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    "textoCompleto" => "<span class="elsevierStyleSections"><p id="par0005" class="elsevierStylePara elsevierViewall">Congenital Melanocytic Nevus &#40;CMN&#41; is characterized by pigmented lesions present at birth or in the first weeks of life&#46; The Giant Congenital Melanocytic Nevus &#40;GCMN&#41; form presents a minimum extension of 20<span class="elsevierStyleHsp" style=""></span>cm in adult life and is rare &#40;1&#58;20&#44;000 newborns&#41;&#46; In addition to being an unsightly condition&#44; giant CMN presents additional risks of extracutaneous morbidities such as neurological complications&#46; A complicating factor is the presence of melanocytic cells in the Central Nervous System &#40;CNS&#41;&#44; a comorbidity called Neurocutaneous Melanosis &#40;NCM&#41;&#46;<a class="elsevierStyleCrossRef" href="#bib0030"><span class="elsevierStyleSup">1</span></a></p><p id="par0010" class="elsevierStylePara elsevierViewall">We report a case of female patient&#44; accompanied since the age of 2 years with GCMN in garment&#46; In clinical follow-up&#44; no neurological symptoms or changes in psychomotor development were detected&#46; A brain Magnetic Resonance Imaging &#40;MRI&#41; confirmed the presence of T1-Weighted &#40;W&#41; images in the left parietal region&#44; corresponding to neurocutaneous melanosis&#46; The diagnosis was made upon typical imaging and skin findings&#46;</p><p id="par0015" class="elsevierStylePara elsevierViewall">Dermatological examination revealed a brownish-black plaque&#44; with evident hypertrichosis&#44; extending from the cervical region to the knees&#44; garment-like&#44; with multiple satellite lesions on the face&#44; upper and lower limbs &#40;<a class="elsevierStyleCrossRef" href="#fig0005">Fig&#46; 1</a>&#41;&#46; The patient had nodules on the back corresponding to schawannoma&#46; At 10 years old&#44; after loss of clinical follow-up for 3 years&#44; she started a sudden onset of seizures&#44; right hemisphere paresis&#44; headache and vomiting&#46;</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><p id="par0020" class="elsevierStylePara elsevierViewall">Brain MRI scan demonstrated the presence of a single solid expansive lesion measuring 5<span class="elsevierStyleHsp" style=""></span>cm<span class="elsevierStyleHsp" style=""></span>&#215;<span class="elsevierStyleHsp" style=""></span>3&#46;5<span class="elsevierStyleHsp" style=""></span>cm in the left fronto-parietal region&#44; associated with an intense vasogenic edema&#44; promoting midline deviation &#40;<a class="elsevierStyleCrossRef" href="#fig0010">Fig&#46; 2</a>&#41;&#46; Histopathology showed a neoplasm formed by the proliferation of atypical cells&#44; containing granular brown pigment similar to melanin and with hyperchromatic&#44; enlarged central nuclei with evident nucleoli&#44; frequent atypical mitoses&#44; preferentially infiltrating the meningeal but also the adjacent brain parenchyma&#44; amid areas of necrosis and hemorrhage &#40;<a class="elsevierStyleCrossRef" href="#fig0015">Fig&#46; 3</a>&#41;&#46;</p><elsevierMultimedia ident="fig0010"></elsevierMultimedia><elsevierMultimedia ident="fig0015"></elsevierMultimedia><p id="par0025" class="elsevierStylePara elsevierViewall">The immunohistochemical examination showed strong reactivity to the panel of antibodies S100&#44; HBM45 and Melan A&#46; Additional imaging studies showed no metastasis&#46; The final diagnosis was primary melanoma of leptomeningeal&#46; The patient died from intracranial hemorrhage followed by cardiorespiratory arrest four months after diagnosis&#46;</p><p id="par0030" class="elsevierStylePara elsevierViewall">Primary CNS melanoma is a rare disease&#46; It represents 1&#37; of melanomas and approximately 0&#46;05&#37; of primary malignancies of cranial tumors&#46; These can be divided into nodular intraparenchymal and diffuse leptomeningeal patterns&#46;<a class="elsevierStyleCrossRef" href="#bib0035"><span class="elsevierStyleSup">2</span></a> Primary leptomeningel Malignant Melanoma &#40;MM&#41; is extremely rare&#44; with an incidence of one case per 20 million individuals&#44; generally showing aggressive progression and resistance to chemotherapy and radiotherapy&#46;<a class="elsevierStyleCrossRefs" href="#bib0030"><span class="elsevierStyleSup">1&#44;2</span></a></p><p id="par0035" class="elsevierStylePara elsevierViewall">The risk of estimated lifetime MM-all sites for individuals with CMN is around 5&#37;&#44; with increased risk to 12&#37; in patients with neurocutaneous melanosis&#46; This is characterized by the migration and erroneous