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Review
. 2023 Aug 30;53(9):764-773.
doi: 10.1093/jjco/hyad062.

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

Hiroshi Imaoka  1 , Masafumi Ikeda  1 , Kumiko Umemoto  2 , Yu Sunakawa  2 , Makoto Ueno  3 , Hideki Ueno  4 , Masato Ozaka  5 , Takamichi Kuwahara  6 , Naohiro Okano  7 , Masashi Kanai  8 , Terumasa Hisano  9 , Yuko Suzuki  10 , Akinori Asagi  11 , Kazuhiko Shioji  12 , Akiko Todaka  13 , Kunihiro Tsuji  14 , Kenji Ikezawa  15 , Ikuya Miki  16 , Yoshito Komatsu  17 , Noriyuki Akutsu  18 , Tatsuya Yamashita  19 , Hiroyuki Okuyama  20 , Junji Furuse  3 , Hiroaki Nagano  21
Affiliations
Review

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

Hiroshi Imaoka et al. Jpn J Clin Oncol. .

Abstract

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.

Keywords: anaplastic carcinoma; carcinosarcoma; osteoclast-like giant cells; sarcomatoid undifferentiated carcinoma; undifferentiated carcinoma.

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Conflict of interest statement

Hiroshi Imaoka has received honoraria from Yakult Honsha. Masafumi Ikeda has received research funding from Eli Lilly Japan, Yakult, Pfizer and Takeda and honoraria from Eli Lilly Japan, Yakult Honsha, Taiho Pharmaceutical and Takeda. Kumiko Umemoto has received honoraria from Yakult Honsha. Makoto Ueno has received research funding from Taiho Pharmaceutical, AstraZeneca, Merck Biopharma, MSD, Astellas Pharma, Eisai, Ono Pharmaceutical, Incyte, CHUGAI PHARMACEUTICAL, DFP, Daiichi Sankyo, Novartis, Boehringer Ingelheim and J-pharma and honoraria from Taiho Pharmaceutical, AstraZeneca, Yakult Honsha, MSD, Nihon Servier, Ono Pharmaceutical, Incyte, CHUGAI PHARMACEUTICAL, Boehringer Ingelheim and J-pharma. Masato Ozaka has received honoraria from Taiho Pharmaceutical, Yakult Honsha, MSD, Ono Pharmaceutical, Nihon Servier, Bayer and Pfizer. Naohiro Okano has received honoraria from Taiho Pharmaceutical, Eli Lilly Japan, Eisai, Bayer Yakuhin, Chugai Pharma, Ono Pharmaceutical, Takeda, Daiichi Sankyo and AstraZeneca and is a member of Data Safety Monitoring Board or advisory board for GlaxoSmithKline. Kenji Ikezawa has received research funding from ASKA Pharmaceutical and honoraria from Taiho Pharmaceutical, Yakult Honsha, Ono Pharmaceutical, MSD, Myriad Genetics, Asahi Kasei Pharma and Incyte Biosciences Japan. Junji Furuse has received research funding from Taiho Pharmaceutical and Daiichi Sankyo and honoraria from Eli Lilly, Yakult Honsha, Taiho Pharmaceutical and Daiichi Sankyo and is a member of Data Safety Monitoring Board or advisory board for Taiho Pharmaceutical.

Figures

Figure 1
Figure 1
Representative case of anaplastic undifferentiated carcinoma (UC). (A) Computed tomography (CT) showing a hypovascular tumour at the body of the pancreas with upstream main pancreatic duct dilatation. (B) Tissue sample obtained by endoscopic ultrasound-guided tissue acquisition showing neoplastic cells, necrosis with neutrophil infiltration. In the high-powered field, (C) pleomorphic mononuclear neoplastic cells admixed with bizarre-appearing giant cells with eosinophilic cytoplasm were present.
Figure 2
Figure 2
Representative case of rhabdoid UC. (A) CT showing a hypovascular tumour at the tail of the pancreas. In the surgical specimen, (B) abundant pleomorphic neoplastic giant cells with rhabdoid inclusion were present.
Figure 3
Figure 3
Representative case of UC with osteoclast-like giant cells. (A) CT showing a large tumour at the tail of the pancreas. (B) Tissue sample obtained by EUS-TA showing a mixture of neoplastic pleomorphic mononuclear cells and multinucleated OGCs. (C) Multinucleated OGCs were immunohistochemically positive for CD68.
See this image and copyright information in PMC

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