Yolk sac tumor with a unique uniform hepatoid pattern histology.

A Konno, M Kudo, T Ichihara, M Yamagami, S Horita, K Ohama, K Taketa, S Koizumi
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引用次数: 3

Abstract

Background: Yolk sac tumors (YST) exhibit several different histological subtypes and the mechanisms of cellular differentiation and prognosis of each subtype remain unknown.

Results: We report two infants with sacrococcygeal YST; one represented a typical histological subtype and the other showed a hepatoid subtype with uniform histology. The isoform of alpha-fetoprotein (AFP) in the patient with the hepatoid pattern was examined by lectin-affinity immunoelectrophoresis and represented as a YST, but not hepatocellular, subtype. The patient with typical YST responded well to VAB-6 combination chemotherapy. However, this regimen was only partially effective to the patient with the pure hepatoid histological subtype, and an etoposide with ifosfamide and cisplatin (VIP) regimen as a salvage chemotherapy combined with complete tumor resection was useful to achieve complete remission (CR). Both of the patients have been in CR for more than four years.

卵黄囊肿瘤具有独特统一的肝样组织学。
背景:卵黄囊肿瘤(YST)表现出几种不同的组织学亚型,每种亚型的细胞分化和预后机制尚不清楚。结果:我们报告了2例婴儿骶尾骨囊肿;一个代表典型的组织学亚型,另一个显示具有统一组织学的类肝亚型。通过凝集素亲和免疫电泳检测了肝样型患者的甲胎蛋白(AFP)亚型,并将其表示为YST亚型,而不是肝细胞亚型。典型YST患者对VAB-6联合化疗反应良好。然而,该方案仅对纯肝样组织学亚型患者部分有效,依托泊苷与异环磷酰胺和顺铂(VIP)方案作为补救性化疗联合完全肿瘤切除术有助于实现完全缓解(CR)。两例患者的CR均超过4年。
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