&#46;7 months&#46;<a class="elsevierStyleCrossRef" href="#bib0195"><span class="elsevierStyleSup">11</span></a></p></span><span id="sec0020" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0070">Conclusion</span><p id="par0075" class="elsevierStylePara elsevierViewall">The diagnosis of primary malignant melanoma of the central nervous system is a diagnosis by exclusion&#46; However&#44; it represents a challenge&#44; since it first has to be suspected&#44; and then try to exclude primary melanotic lesions with higher incidence and prevalence&#46; The difficulty in establishing this diagnosis is secondary to the variants of behaviour presented in imaging studies of this tumour&#46; In the case we presented&#44; an intraventricular meningioma was suspected&#44; and the signs observed in the magnetic resonance were not taken into consideration&#44; in accordance with the malanotic pattern of iso&#47;hyperintensity in T1 and hypointensity in T2&#46;</p><p id="par0080" class="elsevierStylePara elsevierViewall">The extremely low incidence of this tumour has not allowed the development of management and diagnostic guidelines&#59; therefore&#44; the prognosis is still discouraging in general&#44; only supported by the fact of a resection as extensive as possible of the lesion&#46; It is necessary to perform a deep study of the genesis of primary melanoma of the central nervous system in order to use specific therapeutic approaches with immunotherapy&#44; chemotherapy and radiotherapy&#46; Further&#44; the differential diagnosis between primary and metastatic malignant melanoma of the central nervous system continues to be complex&#46; Hayward&#39;s criteria are used to perform the differential diagnosis between these two diseases&#46;<a class="elsevierStyleCrossRef" href="#bib0280"><span class="elsevierStyleSup">28</span></a> An extracranial lesion has to be ruled out scrupulously before making a diagnosis of primary melanoma of the central nervous system&#46;</p></span><span id="sec0025" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0075">Conflict of interest</span><p id="par0085" class="elsevierStylePara elsevierViewall">The authors declare that there are no conflicts of interest&#46;</p></span></span>"
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        "resumen" => "<span id="abst0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0010">Background</span><p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">The rare incidence of primary malignant melanoma of the central nervous system and its ability to mimic other melanocytic tumours on images make it a diagnostic challenge for the neurosurgeon&#46;</p></span> <span id="abst0010" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0015">Clinical case</span><p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">A 51-year-old patient&#44; with a tumour located in the right forniceal callosum area&#46; Total surgical excision was performed&#46; Histopathological result was consistent with the diagnosis of primary malignant melanoma of the central nervous system&#44; after ruling out extracranial and extraspinal melanocytic lesions&#46;</p></span> <span id="abst0015" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0020">Conclusions</span><p id="spar0015" class="elsevierStyleSimplePara elsevierViewall">The primary malignant melanoma of the central nervous system is extremely rare&#46; There are features in magnetic resonance imaging that increase the diagnostic suspicion&#59; nevertheless there are other tumours with more prevalence that share some of these features through image&#46; Since there is not an established therapeutic standard its prognosis is discouraging&#46;</p></span>"
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        "resumen" => "<span id="abst0020" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0030">Antecedentes</span><p id="spar0020" class="elsevierStyleSimplePara elsevierViewall">La rara incidencia del melanoma maligno primario del sistema nervioso central y su capacidad de mimetizar por imagen otros tumores melanoc&#237;ticos lo hacen ser un reto diagn&#243;stico para el neurocirujano&#46;</p></span> <span id="abst0025" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0035">Caso cl&#237;nico</span><p id="spar0025" class="elsevierStyleSimplePara elsevierViewall">Presentamos el caso de una paciente femenina de 51 a&#241;os de edad&#44; con un tumor localizado en el &#225;rea calloso forniceal derecha&#46; Se realiz&#243; ex&#233;resis quir&#250;rgica total&#44; y el resultado histopatol&#243;gico fue compatible con el diagn&#243;stico de melanoma maligno primario del sistema nervioso central&#44; habi&#233;ndose descartado lesiones extracraneales y extrarraqu&#237;deas mucotegumentarias melanoc&#237;ticas&#46;</p></span> <span id="abst0030" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0040">Conclusiones</span><p id="spar0030" class="elsevierStyleSimplePara elsevierViewall">El melanoma maligno primario del sistema nervioso central es extremadamente raro&#46; Existen caracter&#237;sticas en imagen de resonancia magn&#233;tica que incrementan la sospecha diagn&#243;stica&#46; Sin embargo&#44; hay otros tumores m&#225;s prevalentes que comparten algunas de esas caracter&#237;sticas por imagen&#46; No hay un est&#225;ndar terap&#233;utico establecido&#46; Su pron&#243;stico es desalentador&#46;</p></span>"
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            "identificador" => "abst0020"
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        "etiqueta" => "&#9734;"
        "nota" => "<p class="elsevierStyleNotepara" id="npar0005">Please cite this article as&#58; Quillo-Olvera J&#44; Uribe-Olalde JS&#44; Alc&#225;ntara-G&#243;mez LA&#44; Rej&#243;n-P&#233;rez JD&#44; Palomera-G&#243;mez HG&#46; Melanoma maligno primario del sistema nervioso central&#58; un reto diagn&#243;stico&#46; Cir Cir&#46; 2015&#59; 83&#58; 129&#8211;134&#46;</p>"
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                          "etal" => false
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ISSN: 24440507
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