Muraki Takashi, Reid Michelle D, Basturk Olca, Jang Kee-Taek, Bedolla Gabriela, Bagci Pelin, Mittal Pardeep, Memis Bahar, Katabi Nora, Bandyopadhyay Sudeshna, Sarmiento Juan M, Krasinskas Alyssa, Klimstra David S, Adsay Volkan
Departments of *Pathology and Laboratory Medicine ∥Radiology #Surgery, Emory University School of Medicine, Atlanta, GA †Department of Pathology, Memorial Sloan Kettering Cancer Center, New York, NY ¶Department of Pathology, Detroit Medical Center, Detroit, MI ‡Department of Pathology and Translational Genomics, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea §Department of Pathology, Marmara University, Istanbul, Turkey.
Am J Surg Pathol. 2016 Sep;40(9):1203-16. doi: 10.1097/PAS.0000000000000689.
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, 4 in intraductal papillary mucinous neoplasms 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 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 yr, 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 mucinous cystic neoplasms/intraductal papillary mucinous neoplasms, and 61% show intraductal/intracystic polypoid growth. OGCs have a significantly better prognosis than is currently believed in the literature.
胰腺伴有破骨细胞样巨细胞的未分化癌(OGC)是罕见肿瘤。目前文献中的印象是,它们是高度侵袭性肿瘤,预后与导管腺癌相似。在本研究中,对38例切除的OGC的临床病理特征进行了研究,并与725例切除的无破骨细胞的胰腺导管腺癌(PDC)进行了对比。在系统回顾的胰腺癌中,其发生率为1.4%。OGC以女性略占优势(62.9%,而PDC为51.4%)。平均年龄为57.9岁(而PDC为65.0岁)。浸润性癌的平均大小为5.3 cm(而PDC为3.2 cm)。其特征为结节状、推挤性边界生长,8例起源于肿瘤上皮内瘤变(4例在黏液性囊性肿瘤中,4例在导管内乳头状黏液性肿瘤样病变中),23例(61%)也表现出显著的导管内/囊内生长。29例(76%)有浸润性导管/小管腺癌成分。12例可见骨样组织。尽管其肿瘤较大,但神经周围侵犯和淋巴结转移并不常见(分别为31.6%和22.6%,而PDC分别为85.5%和64.0%)。对24例进行的免疫组织化学显示,破骨细胞样细胞表达组织细胞标志物CD68,背景梭形细胞和多形性/巨癌细胞常显示p53,且常缺乏细胞角蛋白。OGC的生存率明显优于PDC(5年生存率分别为59.1%和15.7%,P = 0.0009)。总之,胰腺OGC比PDC的肿瘤更大,患者年龄略小,21%起源于黏液性囊性肿瘤/导管内乳头状黏液性肿瘤,61%表现为导管内/囊内息肉样生长。OGC的预后明显好于目前文献中的认识。