[Malignant peripheric nerve sheath tumor of the orbit: first description of orbital location in a patient with NF1]. / 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.

INTRODUCTION: 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. AIM: 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. CLINICAL CASE: 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. CONCLUSION: 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.

Tumor maligno de la vaina del nervio periférico (MPNST) glandular de la órbita: primera descripción de la literatura de localización orbitaria en un paciente conneurofibromatosis tipo 1 / Malignant peripheric nerve sheath tumor of the orbit: First description of orbital location in a patient with NF1

Introducción. El tumor maligno de la vaina delnervio periférico (MPNST, por sus siglas en inglesMalignant Peripheral Sheath Tumor), es una neoplasiamaligna originada en las células de Schwann de la vainade revestimiento de los nervios periféricos. Esta neoplasiapuede presentar componentes heterólogos benignoso malignos, con diferenciación divergente, como la diferenciaciónglandular.Objetivo. Describir el primer caso en la literatura deMPNST glandular maligno localizado a nivel orbitarioy realizar una revisión sobre esta neoplasia.Caso clínico. Niño de 9 años de edad, con diagnosticode NF1, quien presentó exoftalmos ocular, dolorretro-ocular, cefalea, asimetría facial y descenso delglobo ocular derecho de 1 año de evolución; a quien sedocumento masa sólida orbitaria, delimitada, lobulada,que se proyecta al parénquima cerebral frontal y temporalen los estudios de tomografía computarizada yresonancia magnética. La lesión se abordó en formafronto-orbito-cigomática con resección completa de lamisma. Posteriormente, se hizo una plastia dural enbase de cráneo y reconstrucción con malla de titanio.Actualmente el paciente se encuentra asintomáticodespués de 6 meses de tratamiento. En el estudioanatomopatológico se observó una neoplasia malignabifásica, reactiva en los elementos mesenquimales paraS100, PGP 9.5, neurofilamentos y vimentina. El componenteglandular fue positivo para AE1/AE3, EMA, CEAy focalmente para CD57. Se observó además reactividadpara cromogranina, sinaptofisina, serotonina y somatostatina.Se realizo el diagnostico de MPNST glandularde la órbita (AU)

Introduction. The malignant peripheric nerve sheathtumor (MPNST), is a malignant neoplastic lesion originatedin Schwann cells of the lining sheath of peripheralnerves. This neoplasia may appear with benignor malignant heterologous components, with divergentdifferentiation, as the glandular one.Aim. To describe for the first time in the literature, acase of a glandular MPNST, located at the orbit and torevise the literature on this tumoral lesion.Clinical case. Nine year old male, with a base diagnosisof NF1, who had exophthalmos, retro-ocular pain,headache, facial asymmetry and descent of the righteyeball, that started 1 year earlier. This patient showedin 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 madewith fronto -orbital- zygomatic resection of the tumorallesion. Later, a dural plasty and reconstruction withtitanium 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 componentwas positive for AE1/AE3, EMA, CEA and focallyfor CD57. There was also reactivity to cromogranin,synaptophysin, serotonin and somatostatin. The diagnosisof Glandular MPNST was made (AU)