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      Undifferentiated carcinoma of the pancreas with osteoclast-like giant cells reported in an asymptomatic patient: a rare case and literature review

      case-report

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          Abstract

          Undifferentiated carcinoma of the pancreas with osteoclast-like giant cells (UC-OGC) is a rare and poorly described pancreatic malignancy. It is comprised of mononuclear, pleomorphic, and undifferentiated cells as well as osteoclast-like giant cells (OGC’s). It constitutes less than 1% of pancreatic non-endocrine neoplasia and is twice as likely to occur in females as in males. Its histopathologic properties remain poorly understood. It is suspected that UC-OGC is of epithelial origin that can then transition to mesenchymal elements. As part of this study, we describe a case of a malignant pancreatic neoplasm that was discovered in a 69-year old patient as an incidental finding. We also provide an overview of previously published data to highlight UC-OGC’s clinical and pathologic features.

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          Undifferentiated Carcinoma With Osteoclastic Giant Cells of the Pancreas: Clinicopathologic Analysis of 38 Cases Highlights a More Protracted Clinical Course Than Currently Appreciated.

          Takashi Muraki, Michelle D Reid, Olca Basturk … (2016)
          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.
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            Pancreatic undifferentiated carcinoma with osteoclast-like giant cells is genetically similar to, but clinically distinct from, conventional ductal adenocarcinoma.

            Claudio Luchini, Antonio Pea, Gemma Lionheart … (2017)
            Undifferentiated carcinoma of the pancreas with osteoclast-like giant cells (UCOGC) is currently considered a morphologically and clinically distinct variant of pancreatic ductal adenocarcinoma (PDAC). In this study, we report clinical and pathological features of a series of 22 UCOGCs, including the whole exome sequencing of eight UCOGCs. We observed that 60% of the UCOGCs contained a well-defined epithelial component and that patients with pure UCOGC had a significantly better prognosis than did those with an UCOGC with an associated epithelial neoplasm. The genetic alterations in UCOGC are strikingly similar to those known to drive conventional PDAC, including activating mutations in the oncogene KRAS and inactivating mutations in the tumor suppressor genes CDKN2A, TP53, and SMAD4. These results further support the classification of UCOGC as a PDAC variant and suggest that somatic mutations are not the determinants of the unique phenotype of UCOGC. Copyright © 2017 Pathological Society of Great Britain and Ireland. Published by John Wiley & Sons, Ltd.
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              Huge undifferentiated carcinoma of the pancreas with osteoclast-like giant cells.

              Sungho Jo (2014)
              Undifferentiated carcinoma of the pancreas with osteoclast-like giant cells (OGCs) is very rare, less than 1% of all pancreatic malignancies, and shows worse prognosis than that of invasive ductal adenocarcinoma of the pancreas. We present a case of en bloc resection for a huge undifferentiated carcinoma with OGCs that invaded the stomach and transverse mesocolon. A 67-year female was admitted for left upper quadrant pain and computed tomography demonstrated a mass occupying the lesser sac and abutting the stomach and pancreas. There were no distant metastases and the patient underwent subtotal pancreatectomy with splenectomy, total gastrectomy, and segmental resection of the transverse colon. Histopathological examination confirmed an 11 cm-sized undifferentiated carcinoma of the pancreas with OGCs. Immunohistochemical staining revealed reactivity with pan-cytokeratin in adenocarcinoma component, with vimentin in neoplastic multi-nucleated cells, with CD45/CD68 in OGCs, and with p53 in tumor cells, respectively. The patient had suffered from multiple bone metastases and survived 9 mo after surgery. This case supports the ductal epithelial origin of undifferentiated carcinoma with OGCs and early diagnosis could result in favorable surgical outcomes. Investigations on the surgical role and prognostic factors need to be warranted in this tumor.
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                Author and article information

                Journal
                Journal ID (nlm-ta): Autops Case Rep
                Journal ID (iso-abbrev): Autops Case Rep
                Title: Autopsy & Case Reports
                Publisher: São Paulo, SP: Universidade de São Paulo, Hospital Universitário
                ISSN (Electronic): 2236-1960
                Publication date (Electronic): 8 December 2017
                Publication date Collection: Oct-Dec 2017
                Volume: 7
                Issue: 4
                Pages: 51-57
                Affiliations
                [a ]St. John Hospital and Medical Center, Department of Pathology . Detroit, MI, USA.
                [b ]Vanderbilt University Medical Center, Department of Pathology . Nashville, TN, USA.
                Author notes

                Author contributions: All the authors made significant contribution to the manuscript. Sakhi R designed and wrote the manuscript after gathering all required information. He also grossed and assisted in signing out the case. Hamza A wrote significant portions of the introduction and discussion and edited the entire manuscript. Khurram MS did the literature search and made valuable additions to the discussion. Ibrar W took the pictures, and wrote the figure descriptions. Mazzara P was the staff pathologist who signed out the case. He also proof read the manuscript and provided valuable input to improve it.

                Conflict of interest: None

                Correspondence
Ramen Sakhi
Department of Pathology - St John Hospital and Medical Center
22101 Moross Rd, 48236 – Detroit/MI – USA
Phone: +1 (131) 409-0234
 ramen_sakhi@ 123456hotmail.com
                Article
                Publisher ID: autopsy-07-04051
                DOI: 10.4322/acr.2017.042
                PMC ID: 5724056
                PubMed ID: 29259932
                SO-VID: 8871553c-d29e-4d66-b69f-523e6b56f604
                Copyright © Autopsy and Case Reports. ISSN 2236-1960. Copyright © 2017.
                License:

                This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License, which permits unrestricted non-commercial use, distribution, and reproduction in any medium provided the article is properly cited.

                History
                Date received : 17 October 2017
                Date accepted : 19 November 2017
                Categories
                Subject: Article / Clinical Case Report

                Keywords: adenocarcinoma,carcinoma, pancreatic ductal,osteoclasts,pancreatic neoplasms
                Data availability:
                Keywords: adenocarcinoma, carcinoma, pancreatic ductal, osteoclasts, pancreatic neoplasms

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