proliferation of melanocytic cells in the CNS from neural crest melanoblasts&#46;<a class="elsevierStyleCrossRefs" href="#bib0035"><span class="elsevierStyleSup">2&#44;3</span></a></p><p id="par0040" class="elsevierStylePara elsevierViewall">NCM involves several additional comorbidities which include hydrocephalus&#44; convulsions&#44; cranial nerve palsy&#44; neuropsychiatric disorders and the risk of malignant degeneration of the cells&#46; Mortality rate is close to 100&#37; for CNS MM cases and 70&#37; of patients with neurocutaneous melanosis will die before 10 years of age&#46;<a class="elsevierStyleCrossRefs" href="#bib0030"><span class="elsevierStyleSup">1&#44;3</span></a></p><p id="par0045" class="elsevierStylePara elsevierViewall">This aggressive entity found within the context of CMN is due to a different biological behavior with the presence of somatic mutations in 81&#37; of cases in the NRAS gene of the melanocytes&#44; in detriment of the mutations BRAF&#44; demonstrating that they are genetically different from nevi developed after birth and an important risk factor for primary CNS and cutaneous melanoma&#46;<a class="elsevierStyleCrossRef" href="#bib0045"><span class="elsevierStyleSup">4</span></a></p><p id="par0050" class="elsevierStylePara elsevierViewall">NRAS-mutant tumors tend to behave more aggressively particularly in early stages of the disease&#46; In view of this differential genetic behavior&#44; target therapies have been investigated for CNS melanoma in patients with CMN and the proven mutation of the protoncogene NRAS&#46; Initial studies have demonstrated results of MEK inhibitors&#44; Trametinib&#44; in symptom control and improved quality of life&#44; an important step in the discovery of treatment for this condition&#46;<a class="elsevierStyleCrossRefs" href="#bib0040"><span class="elsevierStyleSup">3&#44;5</span></a></p><p id="par0055" class="elsevierStylePara elsevierViewall">Evidence indicates a higher incidence of this neoplasm in patients presenting multiple satellite lesions&#44; such as the pattern in garment-like&#44; and&#47;or paravertebral or axial location&#46;<a class="elsevierStyleCrossRefs" href="#bib0030"><span class="elsevierStyleSup">1&#44;3</span></a></p><p id="par0060" class="elsevierStylePara elsevierViewall">CNS melanoma currently emerges as the major limiting prognostic factor in children with CMN&#46; In this scenario&#44; cutaneous melanoma plays a less decisive role&#44; influencing the decision toward prophylactic surgical excision&#46; Brain MRI is important in this scenario&#44; which should preferably be performed in the first year of life&#44; since the incidence of CNS and cutaneous MM in the group with altered examination is 12&#37;&#44; as opposed to MM incidence of 1&#37; in the group with normal CNS MRI at birth&#46; The clinical follow-up of patients with altered MRI examinations should be annual&#46;<a class="elsevierStyleCrossRef" href="#bib0040"><span class="elsevierStyleSup">3</span></a></p><span id="sec0010" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0010">Financial support</span><p id="par0085" class="elsevierStylePara elsevierViewall">None declared&#46;</p></span><span id="sec0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0005">Authors&#8217; contributions</span><p id="par0065" class="elsevierStylePara elsevierViewall">Adriana Kamilly Leit&#227;o Pitman Machado&#58; Approval of the final version of the manuscript&#59; conception and planning of the study&#59; elaboration and writing of the manuscript&#59; critical review of the literature&#59; critical review of the manuscript&#46;</p><p id="par0070" class="elsevierStylePara elsevierViewall">D&#233;bora Bacellar Cruz Nunes&#58; Elaboration and writing of the manuscript&#46;</p><p id="par0075" class="elsevierStylePara elsevierViewall">Francisca Regina Oliveira Carneiro&#58; Intellectual participation in the propaedeutic and&#47;or therapeutic conduct of the studied cases&#46;</p><p id="par0080" class="elsevierStylePara elsevierViewall">Alena Margareth Darwich Mendes&#58; Intellectual participation in the propaedeutic and&#47;or therapeutic conduct of the studied cases&#46;</p></span><span id="sec0015" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0015">Conflicts of interest</span><p id="par0090" class="elsevierStylePara elsevierViewall">None declared&#46;</p></span></span>"
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Case Letter
Primary melanoma of leptomeninge in a patient with giant congenital melanocytic nevus
Adriana Kamilly Leitão Pitman Machado
Autor para correspondência
adriana_kamilly@hotmail.com

Corresponding author.
