Undifferentiated Carcinoma With Osteoclastic Giant Cells of the Pancreas: Clinicopathologic Analysis of 38 Cases Highlights a More Protracted Clinical Course Than Currently Appreciated.
Undifferentiated Carcinoma With Osteoclastic Giant Cells of the Pancreas: Clinicopathologic Analysis of 38 Cases Highlights a More Protracted Clinical Course Than Currently Appreciated.
复制标题
DOI：
10.1097/pas.0000000000000689
复制
发表时间：
2016-09
期刊：
影响因子：
--
通讯作者：
Adsay V
中科院分区：
文献类型：
--
作者：
Muraki T;Reid MD;Basturk O;Jang KT;Bedolla G;Bagci P;Mittal P;Memis B;Katabi N;Bandyopadhyay S;Sarmiento JM;Krasinskas A;Klimstra DS;Adsay V
胰腺骨巨细胞未分化癌是一种罕见的肿瘤。目前文献的印象是，它们是高度侵袭性的肿瘤，预后与导管腺癌相似。本研究对38例OGC手术切除的临床病理特征进行了研究，并与725例无骨肉瘤细胞（PDCs）的胰腺导管腺癌手术切除的临床病理特征进行对比。在系统回顾的胰腺癌中，发生率为1.4%。OGC显示女性略占优势（62.9%，PDCs为51.4%）。平均年龄为57.9岁（vs 65.0岁）。浸润性癌的平均大小为5.3 cm（vs3.2）。其特征为结节状、推缘生长，8例发生于肿瘤上皮内肿瘤[4例发生于粘液性囊性肿瘤（MCN），4例发生于导管内乳头状粘液性肿瘤（IPMN）型病变]，23例（61%）还显示出明显的导管内/囊内生长。29例（76%）有浸润性导管/管状腺癌成分。12例可见类骨质。尽管肿瘤体积较大，但神经周围浸润和淋巴结转移并不常见（分别为31.6%和22.6%，vs85.5%和64.0%）。免疫组化显示24例骨肉瘤细胞表达组织细胞标志物CD 68，背景梭形细胞和多形性/巨形癌细胞常显示p53，且常缺乏细胞角蛋白。OGC的生存率明显优于PDCs（5年，分别为59.1%和15.7%，p=0.0009）。总之，胰腺OGC的肿瘤体积更大，患者年龄比PDC稍小，21%发生在MCN/IPMN，61%显示导管内/囊内息肉样生长。OGC的预后比目前文献中认为的要好得多。
Undifferentiated carcinomas with osteoclastic giant cells of the pancreas (OGC) are rare tumors. The current impression in the literature is that they are highly aggressive tumors similar in prognosis to ductal adenocarcinomas. In this study, the clinicopathologic characteristics of 38 resected OGCs were investigated and contrasted with 725 resected pancreatic ductal adenocarcinomas without osteoclastic cells (PDCs). The frequency among systematically reviewed pancreatic cancers was 1.4%. OGCs showed a slight female predominance (62.9%, vs 51.4% in PDCs). The mean age was 57.9 years (vs 65.0). The mean size of invasive cancer was 5.3 cm (vs 3.2). They were characterized by nodular, pushing-border growth, and 8 arose in tumoral intraepithelial neoplasms [4 in mucinous cystic neoplasms (MCN), 4 in intraductal papillary mucinous neoplasms (IPMN) type lesions] and 23 (61%) also showed prominent intraductal/intracystic growth. Twenty nine (76%) had an invasive ductal/tubular adenocarcinoma component. Osteoid was seen in 12. Despite of their larger size, perineural invasion and nodal metastasis were uncommon (31.6 and 22.6%, vs 85.5 and 64.0% respectively). Immunohistochemistry performed on 24 cases revealed that osteoclastic cells expressed the histiocytic marker CD68, and background spindle cells and pleomorphic/giant carcinoma cells often showed p53 and often lacked cytokeratin. Survival of OGCs was significantly better than that of PDCs (5-year, 59.1 vs 15.7%, respectively, p=0.0009). In conclusion, pancreatic OGCs present with larger tumor size and in slightly younger patients than PDC, 21% arise in MCN/IPMN, and 61% show intraductal/intracystic polypoid growth. OGCs have a significantly better prognosis than is currently believed in the literature.