[Isolated intracranial Rosai-Dorfman disease: a case report and literature review]. / Enfermedad de Rosai-Dorfman intracraneal. Presentación de un caso y revisión de la literatura.

INTRODUCTION: Sinus histiocytosis with massive lymphadenopathy, also known as Rosai-Dorfman disease (RDD) is an idiopathic histiocytosis that usually affects the lymph nodes. Occasionally it may affect the CNS, being exceptional intracranial involvement without lymph node lesions. In the absence of typical radiological signs, affected patients are generally operated under the suspicion of a meningioma. The histological diagnosis is obtained after the surgical procedure. It is a clinicopathological entity not well known, controversy exists about its pathogenesis, clinical course and therapeutic management. CASE REPORT: We report the case of a 40-year-old male presented two generalized tonic-clonic seizures and brain MRI showed a left parieto-occipital extra-axial lesion extending into the posterior fossa, without presenting lesions at other levels. A partial resection of the lesion was performed and the histological findings were reported as Rosai-Dorfman disease. CONCLUSIONS: Despite its low frequency, the ERD should be included in the differential diagnosis of dural-based masses, compared to more common, such as meningiomas. Due to lack of specificity of additional studies its diagnosis is fundamentally histologic. More research is needed to define the best therapeutic option.

Enfermedad de Rosai-Dorfman intracraneal. Presentación de un caso y revisión de la literatura / Isolated intracranial Rosai-Dorfman disease: A case report and literature review

Introducción. La histiocitosis sinusal con linfadenopatías masivas (HSLM), también conocida como enfermedad de Rosai-Dorfman (ERD), es una histiocitosis idiopática que suele afectar a los ganglios linfáticos. Ocasionalmente puede afectar al SNC, siendo excepcio- nal la afectación intracraneal sin lesiones ganglionares. En ausencia de signos radiológicos típicos, los pacientes afectos son generalmente intervenidos bajo la sospecha de un meningioma, obteniéndose el diagnóstico histológico tras el procedimiento quirúrgico. Es una entidad clínicopatológica poco conocida, existiendo controversia sobre su etiopatogenia, curso clínico y manejo terapéutico. Caso clínico. Presentamos el caso de un paciente de 40 años que debutó con dos crisis tónicoclónicas generalizadas y en la RM cerebral se objetivó una lesión extraaxial parieto-occipital izquierda con extensión hacia la fosa posterior, sin presentar lesiones a otros niveles. Se realizó una resección parcial de la lesión y la anatomía patológica fue informada como enfermedad de RosaiDorfman. Conclusiones. A pesar de su baja frecuencia, deberíamos incluir a la ERD en el diagnóstico diferencial de las lesiones nodulares durales, frente a otras más frecuentes como son los meningiomas. Debido a la inespecificidad de las pruebas complementarias su diagnóstico es eminentemente histológico. Se preci-san más estudios para definir la mejor opción terapéutica (AU)

Introduction. Sinus histiocytosis with massive lym- phadenopathy, also known as Rosai-Dorfman disease (RDD) is an idiopathic histiocytosis that usually affects the lymph nodes. Occasionally it may affect the CNS, being exceptional intracranial involvement without lymph node lesions. In the absence of typical radio logical signs, affected patients are generally operated under the suspicion of a meningioma. The histological diagnosis is obtained after the surgical procedure. It is a clinicopathological entity not well known, controversy exists about its pathogenesis, clinical course and therapeutic management. Case report. We report the case of a 40-year-old male presented two generalized tonic-clonic seizures and brain MRI showed a left parieto-occipital extraaxial lesion extending into the posterior fossa, without pre- senting lesions at other levels. A partial resection of the lesion was performed and the histological findings were reported as Rosai-Dorfman disease. Conclusions. Despite its low frequency, the ERD should be included in the differential diagnosis of dural-based masses, compared to more common, such as meningiomas. Due to lack of specificity of additional studies its diagnosis is fundamentally histologic. More research is needed to define the best therapeutic option (AU)