Undifferentiated Carcinoma With Osteoclastic Giant Cells of the Pancreas

Abstract

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.

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

“… 3 , 7 , 9 - 12 The mean tumor size in reported cases was 5-8 cm. 6 , 7 , 9 - 12 The tumor is usually a heterogeneous mass that can cause compression of adjacent normal structures, or constitutional symptoms such as fatigue and weight loss. 3 , 7 , 9 - 12 Two-thirds of cases in the literature reported abdominal or back pain, one-third presented with painless jaundice.…”

“… 5 UCP has a much poorer prognosis than typical ductal adenocarcinoma of the pancreas. 1 , 6 Survival in some reports has been on average as low as 5 months from the time of diagnosis. 3 , 6 Based on histologic features, UCP subtypes and UC-OGC have notable overlap, such as the presence of heterologous elements including osteoid, muscle and cartilage.…”

“…1 , 6 Survival in some reports has been on average as low as 5 months from the time of diagnosis. 3 , 6 Based on histologic features, UCP subtypes and UC-OGC have notable overlap, such as the presence of heterologous elements including osteoid, muscle and cartilage. 6 However, the presence of OGC’s is a defining characteristic of UC-OGC in the World Health Organization (WHO) classification of pancreatic malignancies.…”

“… 3 , 6 Based on histologic features, UCP subtypes and UC-OGC have notable overlap, such as the presence of heterologous elements including osteoid, muscle and cartilage. 6 However, the presence of OGC’s is a defining characteristic of UC-OGC in the World Health Organization (WHO) classification of pancreatic malignancies. 7 This entity makes up less than 1% of pancreatic malignancies and has a distinct histopathological and clinical profile.…”

PD-1, PD-L1, and CD163 in pancreatic undifferentiated carcinoma with osteoclast-like giant cells: expression patterns and clinical implications

“…In addition, differences in somatic mutations are unlikely to explain the unique clinical course of UCOGC, with prolonged survival particularly in cases of "pure" UCOGC (i.e. not PDAC-associated) [7,8].…”