Central or Peripheral Precocious Puberty: Diagnostic Difficulties.

Background: An underlying identifiable organic cause is present in up to 50% cases of central precocious puberty in male patients. Case characteristics: A 7-years-8-months-old presented with delayed puberal development. Analytical examinations showed suppressed basal and stimulated levels of testosterone, LH and FSH. Abdominal ultrasound and contrast cranial magnetic resonance results were initially negative. Outcome: Germinoma was found on cranial computer tomography. Conclusion: There is often a wide time-lapse between symptoms and diagnosis of germinoma, so frequent monitoring is vital.

Neurocitoma central atípico: relato de caso / Atypical central neurocytoma: case report

Neurocitomas centrais são tumores do sistema nervoso central de comportamento usualmente benigno, que costumam ocorrer nos ventrículos laterais de indivíduos jovens. Apresentamos o caso de um paciente do sexo masculino, de 31 anos de idade, com uma lesão expansiva intraventricular ocasionando quadro de hidrocefalia e queixas visuais. O paciente foi submetido à ressecção completa da lesão. Apresentou hidrocefalia persistente no pós-operatório, necessitando de derivação ventrículo-peritoneal. A evolução foi favorável e o paciente recebeu alta para seguir acompanhamento ambulatorial. Exame anatomopatológico evidenciou neurocitoma central atípico. O objetivo deste relato é apresentar um caso clássico de uma neoplasia rara, realizando uma revisão da literatura e mostrando sua importância, dado o prognóstico favorável.

Central neurocytomas are central nervous system tumors. They are usually benign and tend to occur in the lateral ventricles of young adults. We report the case of a 31-year-old male patient with an intraventricular lesion, causing hydrocephalus and visual complaints. The patient was submitted to a complete surgical resection of the lesion. However, he persisted with hydrocephalus in the postoperative period and required a ventriculoperitoneal shunt. The clinical course was favorable and the patient was discharged to follow-up. Anatomic pathology test revealed an atypical central neurocytoma. The objective of this case report is to describe a classic presentation of a rare neoplasm, including a review of the literature, highlighting the importance of this diagnosis because of the favorable prognosis.

Gliomatose cerebral na infância com evolução favorável: relato de caso / Gliomatosis cerebri with favorable outcome in a child: a case report

OBJETIVO: Relatar caso clínico raro de gliomatose cerebral difusa em criança, que, contrariando o observado na literatura, evoluiu de maneira clinicamente satisfatória. DESCRIÇÃO: Relatamos caso de criança que desenvolveu gliomatose cerebral com manifestações clínicas graves e progressivas. Exames iniciais foram inespecíficos. Melhora clínica inicial após cirurgia neuroendoscópica, porém piorou progressivamente, manifestando hidrocefalia obstrutiva, paraparesia e dificuldade de deambulação. Foram evidenciadas lesões disseminadas no espaço subaracnóideo, sendo realizadas nova intervenção cirúrgica, quimioterapia e radioterapia. Atualmente, após 6 anos de evolução, lesões no encéfalo e canal raquidiano estão inalteradas e há importante seringomielia. Entretanto, a criança apresenta-se clinicamente estável, com desenvolvimento adequado para sua idade, evidenciando resposta satisfatória ao tratamento. COMENTÁRIOS: A apresentação clínica e a propedêutica do caso levaram ao diagnóstico de gliomatose cerebral difusa do sistema nervoso central. Há poucos relatos na literatura desse tipo de tumor em crianças, e não se encontrou nenhum relato com evolução favorável como no caso apresentado.

OBJECTIVE: To report a rare clinical case of gliomatosis cerebri with favorable outcome in a 3-year old child. DESCRIPTION: A 3-year old child developed severe and progressive symptoms of gliomatosis cerebri. The initial tests were unspecific. After clinical improvement following neuroendoscopic surgery, there was a progressive decline in clinical status with development of obstructive hydrocephalus, paraparesis and difficulty in walking. The child was again submitted to surgery after disseminated injuries in the subarachnoid space were identified. She also received chemotherapy and radiotherapy. Currently, 6 years later, spinal canal and brain injuries remain unaltered, with marked syringomyelia. However, the child is clinically stable, with adequate development for her age, indicating a satisfactory response to treatment. COMMENTS: The child's clinical presentation and the combination of symptoms led to the diagnosis of gliomatosis cerebri. There are few descriptions of this kind of tumor in children in the literature, and none reports a favorable outcome as in the present case.

A Case of Subcutaneous Sacrococcygeal Myxopapillary Ependymoma: A Case Report

Ependymomas are originated from the ependymal cells and are mostly confined to the central nervous system. Rarely, they may occur in extradural sites such as skin or subcutaneous tissue of sacrococcygeal region. The myxopapillary ependymoma is the most common type of ependymoma in the sacrococcygeal region. We operated on 54-years old female with a sacrococcygeal mass. Tumor was removed totally and histologic study revealed as myxopapillary ependymoma. Clinicopathologic features are discussed along with pertienent literature.