Tumor maligno de la vaina del nervio periférico (MPNST) glandular de la órbita: Primera descripción de la literatura de localización orbitaria en un paciente con neurofibromatosis tipo 1
Malignant peripheric nerve sheath tumor of the orbit: First description of orbital location in a patient with NF1
A.E. Romero-Rojas
, J.A. Díaz-Pérez*,**, A. Lozano-Castillo*
,
Corresponding author
aromero@cancer.gov.co
Correspondencia: Grupo de Patología, Instituto Nacional de Cancerología, Cra 9 No 1-85, Bogotá Colombia.
Correspondencia: Grupo de Patología, Instituto Nacional de Cancerología, Cra 9 No 1-85, Bogotá Colombia.
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"apellidos" => "Romero-Rojas" "email" => array:1 [ 0 => "aromero@cancer.gov.co" ] "referencia" => array:2 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">*</span>" "identificador" => "aff0005" ] 1 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">¿</span>" "identificador" => "cor0005" ] ] ] 1 => array:3 [ "nombre" => "J.A." "apellidos" => "Díaz-Pérez" "referencia" => array:2 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">*</span>" "identificador" => "aff0005" ] 1 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">**</span>" "identificador" => "aff0010" ] ] ] 2 => array:3 [ "nombre" => "A." "apellidos" => "Lozano-Castillo" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">*</span>" "identificador" => "aff0005" ] ] ] ] "afiliaciones" => array:2 [ 0 => array:3 [ "entidad" => "Grupo de Patología del Instituto Nacional de Cancerología Universidad de Santander UDES. Bucaramanga. Colombia" "etiqueta" => "<span class="elsevierStyleSup">*</span>" "identificador" => "aff0005" ] 1 => array:3 [ "entidad" => "Bogotá. Colombia. Fundación para el Avance de la Anatomía Patológica, Citología y Clínica Molecular FAPCIM Universidad de Santander UDES. Bucaramanga. Colombia" "etiqueta" => "<span class="elsevierStyleSup">**</span>" "identificador" => "aff0010" ] ] "correspondencia" => array:1 [ 0 => array:3 [ "identificador" => "cor0005" "etiqueta" => "⁎" "correspondencia" => "Correspondencia: Grupo de Patología, Instituto Nacional de Cancerología, Cra 9 No 1-85, Bogotá Colombia." ] ] ] ] "titulosAlternativos" => array:1 [ "en" => array:1 [ "titulo" => "Malignant peripheric nerve sheath tumor of the orbit: First description of orbital location in a patient with NF1" ] ] "pdfFichero" => "main.pdf" "tienePdf" => true "fechaRecibido" => "2009-05-06" "fechaAceptado" => "2009-09-20" "PalabrasClave" => array:2 [ "es" => array:1 [ 0 => array:4 [ "clase" => "keyword" "titulo" => "Palabras clave" "identificador" => "xpalclavsec161084" "palabras" => array:6 [ 0 => "Tumor maligno de la vaina del nervio periférico" 1 => "Glándula" 2 => "Órbita" 3 => "Caso clínico" 4 => "Revisión de la literatura" 5 => "(Fuente: DeCS)" ] ] ] "en" => array:1 [ 0 => array:4 [ "clase" => "keyword" "titulo" => "Key words" "identificador" => "xpalclavsec161083" "palabras" => array:6 [ 0 => "Malignant peripheral nerve sheath tumor" 1 => "Gland" 2 => "Epithelioid" 3 => "Orbit" 4 => "Case report" 5 => "Review (Source: MeSH)" ] ] ] ] "tieneResumen" => true "resumen" => array:2 [ "es" => array:2 [ "titulo" => "Resumen" "resumen" => "<span class="elsevierStyleSectionTitle">Introducción</span><p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">El tumor maligno de la vaina del nervio periférico (MPNST, por sus siglas en ingles <span class="elsevierStyleItalic">Malignant Peripheral Sheath Tumor</span>), es una neoplasia maligna originada en las células de Schwann de la vaina de revestimiento de los nervios periféricos. Esta neoplasia puede presentar componentes heterólogos benignos o malignos, con diferenciación divergente, como la diferenciación glandular.