Immunohistochemical and electron microscopic studies of a case of duodenal gangliocytic paraganglioma
Immunohistochemical and electron microscopic studies of a case of duodenal gangliocytic paraganglioma
复制标题
DOI：
10.1007/s00795-009-0442-2
复制
发表时间：
2009-12-01
影响因子：
1.8
通讯作者：
Furihata, Mutsuo
中科院分区：
文献类型：
--
作者：
Ohtsuki, Yuji;Watanabe, Ryohei;Furihata, Mutsuo
神经节细胞性副神经节瘤（GPG）是一种罕见的肿瘤，几乎只发生在十二指肠。本例中，粘膜下肿瘤位于 Vater 乳头肛门侧，大小为 2.5 x 2 x 1.5 cm，切面检查边缘清晰，无包膜。肿瘤细胞包括三种类型的细胞：神经节样细胞（GC）、内分泌细胞（EC）和施万细胞（SC）。 GC 较大，细胞核偏心，核仁较大，细胞质清晰丰富。在小巢中检测到 EC，周围环绕着 SC 束。免疫组织化学显示，GC 突触素、神经元特异性烯醇化酶 (NSE) 和 CD56 呈阳性。 EC 的嗜铬粒蛋白 A、NSE、生长抑素、胰多肽和 CD56 呈阳性，并且与 S100 蛋白阳性 SC 相关。精细结构检查显示，EC 的细胞质中含有大量膜边界分泌颗粒，直径为 250-450 nm，周围有分支的复杂基底层。 SCs 沿着其长的交错的细胞质过程具有基底层。 GPG 的组织发生很可能涉及十二指肠隐窝中多能干细胞作为真正肿瘤的增殖和分化，尽管 GC 和 SC 的腹膜后成分也可能与 EC 一起从十二指肠中胰腺的腹侧原始组织增殖。报道了一例 GPG 的免疫组织化学和超微结构发现，重点关注三个主要细胞成分：GC、EC 和 SC。
Gangliocytic paraganglioma (GPG) is a rare tumor, occurring almost exclusively in the duodenum. In the present case, a submucosal tumor 2.5 x 2 x 1.5 cm in size was located on the anal side of the papilla of Vater, with clear margins and without capsule on cut-surface examination. Tumor cells included three types of cells: ganglion-like cells (GCs), endocrine cells (ECs), and Schwann cells (SCs). The GCs were large with eccentric nuclei with large nucleoli and clear abundant cytoplasm. ECs were detected in small nests, surrounded by bundles of SCs. Immunohistochemically, GCs were positive for synaptophysin, neuron-specific enolase (NSE), and CD56. ECs were positive for chromogranin A, NSE, somatostatin, pancreatic polypeptide, and CD56, and were associated with S100 protein-positive SCs. On fine structural examination, ECs contained numerous membrane-bounded secretory granules, 250-450 nm in diameter, in their cytoplasm, surrounded by a branched, complex basal lamina. SCs possessed basal lamina along their long interlacing cytoplasmic processes. The histogenesis of GPG most likely involves proliferation and differentiation of pluripotent stem cells in the duodenal crypts in the duodenum as a true tumor, although it is also possible that the retroperitoneal components of both GCs and SCs proliferate, together with ECs, from ventral primordial tissue of the pancreas in the duodenum. The immunohistochemical and ultrastructural findings of a case of GPG are reported, focusing on three major cellular components: GCs, ECs, and SCs.