Bond J V
Arch Dis Child. 1976 Nov;51(11):879-82. doi: 10.1136/adc.51.11.879.
Four infants are described who presented with rapid enlargement of the liver. This was found to be due to neuroblastoma which had metastasized to the liver; the condition was associated with high levels of urinary vanillylmandelic acid (VMA). In 3 infants the primary tumour was in the adrenal gland and in one it was not identified. One infant died after laparotomy and 2 infants survive disease free with normal VMA levels, one after adrenalectomy and hepatic irradiation and one after a short course of chemotherapy. The fourth patient responded initially to hepatic irradiation and chemotherapy, but relapsed 2 years later with recurrent disease; at the same time the VMA level which had been normal, again rose. The importance of screening for an abnormal VMA level in any infant with a rapidly enlarging liver in order to obtain an early diagnosis is stressed. Careful follow-up, with serial VMA estimations, is essential to detect recurrent disease. The prognosis for some infants with this distribution of neuroblastoma which has metastasized to the liver, but not to the bones or oribt, is good.
本文描述了4例肝脏迅速增大的婴儿。发现这是由于神经母细胞瘤转移至肝脏所致；该病症与尿香草扁桃酸（VMA）水平升高有关。3例婴儿的原发肿瘤位于肾上腺，1例未明确。1例婴儿在剖腹手术后死亡，2例婴儿VMA水平正常且无疾病存活，1例在肾上腺切除和肝脏放疗后存活，另1例在短期化疗后存活。第4例患者最初对肝脏放疗和化疗有反应，但2年后疾病复发；同时，原本正常的VMA水平再次升高。强调了对任何肝脏迅速增大的婴儿进行VMA水平异常筛查以获得早期诊断的重要性。通过连续测定VMA进行仔细随访对于检测疾病复发至关重要。一些神经母细胞瘤转移至肝脏但未转移至骨骼或眼眶的婴儿预后良好。
