[Melanotic neuroectodermal tumor of infancy. Clinical case of large fontanel tumor and literature review]. / Melanoticheskaya neiroektodermal'naya opukhol' mladentsev. Klinicheskii sluchai opukholi v oblasti bol'shogo rodnichka i obzor literatury.

Melanotic neuroectodermal tumor of infancy (MNTI) is a neonatal tumor with progressive growth and high recurrence rate. Aggressive growth and localization of tumor often lead to significant cosmetic defects of cranial and facial bones. The authors report MNTI in a 6-month-old boy with lesion of the large fontanel. Total resection was followed by recurrence after 3 weeks. Repeated resection with subsequent radiotherapy was performed. The follow-up period was 6 months after repeated resection. There was no tumor growth throughout this period. Considering this case and world experience, we can conclude that treatment strategy for MNTI is still unclear.

[Basal ganglia germinomas in children. Four clinical cases and a literature review].

UNLABELLED: Basal ganglia germinomas are a specific group of intracranial germinomas. Their early diagnosis is complicated due to their atypical localization and diversity of neuroimaging and clinical signs. MATERIAL AND METHODS: We describe 4 cases of basal ganglia germinoma in boys of 13, 14, 15, and 16 years of age. The medical history data, clinical features, neuroimaging and histological characteristics of basal ganglia germonomas, and preliminary results of the treatment are presented. CONCLUSION: Basal ganglia germinomas are usually verified at the late stage of the disease when patients are detected with extended lesions of the basal ganglia and severe neurological and neuroendocrine deficits. This situation is due to clinical and imaging signs that are untypical of common germinomas.

[Growing teratoma syndrome in a patient with intracranial germ cell tumor].

A six-year-old patient with non-germinomatous germ cell tumor of the chiasmatic-sellar area developed polyuria and polydipsia as the first symptoms of the disease. Then there were signs of precocious puberty and vision impairment. MRI examination revealed a shiasmatic sellar tumor and occlusive hydrocephalus. Tumor marker levels in blood serum were elevated. The alpha-fetoprotein level was increased 5-fold; human chorionic gonadotropin 20-fold. These levels increased over time. The patient received 2 cycles of PEI multiagent chemotherapy (Ifosfamide 1.5 g/m(2), Cisplatin 20 mg/m(2), Etoposide 100 mg/m(2)) during 5 days and 1 cycle of second-line multiagent chemotherapy (Cisplatin 100 mg/m(2) for 1 day and Endoxan 1500 mg/m(2) for 2 days). Despite the decrease in tumor marker levels to normal values, the patient's vision still deteriorated. MRI examination revealed that tumor size increased and its structure changed. Total tumor resection led to vision improvement and regression of intracranial hypertension. Histological analysis of tumor tissue only revealed a mature teratoma. This phenomenon, known as growing teratoma syndrome, is very rare among patients with intracranial non-germinomatous germ cell tumors.

[Characteristics of medulloblastoma in children under age of three years].

We present a series of 51 medulloblastoma in children under three years, collected in N.N. Burdenko Neurosurgical Institute from 2000 to 2010. 57% of the tumors showed desmoplastic/nodular histology. Performed fluorescence in situ hybridization (FISH) analysis revealed the MYC oncogene amplification in 4%, the MYCN oncogene amplification - in 8%, isochromosome 17q - in 16% of cases. 9q deletion was found in 8% of desmoplastic/ nodular medulloblastomas. Our results showed that desmoplastic/nodular medulloblastoma has a positive predictive value for progression-free survival. Another feature of a biology of medulloblastomas in children younger than three years is the lack of nuclear accumulation of beta-catenin, and 6q deletion. Medulloblastomas with MYCN oncogene amplification often exhibit desmoplastic/nodular histology and a relatively favorable outcome. The most unfavorable prognostic marker is the MYC oncogene amplification, which in our series of 100% combined with the large cell/anaplastic medulloblastoma and isochromosome 17q - such tumors should be included in the "high risk" protocol.

[Glioneuronal tumor with neuropil-like islands in a neonate]. / Glioneironal'naia opukhol' s uchastkami neiropilia u rebenka grudnogo vozrasta.

Glioneuronal tumor with neuropil-like islands - GTNI is a rare histopatological subtype of anaplastic astrocytoma. English-language literature contains only 43 observations, among them only 4 observations in children up to 18 years. Neuropil is an eosinophilic stained a cellular foci of tumor tissue. These cells demonstrate less proliferation activity than prevailing glial component, which is characterized by a high degree of atypia - Gr III according to WHO classification. Single reports about verification of the glioneuronal tumor with neuropil-like islands in children are supplemented with an observation of the case with tumor, located in lateral ventricular in infant with the onset of hypertension symptoms at the age of 11 months. The potential for complete removal of these tumors despite its large size and young age of the child is presented. However, the prognosis in infants with GTNI despite completeness of removal and chemotherapy might be extremely unfavorable - in case of intraventricular localization progression in the form of tumor implantation on ventricular ependyma is possible.