Assessment and management of undifferentiated carcinoma with osteoclastic like giant cells of the pancreas: a case report and revision of literature

Abstract

Background Undifferentiated carcinoma with osteoclast-like giant cells (UCOGCs) is a rare and aggressive non endocrine pancreatic carcinoma characterized by the presence of osteoclastic giant cells mixed with mononuclear cell. Very few cases have been reported in the literature and the histogenesis is controversial as, at the time of diagnosis, the tumor is often of advanced size and stage and it is difficult to pathologically observe its relationship with the pancreatic duct. Case presentation We present a case of 65-year-old male patient presenting with abdominal pain, nausea, and weight loss, which was treated with surgical resection. Histological examination revealed an undifferentiated pancreatic carcinoma with osteoclast-like giant cells. The patient underwent to a routine pylorus preserving pancreatoduodenectomy. Actually, the patient was in good performance status and disease-free five months. Conclusions Based on the present case and limited previous data, further researches preferably with large cohorts are necessary to clarify the pathogenesis of the neoplasm. However, as show in this case, histopathological and immunohistochemically studies are the gold standard for the diagnosis of UCPOGC. Investigation of the genomic alterations in UPOGCs could help to explain the histologic diversity of variant tumor and could provide a genetic basis for prognosis and treatment.

Undifferentiated Pancreatic Carcinomas, Clinical Features and Therapeutic Options: What We Know

“…This is the most frequently reported subtype of undifferentiated pancreatic carcinoma [ 3 , 7 , 8 , 9 , 10 , 11 , 12 , 13 , 14 , 15 , 16 , 17 , 18 , 19 ]. UCOGCs are pathologically characterized by the presence of giant cells that resemble the giant cells of the bone; hence, they are called osteoclast-like giant cells [ 5 ].…”

“…The immunohistochemistry profiles of undifferentiated pancreatic carcinoma have been reported in numerous studies. They are characterized by positive pan-cytokeratin (CKAE1/AE3) in the mononuclear pleomorphic cells [ 8 , 9 , 17 ]. Pleomorphic cells may also exhibit a low Ki-67 labeling index [ 8 , 14 ].…”

“…In rhabdoid pancreatic carcinoma, the neoplastic cells express a loss of INI1 expression [ 16 , 34 , 35 , 36 ]. Osteoclastic-like giant cells are consistently non-reactive with CKAE1/AE3 (pan-cytokeratin) [ 8 , 9 , 12 ]. They have a strong reactivity to histiocytic markers, predominantly CD68.…”

“…Genetic variations in undifferentiated pancreatic carcinoma have some commonalities with PDAC. There are frequent alterations in KRAS, TP53 and CDKN2A [ 3 , 9 , 14 , 22 , 23 ]. KRAS mutations may be absent in some cases, especially in the rhabdoid subtype [ 30 , 33 , 36 ].…”

“…These tumors have three histological classifications: pleomorphic, osteoclastic and mixed-type [ 5 , 6 ]. In the literature, they are mostly reported as undifferentiated carcinomas with osteoclast-like giant cells (UCOGC) [ 3 , 7 , 8 , 9 , 10 , 11 , 12 , 13 , 14 , 15 , 16 , 17 , 18 , 19 ], giant cell pancreatic carcinoma [ 20 ], sarcomatoid carcinoma of the pancreas [ 21 , 22 ], or rhabdoid pancreatic carcinomas [ 23 , 24 , 25 ]. However, the histogenesis of these carcinomas remains controversial.…”