</p> <span class="elsevierStyleSectionTitle">Objetivo</span><p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">Describir el primer caso en la literatura de MPNST glandular maligno localizado a nivel orbitario y realizar una revisión sobre esta neoplasia.</p> <span class="elsevierStyleSectionTitle">Caso clínico</span><p id="spar1010" class="elsevierStyleSimplePara elsevierViewall">Niño de 9 años de edad, con diagnostico de NF1, quien presentó exoftalmos ocular, dolor retro-ocular, cefalea, asimetría facial y descenso del globo ocular derecho de 1 año de evolución; a quien se documento masa sólida orbitaria, delimitada, lobulada, que se proyecta al parénquima cerebral frontal y temporal en los estudios de tomografía computarizada y resonancia magnética. La lesión se abordó en forma fronto-orbito-cigomática con resección completa de la misma. Posteriormente, se hizo una plastia dural en base de cráneo y reconstrucción con malla de titanio. Actualmente el paciente se encuentra asintomático después de 6 meses de tratamiento. En el estudio anatomopatológico se observó una neoplasia maligna bifásica, reactiva en los elementos mesenquimales para S100, PGP 9.5, neurofilamentos y vimentina. El componente glandular fue positivo para AE1/AE3, EMA, CEA y focalmente para CD57. Se observó además reactividad para cromogranina, sinaptofisina, serotonina y somatostatina. Se realizo el diagnostico de MPNST glandular de la órbita.</p> <span class="elsevierStyleSectionTitle">Conclusión</span><p id="spar0015" class="elsevierStyleSimplePara elsevierViewall">Se describe el primer caso de MPNST glandular localizado en la órbita, el cual se presentó en un niño con NF1. Esta neoplasia extremadamente infrecuente debe ser tenida en cuenta en el estudio de lesiones bifásicas malignas, ya que su diagnostico es de peculiar importancia debido al pésimo pronóstico de los pacientes afectados.</p>" ] "en" => array:2 [ "titulo" => "Summary" "resumen" => "<span class="elsevierStyleSectionTitle">Introduction</span><p id="spar0020" class="elsevierStyleSimplePara elsevierViewall">The malignant peripheric nerve sheath tumor (MPNST), is a malignant neoplastic lesion originated in Schwann cells of the lining sheath of peripheral nerves. This neoplasia may appear with benign or malignant heterologous components, with divergent differentiation, as the glandular one.</p> <span class="elsevierStyleSectionTitle">Aim</span><p id="spar1025" class="elsevierStyleSimplePara elsevierViewall">To describe for the first time in the literature, a case of a glandular MPNST, located at the orbit and to revise the literature on this tumoral lesion.</p> <span class="elsevierStyleSectionTitle">Clinical case</span><p id="spar0025" class="elsevierStyleSimplePara elsevierViewall">Nine year old male, with a base diagnosis of NF1, who had exophthalmos, retro-ocular pain, headache, facial asymmetry and descent of the right eyeball, that started 1 year earlier. This patient showed in the Computed Tomography an Magnetic Resonance, a well delimited, lobulated, solid mass at the eyeball, which extended to the fontal and temporal brain parenchyma. A right Fronto-temporal craniotomy was made with fronto -orbital- zygomatic resection of the tumoral lesion. Later, a dural plasty and reconstruction with titanium mesh was made at the skull base. At present, the patient is asymptomatic after 4 months of follow up. A malignant biphasic neoplastic lesion was observed, reactive in the mesenchymal elements S100, PGP 9.5, neurofilaments and vimentin. The glandular component was positive for AE1/AE3, EMA, CEA and focally for CD57. There was also reactivity to cromogranin, synaptophysin, serotonin and somatostatin. The diagnosis of Glandular MPNST was made.