CONGENITAL HYPOTHALAMIC HAMARTOBLASTOMA, HYPOPITUITARISM, IMPERFORATE ANUS, AND POSTAXIAL POLYDACTYLY - A NEW SYNDROME .2. NEUROPATHOLOGICAL CONSIDERATIONS
CONGENITAL HYPOTHALAMIC HAMARTOBLASTOMA, HYPOPITUITARISM, IMPERFORATE ANUS, AND POSTAXIAL POLYDACTYLY - A NEW SYNDROME .2. NEUROPATHOLOGICAL CONSIDERATIONS
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DOI：
10.1002/ajmg.1320070111
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发表时间：
1980-01-01
期刊：
影响因子：
--
通讯作者：
HALL, JG
中科院分区：
文献类型：
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作者：
CLARREN, SK;ALVORD, EC;HALL, JG
罕见的和几乎相同的下丘脑肿瘤最近在三个无关的新生儿类似的复杂畸形综合征进行了研究。下丘脑肿瘤或该综合征均未见文献报道，肿瘤位于大脑下表面，从视交叉延伸至脚间窝。肿瘤取代了下丘脑和起源于胚胎下丘脑板的其他核;它主要由类似原始未分化的生殖细胞的细胞组成。术语“错构母细胞瘤”用于指定这些肿瘤，以强调畸形和肿瘤方面。此外，短嗅束提示与无脑畸形野缺陷有关。
Unusual and virtually identical hypothalamic tumors were recently studied in three unrelated neonates with a similar complex malformation syndrome. Previous reports of either the hypothalamic tumors or the syndrome as a whole have not been found. Each tumor was apparent on the inferior surface of the cerebrum and extended from the optic chiasma to the interpeduncular fossa. The tumor replaced the hypothalamus and other nuclei which originate in the embryonic hypothalamic plate; it was principally composed of cells resembling primitive, undifferentiated germinal cells. The term “hamartoblastoma” is used to designate these tumors in order to emphasize the malformational and neoplastic aspects. In addition, short olfactory tracts suggest a relation to the arrhinencephaly field defect.