Ganglioneuroblastoma: Case report and review of the literature

Abstract

Neuroblastoma are among the most important tumors of extracranial origin in pediatric patients. They arise from embryonal cells involved in the development of the sympathetic nervous system, whose differentiation has been arrested [1,2]. They are the tumors most frequently diagnosed during the first decade of life. Their evolution is unpredictable, ranging from progression to spontaneous regression or maturation, and their location and metastatic potential vary. Little is known about the cause of these tumors and the risk factors associated with their development. This article describes a typical case of ganglioneuroblastoma and provides a review of the literature on this type of tumor.Sommario Il neuroblastoma è uno dei più importanti tumori pediatrici di derivazione extracranica; esso origina dalle cellule embrionali coinvolte nello sviluppo del sistema nervoso simpatico a causa di un blocco nel loro processo di differenziamento [1,2]. È la più frequente neoplasia della prima decade di vita; la sua progressione biologica è imprevedibile, regressione spontanea, maturazione a ganglioneuroma, localizzazione e metastatizzazione variabili. Poco è noto a riguardo dei fattori di rischio e della sua eziopatogenesi. Viene presentato un caso tipico di ganglioneuroblastoma e riesaminata la letteratura su questa neoplasia. ª

Expression profile of Wilms Tumor 1 (WT1) isoforms in undifferentiated and all-trans retinoic acid differentiated neuroblastoma cells

“…Abnormalities in WT1 gene, have been associated with the pathogenesis of Wilms tumor, Denys-Drash syndrome, and Frasier syndrome [ 10 , 26 , 27 ]. WT1 seems play different functions: it induces apoptosis, inhibits cell growth, and suppresses tumorigenicity both in vivo and in vitro models [ 28 - 34 ]. Furthermore, in tumors with advanced clinical stage and poor prognosis, high levels of WT1 mRNA have been described.…”

Treatment of High-Risk Neuroblastoma

“…Н.Н. Блохина» Минздрава России (далее НИИ ДОГ) [1]. Это наиболее часто встречающаяся в детском возрасте злокачественная солидная опухоль экстракраниальной локализации.…”

“…Чаще всего нейрогенные опухоли поражают надпочечники (35%) и симпатические ганглии забрюшинного пространства (30-35%), реже -симпатические ганглии заднего средостения (20%), шеи (1-5%), малого таза (2-3%), еще реже опухоль может развиться в вилочковой железе, легких, почках и др. [1,3].…”

“…Клинически нейробластома -чрезвычайно гетерогенная опухоль -может спонтанно рег-рессировать или созревать без лечения [4,5], что позволяет занять выжидательную позицию (стратегия «see and wait») 1 [6,7]. Однако возмож-ны агрессивное течение и резистентность к совре-менной интенсивной мультимодальной терапии [8].…”

Testicular neuroblastoma

“…The tumor can develop during the embryonic or postnatal period in any tissue that originates from the neural crest, including the adrenal medulla (35 %), paravertebral ganglia (30-35 %), posterior mediastinum (20 %), head-neck district (1-5 %), and pelvis (2-3 %); less frequent locations are the thymus, lung, kidney, anterior mediastinum, and cauda equina [1,2]. Scrotal, testicular, and paratesticular involvement is rare.…”