Gerald W L, Miller H K, Battifora H, Miettinen M, Silva E G, Rosai J
Department of Pathology, Yale University Medical School, New Haven, Connecticut.
Am J Surg Pathol. 1991 Jun;15(6):499-513.
Nineteen cases of a distinctive type of malignant small-cell tumor are presented. The main features of the entity are as follows: a predilection for adolescent males (mean age: 18.6 years); predominant or exclusive intra-abdominal location, with only inconstant and secondary organ involvement; nesting pattern of growth; focal rhabdoid features; intense desmoplastic reaction; immunohistochemical reactivity for epithelial [keratin, epithelial membrane antigen (EMA)], neural [neuron-specific enolase (NSE)], and muscle (desmin) markers; and highly aggressive behavior. It is proposed that this represents yet another member of the continuously enlarging and evolving family of small round (blue) cell tumors of infancy and childhood that features, more than any other member of this group, the capacity for simultaneous multidirectional phenotypical expression.
本文报告了19例一种独特类型的恶性小细胞肿瘤。该肿瘤实体的主要特征如下：好发于青春期男性（平均年龄：18.6岁）；主要或仅位于腹腔内，仅有不恒定且继发的器官受累；呈巢状生长模式；具有局灶性横纹肌样特征；有强烈的促纤维组织增生反应；对上皮（角蛋白、上皮膜抗原[EMA]）、神经（神经元特异性烯醇化酶[NSE]）和肌肉（结蛋白）标志物具有免疫组化反应性；以及具有高度侵袭性的行为。有人提出，这代表了婴幼儿和儿童期不断扩大和演变的小圆形（蓝色）细胞肿瘤家族中的又一个成员，该家族比该组中的任何其他成员更具同时进行多向表型表达的能力。
