ABSTRACT
Introducción: Los pacientes con Retinoblastoma (RB) hereditario se encuentran en riesgo de desarrollar otros tipos de tumores malignos primarios extraoculares durante la vida. Dentro de estos tumores se encuentra el pinealoblastoma, un tipo de neoplasia maligna que aparece en la glándula pineal y que se puede desarrollar en cualquier momento del diagnóstico del retinoblastoma bilateral. Objetivo: Presentar un caso inusual de retinoblastoma (RB) hereditario tratado y en remisión quien desarrolló un quiste pineal benigno. Diseño del estudio: Reporte de caso y revisión de literatura. Resumen del caso: Presentamos un paciente con diagnóstico de RB hereditario tratado y en remisión quien desarrolló un quiste pineal benigno durante el seguimiento clínico. Las lesiones quísticas de la glándula pineal reportadas por resonancia magnética son infrecuentes en edad pediátrica, sin embargo, su incidencia se ve aumentada en pacientes con RB, lo que genera incertidumbre sobre la posibilidad de presentar una neoplasia maligna. Aún se desconoce el mecanismo fisiopatológico de la aparición de quistes de la glándula pineal en pacientes con RB hereditario, pero podría tener relación con la alteración genética o con el tratamiento quimioterápico que reciben los pacientes para el tumor primario intraocular. Conclusión: Las características imagenológicas son fundamentales para diferenciar entre lesiones benignas y malignas de la glándula pineal en pacientes con retinoblastoma hereditario y para hacer el estrecho seguimiento junto con el examen clínico.
Background: Patients with hereditary Retinoblastoma (RB) are at risk of developing other types of extraocular primary malignancies throughout life. Among these tumors, pinealoblastoma is a type of malignancy that appears in the pineal gland and can develop at any time from the diagnosis of bilateral retinoblastoma. Objective: To present an unusual case of a patient with a diagnosis of hereditary BR who developed a pineal cyst. Study design: Case report. Case summary: We present in this article the case of a patient with a diagnosis of hereditary BR with remission who developed a pineal cyst during clinical follow-up. The cystic lesions of the pineal gland reported by magnetic resonance are infrequent in pediatric age, however its incidence is increased in patients with RB, which generates uncertainty about the possibility of the development of a primary pineal gland malignancy. The pathophysiological mechanism of pineal gland cysts in patients with hereditary RB is still unknown, but it could be related to a genetic alteration or to chemotherapy treatment that these patients receive for the primary intraocular tumor. Conclusion: The imaging characteristics are fundamental to differentiate between benign and malignant lesions of the pineal gland in patients with Hereditary Retinoblastoma and to make a close follow up.
Subject(s)
Retinoblastoma , Pinealoma , Retinoblastoma/therapy , Retinoblastoma/diagnostic imagingABSTRACT
Mollaret's meningitis is a recurrent aseptic meningitis with characteristic clinical features and Mollaret cells in cerebrospinal fluid(CSF). We describe a case of Mollaret's meningitis in a 3-year-old boy who presented with three episodes of aseptic meningitis within a 4-month period. Each episode was characterized by sudden onset of meningeal irritation followed by spontaneous remission in several days. He was free of neurological symptoms between the episodes. In the acute phase of each episode, his CSF showed polymorphonuclear pleocytosis with normal protein and glucose concentrations. In addition, some epithelial cell clusters in the CSF were evident during the third episode. No pathogenic microorganisms were identified in the CSF or the blood cultures. Brain MRI revealed a benign pineal cyst, 0.8 cm in diameter, and epithelial cell clusters were supposed to represent ruptured cystic walls and recurrent episodes of aseptic meningitis were triggered by spontaneous rupture of the cyst. Our case appears to support "spontaneous rupture of epidermoid cysts in the central nervous system" as one of the etiologies of Mollaret's meningitis.
Subject(s)
Child, Preschool , Humans , Male , Brain , Epidermal Cyst , Epithelial Cells , Glucose , Leukocytosis , Magnetic Resonance Imaging , Meningitis , Meningitis, Aseptic , Remission, Spontaneous , Rupture , Rupture, SpontaneousABSTRACT
With advances in MR imaging, reports of pineal cyst have become more common. In contrast to pineal neoplasm, a pineal cyst is usually asymptomatic and is detected incidentally. We reviewed six cases of pineal cyst diagnosed between 1991 and 1996. Only one patient presented with syncopal attack due to compression of the midbrain. MRI typically reveals a cystic mass with a mean diameter of 1.66cm and shows faint rim-like contrast enhancement. Since it shows the anatomic relationship of the cyst to the aquedect, sagittal MRI is the most useful diagnost test. In the one symptomatic patient, we performed suboccipital cramiectomy and removed the cyst. It contained clear fluid and was easily removed. Surgery can be considered only when the lesion produces symptoms due to compression of the quadrigeminal plate, aqueduct, or midbrain. The long term behavior of these lesions is, however, unknown, so only with follow-up for long time will appropriate management techniques become apparent.