, Débora Bacellar Cruz Nunes, Francisca Regina Oliveira Carneiro, Alena Margareth Darwich Mendes
Service of Dermatology, Universidade do Estado do Pará, Belém, PA, Brazil
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In clinical follow-up&#44; no neurological symptoms or changes in psychomotor development were detected&#46; A brain Magnetic Resonance Imaging &#40;MRI&#41; confirmed the presence of T1-Weighted &#40;W&#41; images in the left parietal region&#44; corresponding to neurocutaneous melanosis&#46; The diagnosis was made upon typical imaging and skin findings&#46;</p><p id="par0015" class="elsevierStylePara elsevierViewall">Dermatological examination revealed a brownish-black plaque&#44; with evident hypertrichosis&#44; extending from the cervical region to the knees&#44; garment-like&#44; with multiple satellite lesions on the face&#44; upper and lower limbs &#40;<a class="elsevierStyleCrossRef" href="#fig0005">Fig&#46; 1</a>&#41;&#46; The patient had nodules on the back corresponding to schawannoma&#46; At 10 years old&#44; after loss of clinical follow-up for 3 years&#44; she started a sudden onset of seizures&#44; right hemisphere paresis&#44; headache and vomiting&#46;</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><p id="par0020" class="elsevierStylePara elsevierViewall">Brain MRI scan demonstrated the presence of a single solid expansive lesion measuring 5<span class="elsevierStyleHsp" style=""></span>cm<span class="elsevierStyleHsp" style=""></span>&#215;<span class="elsevierStyleHsp" style=""></span>3&#46;5<span class="elsevierStyleHsp" style=""></span>cm in the left fronto-parietal region&#44; associated with an intense vasogenic edema&#44; promoting midline deviation &#40;<a class="elsevierStyleCrossRef" href="#fig0010">Fig&#46; 2</a>&#41;&#46; Histopathology showed a neoplasm formed by the proliferation of atypical cells&#44; containing granular brown pigment similar to melanin and with hyperchromatic&#44; enlarged central nuclei with evident nucleoli&#44; frequent atypical mitoses&#44; preferentially infiltrating the meningeal but also the adjacent brain parenchyma&#44; amid areas of necrosis and hemorrhage &#40;<a class="elsevierStyleCrossRef" href="#fig0015">Fig&#46; 3</a>&#41;&#46;</p><elsevierMultimedia ident="fig0010"></elsevierMultimedia><elsevierMultimedia ident="fig0015"></elsevierMultimedia><p id="par0025" class="elsevierStylePara elsevierViewall">The immunohistochemical examination showed strong reactivity to the panel of antibodies S100&#44; HBM45 and Melan A&#46; Additional imaging studies showed no metastasis&#46; The final diagnosis was primary melanoma of leptomeningeal&#46; The patient died from intracranial hemorrhage followed by cardiorespiratory arrest four months after diagnosis&#46;</p><p id="par0030" class="elsevierStylePara elsevierViewall">Primary CNS melanoma is a rare disease&#46; It represents 1&#37; of melanomas and approximately 0&#46;05&#37; of primary malignancies of cranial tumors&#46; These can be divided into nodular intraparenchymal and diffuse leptomeningeal patterns&#46;<a class="elsevierStyleCrossRef" href="#bib0035"><span class="elsevierStyleSup">2</span></a> Primary leptomeningel Malignant Melanoma &#40;MM&#41; is extremely rare&#44; with an incidence of one case per 20 million individuals&#44; generally showing aggressive progression and resistance to chemotherapy and radiotherapy&#46;<a class="elsevierStyleCrossRefs" href="#bib0030"><span class="elsevierStyleSup">1&#44;2</span></a></p><p id="par0035" class="elsevierStylePara elsevierViewall">The risk of estimated lifetime MM-all sites for individuals with CMN is around 5&#37;&#44; with increased risk to 12&#37; in patients with neurocutaneous melanosis&#46; This is characterized by the migration and erroneous proliferation of melanocytic cells in the CNS from neural crest melanoblasts&#46;<a class="elsevierStyleCrossRefs" href="#bib0035"><span class="elsevierStyleSup">2&#44;3</span></a></p><p id="par0040" class="elsevierStylePara elsevierViewall">NCM involves several additional comorbidities which include hydrocephalus&#44; convulsions&#44; cranial