Pineoblastoma is a rare malignant tumor of the central nervous system (CNS), which arises from the parenchyma of the pineal gland. It is characterized by aggressive clinical behavior and frequent metastases along the craniospinal axis. Extraneural metastases may occur due to surgical seeding of tumor cells beyond the dura and/or hematogenous spread, ventriculoperitoneal shunting, or through Batson’s plexus. To our knowledge, only six documented cases of intraosseous metastases of pineoblastoma are described in the literature.A 23-year-old female patient presented with clinical and radiological symptoms of a pineal tumor causing secondary hydrocephalus. After initial surgical treatment, chemotherapy, and local radiotherapy with craniospinal irradiation, she developed multiple metastases affecting the anterior skull base, intracranial meninges, frontal bone, and finally, the entire vertebral column. The patient received surgical treatment for the anterior skull base metastasis, repeated irradiation of the neuraxis, radiosurgical and radiotherapeutic procedures, and chemotherapy. The patient survived 57 months after the primary disease manifestation and died of multiple metastases.This presented case is the first known description of metastasis of pineoblastoma in the anterior cranial base. Multiple intracranial metastases were suppressed using CyberKnife radiation treatment and chemotherapy until massive involvement of spinal column occurred. Interestingly, no signs of brain radiation necrosis after repeated radiation treatments were observed, and the patient developed only moderate neurocognitive decline.
ОБЗОРПод достаточно устоявшимся в научной литературе термином «краниотомия в сознании» (англ. awake craniotomy) подразумевают нейрохирургические вмеша-тельства, которые осуществляются с использованием пробуждения пациента из наркозного сна с целью кон-троля сохранности тех или иных функций (речевые, дви-гательные, зрение, счет, письмо и др.), обычно с исполь-зованием электрофизиологических методов стимуляции мозга. Методику интраоперационной электростимуляции в современной нейрохирургии впервые опубликовал W. Penfield Краниотомия в сознании -это нейрохирургическое вмешательство, направленное на выявление и сохранение функцио-нально значимых зон головного мозга во время удаления опухолей, расположенных рядом с корковыми и подкорковыми центрами речи. Представлен обзор современной литературы, посвященной данной проблеме. Представлены анатомиче-ское обоснование, данные предоперационной функциональной нейровизуализации, интраоперационного электрофизио-логического мониторинга, нейропсихологических тестов, а также стратегия активного хирургического вмешательства. Краниотомия в сознании является интенсивно развивающейся методикой, направленной как на сохранение речевых и двигательных функций, так и на совершенствование наших знаний в области психофизиологии речи. Awake craniotomy is a neurosurgical intervention aimed at identifying and preserving the eloquent functional brain areas during resection of tumors located near the cortical and subcortical language centers. This article provides a review of the modern literature devoted to the issue. The anatomical rationale and data of preoperative functional neuroimaging, intraoperative electrophysiological monitoring, and neuropsychological tests as well as the strategy of active surgical intervention are presented. Awake craniotomy is a rapidly developing technique aimed at both preserving speech and motor functions and improving our knowledge in the field of speech psychophysiology.
Aim of the study. To estimate the effectiveness and safety of substitution therapy with growth hormone (GH) in children with various brain tumours. Materials and methods. The study involved 68 patients admitted to N.N. Burdenko Institute of Neurosurgery between 2001 and 2011 including 35 ones with craniopharyngioma, 18 with medulloblastoma, and 15 with germ cell tumours (GCT) in the chiasmosellar region. All patients suffered growth hormone insufficiency and received GH replacement therapy. Their antropometric characteristics (height and growth rate) and IGF-1 levels were measured before, 6 and 12 months after the onset of the treatment. The doses of GH varied from 0.03 to 0.034 mg/kg/day. Results. The substitution therapy with GH resulted in an increase of the growth rate in patients with craniopharyngioma from 2.3±1.6 to 9.4±1.9 cm/year (p<0.001), in those with GCT from 1.2±0.9 to 7.4±2.6 cm/year (p=0.01), and in patients with medulloblastoma from 2.3±1.5 to 6.2±2.6 cm/year (p<0.01). The growth rate in patients treated by spinal irradiation was lower than that in those given no such treatment (6.0±2.3 cm/year and 9.2±2.1 cm/year respectively; p<0.001). The tumour recurred in eight of 35 (23%) patients with craniopharyngioma during GH therapy. The frequency of relapses was not significantly different from that in patients who did not receive growth hormone. Patients with medulloblastoma and GCT did not develop relapses. No adverse reactions to GH therapy were documented in this study.
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