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Comprehensive review of undifferentiated carcinoma of the pancreas: from epidemiology to treatment

Journal

JAPANESE JOURNAL OF CLINICAL ONCOLOGY
Volume 53, Issue 9, Pages 764-773

Publisher

OXFORD UNIV PRESS
DOI: 10.1093/jjco/hyad062

Keywords

osteoclast-like giant cells; carcinosarcoma; sarcomatoid undifferentiated carcinoma; anaplastic carcinoma; undifferentiated carcinoma

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Undifferentiated carcinoma (UC) is an aggressive epithelial neoplasm with a poor prognosis, but recent advances in diagnostic modalities and genomic sequencing technologies are providing new possibilities for diagnosis and expanded treatments. UC of the pancreas is a rare subtype of pancreatic cancer with no definitive direction of differentiation. The World Health Organization (WHO) classifies UC into subtypes, and surgical resection is currently the only curative treatment, though there are promising results from paclitaxel-containing regimens for unresectable UC.
Undifferentiated carcinoma is an aggressive epithelial neoplasm with a poor prognosis. However, recent advances in diagnostic modalities and genomic sequencing technologies are opening up the possibilities for diagnosis and expanded treatments. Undifferentiated carcinoma (UC) of the pancreas is a rare subtype of pancreatic cancer displaying no definitive direction of differentiation. UC has been reported as a highly aggressive malignant neoplasm, with a median overall survival of <1 year, except for several surgical series. On the other hand, UC tissue sometimes contains non-neoplastic osteoclast-like giant cells (OGCs), and such cases have been reported to have relatively longer survival. Thus, the World Health Organization (WHO) classification histologically distinguishes UC with OGCs (UCOGCs) from UC, and UCs were subclassified into three subtypes: anaplastic UC, sarcomatoid UC and carcinosarcoma. However, still less is known about UC due to its rarity, and such situations lead to further difficulties in treatment for UC. To date, only surgical resection can offer curative treatment for patients with UC, and no clear evidence for chemotherapy exists for them. However, a retrospective cohort study and case reports showed that relatively promising results paclitaxel-containing regimens for treatment of patients with unresectable UC. Furthermore, high programmed cell death protein 1 expression has been reported in sarcomatoid UCs and UCOGCs, and promising responses to anti-programmed death-ligand 1 therapy have been described in case reports of UCOGCs. Recent advances in chemotherapeutic agents and molecular technologies are opening up the possibilities for expanded treatments.

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