nerve palsy&#44; neuropsychiatric disorders and the risk of malignant degeneration of the cells&#46; Mortality rate is close to 100&#37; for CNS MM cases and 70&#37; of patients with neurocutaneous melanosis will die before 10 years of age&#46;<a class="elsevierStyleCrossRefs" href="#bib0030"><span class="elsevierStyleSup">1&#44;3</span></a></p><p id="par0045" class="elsevierStylePara elsevierViewall">This aggressive entity found within the context of CMN is due to a different biological behavior with the presence of somatic mutations in 81&#37; of cases in the NRAS gene of the melanocytes&#44; in detriment of the mutations BRAF&#44; demonstrating that they are genetically different from nevi developed after birth and an important risk factor for primary CNS and cutaneous melanoma&#46;<a class="elsevierStyleCrossRef" href="#bib0045"><span class="elsevierStyleSup">4</span></a></p><p id="par0050" class="elsevierStylePara elsevierViewall">NRAS-mutant tumors tend to behave more aggressively particularly in early stages of the disease&#46; In view of this differential genetic behavior&#44; target therapies have been investigated for CNS melanoma in patients with CMN and the proven mutation of the protoncogene NRAS&#46; Initial studies have demonstrated results of MEK inhibitors&#44; Trametinib&#44; in symptom control and improved quality of life&#44; an important step in the discovery of treatment for this condition&#46;<a class="elsevierStyleCrossRefs" href="#bib0040"><span class="elsevierStyleSup">3&#44;5</span></a></p><p id="par0055" class="elsevierStylePara elsevierViewall">Evidence indicates a higher incidence of this neoplasm in patients presenting multiple satellite lesions&#44; such as the pattern in garment-like&#44; and&#47;or paravertebral or axial location&#46;<a class="elsevierStyleCrossRefs" href="#bib0030"><span class="elsevierStyleSup">1&#44;3</span></a></p><p id="par0060" class="elsevierStylePara elsevierViewall">CNS melanoma currently emerges as the major limiting prognostic factor in children with CMN&#46; In this scenario&#44; cutaneous melanoma plays a less decisive role&#44; influencing the decision toward prophylactic surgical excision&#46; Brain MRI is important in this scenario&#44; which should preferably be performed in the first year of life&#44; since the incidence of CNS and cutaneous MM in the group with altered examination is 12&#37;&#44; as opposed to MM incidence of 1&#37; in the group with normal CNS MRI at birth&#46; The clinical follow-up of patients with altered MRI examinations should be annual&#46;<a class="elsevierStyleCrossRef" href="#bib0040"><span class="elsevierStyleSup">3</span></a></p><span id="sec0010" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0010">Financial support</span><p id="par0085" class="elsevierStylePara elsevierViewall">None declared&#46;</p></span><span id="sec0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0005">Authors&#8217; contributions</span><p id="par0065" class="elsevierStylePara elsevierViewall">Adriana Kamilly Leit&#227;o Pitman Machado&#58; Approval of the final version of the manuscript&#59; conception and planning of the study&#59; elaboration and writing of the manuscript&#59; critical review of the literature&#59; critical review of the manuscript&#46;</p><p id="par0070" class="elsevierStylePara elsevierViewall">D&#233;bora Bacellar Cruz Nunes&#58; Elaboration and writing of the manuscript&#46;</p><p id="par0075" class="elsevierStylePara elsevierViewall">Francisca Regina Oliveira Carneiro&#58; Intellectual participation in the propaedeutic and&#47;or therapeutic conduct of the studied cases&#46;</p><p id="par0080" class="elsevierStylePara elsevierViewall">Alena Margareth Darwich Mendes&#58; Intellectual participation in the propaedeutic and&#47;or therapeutic conduct of the studied cases&#46;</p></span><span id="sec0015" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0015">Conflicts of interest</span><p id="par0090" class="elsevierStylePara elsevierViewall">None declared&#46;</p></span></span>"
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