</p> <span class="elsevierStyleSectionTitle">Conclusion</span><p id="spar0030" class="elsevierStyleSimplePara elsevierViewall">For the first time in the literature a case of Glandular MPNST located at the orbit, which occurred in child with NF1, is described. This extremely uncommon neoplasia must be taken into account, in the study of biphasic malignant lesions, as its diagnosis is of great importance because of the bad prognosis of the affected patients.</p>" ] ] "NotaPie" => array:1 [ 0 => array:2 [ "etiqueta" => "☆" "nota" => "<p class="elsevierStyleNotepara">Romero-Rojas, A.E.; Díaz-PérezJ.A.; Lozano-Castillo, A.: Tumor maligno de la vaina del nervio periférico (MPNST) glandular de la órbita: primera descripción de la literatura de localización orbitaria en un paciente con neurofibromatosis tipo 1. Neurocirugía 2010; 21: 37–45.</p>" ] ] "bibliografia" => array:2 [ "titulo" => "Bibliografía" "seccion" => array:1 [ 0 => array:2 [ "identificador" => "bibs0005" "bibliografiaReferencia" => array:34 [ 0 => array:3 [ "identificador" => "bib1" "etiqueta" => "1." 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| Year/Month | Html | Total | |
|---|---|---|---|
| 2023 March | 0 | 3 | 3 |
| 2018 February | 8 | 8 | 16 |
| 2018 January | 14 | 52 | 66 |
| 2017 December | 13 | 22 | 35 |
| 2017 November | 42 | 34 | 76 |
| 2017 October | 10 | 10 | 20 |
| 2017 September | 14 | 23 | 37 |
| 2017 August | 10 | 6 | 16 |
| 2017 July | 9 | 8 | 17 |
| 2017 June | 37 | 9 | 46 |
| 2017 May | 19 | 9 | 28 |
| 2017 April | 15 | 6 | 21 |
| 2017 March | 9 | 30 | 39 |
| 2017 February | 3 | 4 | 7 |
| 2017 January | 9 | 4 | 13 |
| 2016 December | 23 | 13 | 36 |
| 2016 November | 18 | 17 | 35 |
| 2016 October | 22 | 25 | 47 |
| 2016 September | 33 | 29 | 62 |
| 2016 August | 52 | 26 | 78 |
| 2016 July | 34 | 21 | 55 |
| 2015 December | 2 | 0 | 2 |
| 2015 August | 1 | 0 | 1 |
| 2015 July | 2 | 7 | 9 |
| 2015 June | 25 | 9 | 34 |
| 2015 May | 27 | 16 | 43 |
| 2015 April | 19 | 11 | 30 |
| 2015 March | 23 | 12 | 35 |
| 2015 February | 17 | 17 | 34 |
| 2015 January | 20 | 15 | 35 |
| 2014 December | 25 | 28 | 53 |
| 2014 November | 18 | 35 | 53 |
| 2014 October | 19 | 36 | 55 |
| 2014 September | 21 | 32 | 53 |
| 2014 August | 16 | 45 | 61 |
| 2014 July | 31 | 23 | 54 |
| 2014 June | 39 | 24 | 63 |
| 2014 May | 39 | 28 | 67 |
| 2014 April | 24 | 34 | 58 |
| 2014 March | 26 | 38 | 64 |
| 2014 February | 23 | 28 | 51 |
| 2014 January | 16 | 49 | 65 |
| 2013 December | 21 | 43 | 64 |
| 2013 November | 17 | 56 | 73 |
| 2013 October | 18 | 31 | 49 |
| 2013 September | 21 | 26 | 47 |
| 2013 August | 26 | 58 | 84 |
| 2013 July | 25 | 26 | 51 |
| 2013 June | 12 | 45 | 57 |
| 2013 May | 13 | 37 | 50 |
| 2013 April | 15 | 30 | 45 |
| 2013 March | 10 | 33 | 43 |
| 2013 February | 9 | 24 | 33 |
| 2013 January | 11 | 22 | 33 |
| 2012 December | 13 | 19 | 32 |
| 2012 November | 1 | 9 | 10 |
| 2012 October | 0 | 3 | 3 |
| 2012 September | 0 | 2 | 2 |
| 2012 January | 14 | 0 | 14 |
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