Ruck P, Kaiserling E
Institute of Pathology, Eberhard-Karls University, Tübingen, Germany.
Cancer. 1993 Jul 15;72(2):361-8. doi: 10.1002/1097-0142(19930715)72:2<361::aid-cncr2820720209>3.0.co;2-3.
The authors previously have reported that hepatoblastomas may exhibit endocrine differentiation. This report describes a hepatoblastoma in which a melanocytic component was present in addition to endocrine differentiation.
The tumor, which arose in a 15-month-old girl, was subjected to conventional histologic, histochemical, immunohistochemical, and electron microscopic investigation.
The tumor had fetal and embryonal epithelial areas and osteoid. The presence of melanin could be suspected, even on the basis of gross examination. The melanin was found predominantly in macrophages but also was present in a few epithelial tumor cells. The tumor also had HMB45-immunoreactive melanocytic cells, and correspondingly, cells containing dopa-oxidase, an enzyme essential for melanin synthesis. Staining for chromogranin A and serotonin was seen in fetal-type cells, embryonal-type cells, and in epithelial cells of reactive bile ductules at the periphery of the tumor.
Primary melanin-containing tumors of the liver are extremely rare; only one such tumor, referred to as a "teratoid hepatoblastoma," previously has been described in detail. The combination of endocrine and melanocytic differentiation has not been reported previously in liver tumors but occurs in endocrine tumors of other organs. Although it is not possible to define exactly the histogenesis of the melanocytic cells in this tumor, it is most likely that these cells and the other components of the tumor derive from a pluripotent entodermal stem cell by multidirectional differentiation.
作者此前曾报道肝母细胞瘤可能表现出内分泌分化。本报告描述了一例除内分泌分化外还存在黑素细胞成分的肝母细胞瘤。
对一名15个月大女孩体内发生的肿瘤进行常规组织学、组织化学、免疫组织化学和电子显微镜检查。
肿瘤具有胎儿型和胚胎型上皮区域以及类骨质。即使在大体检查的基础上,也可怀疑有黑色素存在。黑色素主要存在于巨噬细胞中,但也存在于少数上皮肿瘤细胞中。肿瘤中还存在HMB45免疫反应性黑素细胞,相应地,还存在含有多巴氧化酶的细胞,多巴氧化酶是黑色素合成所必需的一种酶。在胎儿型细胞、胚胎型细胞以及肿瘤周边反应性胆小管的上皮细胞中可见嗜铬粒蛋白A和5-羟色胺染色。
原发性含黑色素的肝肿瘤极为罕见;此前仅详细描述过一例此类肿瘤,称为“畸胎样肝母细胞瘤”。内分泌和黑素细胞分化的组合此前在肝肿瘤中未见报道,但在其他器官的内分泌肿瘤中出现。虽然无法确切界定该肿瘤中黑素细胞的组织发生,但很可能这些细胞以及肿瘤的其他成分是由多能内胚层干细胞通过多向分化产生的。