Rickert C H, Probst-Cousin S, Blasius S, Gullotta F
Institute of Neuropathology, Westfälische Wilhelms-Universität, Münster, Germany.
Childs Nerv Syst. 1998 Aug;14(8):389-93. doi: 10.1007/s003810050251.
So far, only 25 melanotic progonomas have been found in the central nervous system (male/female ratio 3.5), mostly located in the cerebellum. The average age is 8 years (range 3.5 months to 69 years) with 85% becoming clinically apparent in the first decade of life; 73.7% of the patients reported succumbed to their disease at a mean age of 2.8 years, with a postoperative survival time of just 9 months. Systemic metastases were reported in 9 cases and had mostly spread via cerebrospinal fluid. In contrast to peripheral melanotic progonomas usually found in the maxilla, cerebral progonomas have a much worse outcome and have to be regarded as malignant. We present the case of a 1-year-old boy suffering from a melanotic progonoma of the pineal gland, who died at the age of 22 months with extensive spinal and abdominal metastases 10 months after partial removal of the tumor.
迄今为止，仅在中枢神经系统发现25例黑素性原神经外胚层肿瘤（男女比例为3.5），大多位于小脑。平均年龄为8岁（范围为3.5个月至69岁），85%在生命的第一个十年出现临床症状；报告的患者中有73.7%在平均年龄2.8岁时死于该病，术后生存时间仅为9个月。9例报告有全身转移，大多通过脑脊液扩散。与通常发生在上颌骨的外周黑素性原神经外胚层肿瘤不同，脑原神经外胚层肿瘤预后要差得多，必须视为恶性肿瘤。我们报告一例1岁患松果体黑素性原神经外胚层肿瘤的男孩病例，该患儿在肿瘤部分切除10个月后，于22个月龄时死于广泛的脊柱和腹部转移。
