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胰腺破骨细胞样巨细胞瘤伴胆囊和淋巴结转移。病例报告。

Osteoclast-like giant cell tumor of the pancreas with metastases to gallbladder and lymph nodes. A case report.

作者信息

Sun A P, Ohtsuki Y, Liang S B, Sonobe H, Iwata J, Furihata M, Takeuchi T, Qiu Y, Chen B K, Watanabe R, Ohmori K

机构信息

Department of Pathology, Kochi Medical School, Japan.

出版信息

Pathol Res Pract. 1998;194(8):587-94; discussion 595. doi: 10.1016/S0344-0338(98)80051-4.

DOI:10.1016/S0344-0338(98)80051-4
PMID:9779494
Abstract

Osteoclast-like giant cell tumor of the pancreas (OGTP) is a rare neoplasm, of which the histogenesis is still controversial. Here we report a case of OGTP involving the head of the pancreas in a 71-year-old woman with metastases to the gallbladder and lymph nodes. The primary and metastatic tumors had identical histopathological, immunohistochemical, ultrastructural and molecular biological features. Microscopically, the tumors were characterized by atypical, often pleomorphic mononuclear cells associated with the proliferation of benign-appearing osteoclast-like giant cells (OGCs). Electron microscopic observation provided ultrastructural evidence of epithelial differentiation of the mononuclear cells, including microvilli and desmosomes, which was not obtained for OGCs. On immunohistochemical study, OGCs stained for CD68 (KP-1), LCA and HAM56, whereas mononuclear cells only reacted with PCNA. These findings clearly suggest that mononuclear cells are capable of differentiation and proliferation and may have been the only true tumor cells in this neoplasm, and that OGCs may have been a paraneoplastic product of this rare tumor. On examination of DNA from dewaxed sections of the tumor, we found no p53 mutation in the tumor tissue, but found two K-ras mutations in codon 12; this pattern of mutation commonly occurs in pancreatic carcinoma, indicating a somewhat genetic relationship of OGTP to pancreatic carcinoma. Although OGTP often has a favorable prognosis, the outcome in the present case was poor due to early tumor spread, with less than two years postoperative survival.

摘要

胰腺破骨细胞样巨细胞瘤(OGTP)是一种罕见的肿瘤,其组织发生仍存在争议。在此,我们报告一例发生于一名71岁女性胰头的OGTP,该肿瘤已转移至胆囊和淋巴结。原发肿瘤和转移肿瘤具有相同的组织病理学、免疫组织化学、超微结构和分子生物学特征。显微镜下,肿瘤的特征为非典型的、通常为多形性的单核细胞,伴有外观良性的破骨细胞样巨细胞(OGCs)增殖。电子显微镜观察提供了单核细胞上皮分化的超微结构证据,包括微绒毛和桥粒,而OGCs未观察到这些特征。免疫组织化学研究显示,OGCs对CD68(KP-1)、LCA和HAM56呈阳性染色,而单核细胞仅与PCNA反应。这些发现清楚地表明,单核细胞能够分化和增殖,可能是该肿瘤中唯一真正的肿瘤细胞,而OGCs可能是这种罕见肿瘤的副肿瘤产物。对肿瘤脱蜡切片的DNA进行检测时,我们在肿瘤组织中未发现p53突变,但在密码子12处发现了两个K-ras突变;这种突变模式常见于胰腺癌,表明OGTP与胰腺癌在一定程度上存在遗传关系。尽管OGTP通常预后良好,但本例患者由于肿瘤早期扩散,术后生存不足两年,预后较差。

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