Pineal anlage tumor: a case report and review of the literature.

PURPOSE: Pineal anlage tumors (PAT) are extremely rare malignant tumors in the pediatric population considered to be a variant of pineoblastomas. These tumors are characterized by a combination of neuroectodermal and heterologous ectomesenchymal components. PAT can be clinically aggressive and has a propensity to cerebrospinal fluid (CSF) dissemination. So far, only 13 cases of these tumors have been reported in the literature. CASE ARTICLE: In this article, we report the case of an anlage tumor in a 3-year-old girl with a rapidly aggressive course.

An Unusual Dermoid Cyst of the Pineal Region: Case Report in a Child

Introduction The pineal gland is a common location for intracranial germ cells, but dermoids are not commonly observed in this area. In the present paper, we discuss the clinical and radiological features as well as the treatment and outcome of this cyst in a 6-year- old child with a pineal dermoid cyst. Case Presentation The patient presented with chronic headache 6 months before admission in 2018. On the first admission, an enhanced lesion with a small cyst was detected in brain imaging. Magnetic resonance imaging (MRI) of the brain at follow-up (2 months after the first presentation) showed enlargement of the cyst size with compression on the adjacent structures. Radical excision of the tumor was performed after the endoscopic biopsy due to pressure exerted on the adjacent structures. Conclusion Dermoid cyst should be considered as a differential diagnosis for enhanced lesions of the pineal region.

Tumor pineal resuelto por Abordaje Infratentorial Supracerebeloso Endoscópico. Reporte de un caso y revisión de la literatura / Pineal tumor resolved by Endoscopic Supracerebellar Infratentorial Approach. Case report and literature review

Introducción: Los tumores de la región pineal constituyen un grupo heterogéneo de lesiones localizadas en la porción posterior del tercer ventrículo bajo el esplenio del cuerpo calloso y encima de la lámina cuadrigémina. Dentro de estos, los tumores del parénquima pineal constituyen un grupo frecuente. En estas lesiones no existe actualmente un protocolo establecido debido a la ausencia de grandes series. Se sugiere una combinación de cirugía y radioterapia con o sin quimioterapia. El tratamiento de elección es la resección total, sin embargo, muchas veces no es posible por dificultades técnicas. En este contexto, la cirugía con visualización endoscópica puede contribuir a lograr este objetivo. Descripción del caso: Se presenta un paciente masculino de 22 años de edad con lesión de región pineal e hidrocefalia obstructiva triventricular al que se le practicó una tercerventriculostomía endoscópica y un abordaje infratentorial supracerebeloso con total visualización endoscópica. Se describe la técnica quirúrgica y se realiza un análisis crítico de la literatura actualizada. Conclusiones: Los tumores de la región pineal constituyen un reto terapéutico. La resección total es el principal objetivo en lesiones de grado intermedio o bajo de malignidad. El abordaje infratentorial supracerebeloso con total visualización endoscópica es efectivo en la resección quirúrgica de estas lesiones al mejorar la visualización y minimizar la retracción cerebelosa

Introduction: Pineal region tumors are a variable group of lesions located in the posterior wall of the third ventricle under the corpus callous splenium and above the tectal plate. Pineal gland tumors are frequent. There is not a standard protocol in these lesions due the lack of large series. A combination of surgery and chemo therapy or radiotherapy are recommended. The complete surgical resection is the treatment of choice. However, usually it is not possible to accomplished this goal due to technical limitations. In this scenario, the endoscopic visualization could contribute to archive the goal. Patient characteristics: A 22 years old young male patient with a pineal region lesion and obstructive hydrocephalus is presented. A third ventriculostomy was performed and an infratentorial supracerebelous approach with fully endoscopic visualization. The surgical technique is described and a critical review of literature is performed. Conclusions: Pineal region tumors represents a therapeutic challenge. Total removal is the most important objective in intermediate or low-grade lesions. The infratentorial supracerebelous approach with full endoscopic visualization is effective and improve the visualization while reducing the cerebellar retraction.

Melatonin Therapy Improves Cardiac Autonomic Modulation in Pinealectomized Patients.

The purpose of this investigational study was to assess the effects of melatonin replacement therapy on cardiac autonomic modulation in pinealectomized patients. This was an open-label, single-arm, single-center, proof-of-concept study consisting of a screening period, a 3-month treatment period with melatonin (3 mg/day), and a 6-month washout period. The cardiac autonomic function was determined through heart rate variability (HRV) measures during polysomnography. Pinealectomized patients (n = 5) with confirmed absence of melatonin were included in this study. Melatonin treatment increased vagal-dominated HRV indices including root mean square of the successive R-R interval differences (RMSSD) (39.7 ms, 95% CI 2.0-77.4, p = 0.04), percentage of successive R-R intervals that differ by more than 50 ms (pNN50) (17.1%, 95% CI 9.1-25.1, p = 0.003), absolute power of the high-frequency band (HF power) (1,390 ms2, 95% CI 511.9-2,267, p = 0.01), and sympathetic HRV indices like standard deviation of normal R-R wave interval (SDNN) (57.6 ms, 95% CI 15.2-100.0, p = 0.02), and absolute power of the low-frequency band (LF power) (4,592 ms2, 95% CI 895.6-8,288, p = 0.03). These HRV indices returned to pretreatment values when melatonin treatment was discontinued. The HRV entropy-based regularity parameters were not altered in this study, suggesting that there were no significant alterations of the REM-NREM ratios between the time stages of the study. These data show that 3 months of melatonin treatment may induce an improvement in cardiac autonomic modulation in melatonin-non-proficient patients. ClinicalTrials.gov Identifier: NCT03885258.

Pineal gliosarcoma in a 5-year-old girl.

The purpose of this paper is to report a rare case of a pediatric pineal gliosarcoma. Gliomas on the pineal region are uncommon, representing 0.4%-1% of all brain tumors. Furthermore, pediatric gliosarcomas are a very rare entity. We present a case of a 5-year-old girl, with a history of headache, vomiting, diplopia, and gait disturbances. A pineal tumor was found with pathology results consistent with a gliosarcoma. A total of 25 cases of pediatric gliosarcomas have been reported, none of them in pineal topography. Only 3 gliosarcomas were found in the pineal region, but these were found in adults. To our knowledge, this is the first pediatric pineal gliosarcoma reported in the literature.