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A 3-year-old boy with a history of attention-deficit/hyperactivity disorder (ADHD) presented a single focal tonic seizure. A thorough physical examination revealed no neurological deficit. A contrast enhanced MRI showed an isointense lesion in the anterior part of the cingulate gyrus extending through the left frontal lobe. After initial evaluation, the parents refused surgical treatment and a close follow up was then considered. At the age of five, the ADHD become more evident and the patient was started on methylphenidate. Poor clinical response was seen with the initiation of stimulant. The boy presented a second generalized seizure and the parents agreed surgical management. An interhemispheric approach was then performed and a gross total resection was achieved. The histopathological diagnosis corresponded to a dysembryoplastic neuroepithelial tumor (DNET). Four years after the resection, the patient is seizure free and the ADHD has also resolved without the need of medication. The disappearance of seizures is common after surgical resection of DNET tumors, but this case establishes an association with ADHD.

BACKGROUND Primary central nervous system lymphoma (PCNSL) are very rare in children. CLINICAL CASE: An 11-year-old male presented with a 2 months history with myoclonic movements in the upper right limb, and a sudden frontal headache, gait disturbance due to right hemiparesis and an ipsilateral convulsive episode. Upon admission he had critical condition, with hypertensive skull syndrome, Glasgow of 12, Karnofsky 40%, right hemiparesis, swallowing disorder, facial paralysis, and loss of photo motor reflex and unilateral amaurosis. A CT and MRI showed a huge tumor mass in the left tempo-parietal region, infiltrating the white matter and shifting the midline. A Tumor biopsy was done, and reported diffuse small cell non-Hodgkin lymphoma of high-grade, Burkitt type. Systemic lymphoma workup was negative. He received six cycles of chemotherapy based on high dose methotrexate, rituximab and triple intrathecal.After the second cycle an ophthalmologic evaluation was done, and found infiltration to the right retina, for which 6 cycles of intra vitreous chemotherapy with methotrexate were applied, he showed an excellent response, and recovered all his neurological functions except that right hemianopia persist. Control MRI showed partial response at 2nd cycle and complete response after the 4th cycle. No Radiation was performed. CONCLUSION This report highlights the fact that pediatric PCNSL may be effectively treated by a combination of HDMTX and rituximab-based chemoimmunotherapy without irradiation. Lack of awareness of this rare entity may lead to extense resections of brain, and potential permanent secuelae that were avoided in this illustrative case.

BACKGROUND Rubinstein–Taybi syndrome (RTS) is characterized by multiple congenital anomalies and associated with mutations in CREBBP (70%) and EP300 (5–10%). Previous reports have suggested an increased incidence of benign and possibly also malignant tumors, but the correlation remains unclear. Here we present a case of a patient with RTS and medulloblastoma. CLINICAL CASE: A 5-year-old male presented with increased intracranial pressure. An MRI revealed a 4.2 x 4.7 cm mass in the midline of cerebellum arising from the floor of 4th ventricle. The patient underwent a complete resection and pathology revealed medulloblastoma, classic histology. Staging established no disseminated disease. At diagnosis, a peculiar phenotype consisting in mild mental retardation, microcephaly, down-slanting palpebral fissures, broad nasal bridge, highly arched palate, mild micrognathia, screwdriver incisors and wide thumbs and toes was noted. Clinical genetics evaluation was consistent with RTS. Karyotype was performed and normal. Further genetics testing was not done. Treatment consisted in 8 cycles of chemotherapy and craniospinal radiation (2300 cGy to spine, 5500 cGy Total). At the end of treatment, there was no evidence of disease. He was under surveillance for 33 months free of disease, but relapsed with a supratentorial meningeal disease that ultimately resulted in death. CONCLUSION This report highlights the fact that pediatric medulloblastoma can be associated to RTS, in this case associated to classical histology and recurrent disease.

BACKGROUND Brain tumors are the most common solid tumors in childhood, 35% of them being low-grade gliomas (LGGs). Few data is available regard LGGs in low-and-middle-income countries. This study evaluates LGGs in a tertiary center in Mexico. DESIGN: A retrospective review of clinical files of 105 children diagnosed with LGG other than optic nerve glioma from 2007 to 2019 was done. RESULTS Median age at diagnosis was 7.2 years (from 5 months to 18 years). Male to female ratio was 0.75:1. WHO Grade I represented 68% of the cases. Anatomic sites were: posterior fossa (41%), supratentorial (43.5 %), spinal (8.5%), subependymal (6 %) and pineal (1%). Ten percent of patients had a diagnosed phacomatosis. Treatment was observation without surgery in 3.8%, surgery followed by observation in 49.5%, only chemotherapy in 2.8%, only radiotherapy in 6.7%, and surgery combined with chemotherapy or radiotherapy in 37.2% of cases. Among patients who had surgical intervention, 40% achieved gross total resection, 44% subtotal resection and 16% only biopsy. One or more recurrences were found in 20 % of patients. The 5 and 10-year overall survival (OS) was 83% and 73% respectively. The 5 and 10-year progression-free survival (PFS) was 66 % and 44 % respectively. CONCLUSIONS In this series the OS were lower compared with countries with high income, reflecting the need to improve surgery, since only 40% achieved complete resection that is a determining factor for the prognosis. We observed a decrease in OS until 10-year follow and the PFS was even lower due to recurrence/progression.

BACKGROUND Ependymomas occur in the brain or spinal cord and rarely as an extraspinal variety at the sacrococcygeal region, separated from the spinal cord. This rare presentation is thought to originate from a group of heterotopic ependymal cells called the coccygeal medullary vestige. There are few reports of this occurrence in children. CLINICAL CASE: A 7-year-old male presented with a history of a soft mass arising in the sacrococcygeal area 3 years earlier, diagnosed as pilonidal cyst at primary level and treated with surgery twice, as this mass recurred the boy was sent to our hospital, a 3rd surgery was performed, all tumoral tissue was removed, no attachment with dural space was founded, pathology revealed myxopapilar ependymoma with positivity for PS100, EMA and Vimentin. After surgery a Follow up MRI of cranium and spine showed absence of disease, no radiotherapy neither chemotherapy was implemented. He has been on surveillance from 3 years now without recurrence. CONCLUSION This report highlights the fact that pediatric ependymoma can have an extradural presentation and can be confounded with pilonidal cyst, total resection is needed to control the disease. Potential for recurrence or metastatic disease can continue 20 years from the time of primary tumor, so prolonged surveillance is important.