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Pancreatic Mixed Acinar Carcinoma-Neuroendocrine Carcinoma

MedGen UID:
453216
•Concept ID:
C2063878
•
Neoplastic Process
Synonyms: Mixed Acinar-Endocrine Carcinoma of the Pancreas; Mixed Acinar-Neuroendocrine Carcinoma of the Pancreas; Mixed Carcinoid-Adenocarcinoma of the Pancreas; Mucinous Carcinoid Tumor of the Pancreas; Pancreatic Mixed Acinar Adenocarcinoma-Neuroendocrine Carcinoma; Pancreatic Mixed Acinar-Neuroendocrine Carcinoma

Definition

A carcinoma that arises from the pancreas and is composed of acinar carcinoma and neuroendocrine carcinoma components in both the primary tumor and the metastatic sites. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVPancreatic Mixed Acinar Carcinoma-Neuroendocrine Carcinoma

Recent clinical studies

Etiology

Integrated Genomic and Clinicopathologic Approach Distinguishes Pancreatic Grade 3 Neuroendocrine Tumor From Neuroendocrine Carcinoma and Identifies a Subset With Molecular Overlap.
Umetsu SE, Kakar S, Basturk O, Kim GE, Chatterjee D, Wen KW, Hale G, Shafizadeh N, Cho SJ, Whitman J, Gill RM, Jones KD, Navale P, Bergsland E, Klimstra D, Joseph NM
Mod Pathol 2023 Mar;36(3):100065. Epub 2023 Jan 10 doi: 10.1016/j.modpat.2022.100065. PMID: 36788102
Microsatellite instability in pancreatic and ampullary carcinomas: histology, molecular pathology, and clinical implications.
Luchini C, Scarpa A
Hum Pathol 2023 Feb;132:176-182. Epub 2022 Jun 14 doi: 10.1016/j.humpath.2022.06.009. PMID: 35714836
Pancreatic mixed acinar-neuroendocrine carcinoma in a patient with a germline BRCA2 mutation: a case report.
Ikeda M, Miura S, Kume K, Kikuta K, Hamada S, Takikawa T, Nakagawa K, Unno M, Furukawa T, Masamune A
Clin J Gastroenterol 2022 Oct;15(5):999-1005. Epub 2022 Jul 12 doi: 10.1007/s12328-022-01668-8. PMID: 35819745
Presentation and outcome of mixed neuroendocrine non-neuroendocrine neoplasms of the pancreas.
Nießen A, Schimmack S, Weber TF, Mayer P, Bergmann F, Hinz U, Büchler MW, Strobel O
Pancreatology 2021 Jan;21(1):224-235. Epub 2020 Dec 3 doi: 10.1016/j.pan.2020.11.020. PMID: 33309225
Acinar neoplasms of the pancreas-A summary of 25 years of research.
Klimstra DS, Adsay V
Semin Diagn Pathol 2016 Sep;33(5):307-18. Epub 2016 May 13 doi: 10.1053/j.semdp.2016.05.009. PMID: 27320062

Diagnosis

Integrated Genomic and Clinicopathologic Approach Distinguishes Pancreatic Grade 3 Neuroendocrine Tumor From Neuroendocrine Carcinoma and Identifies a Subset With Molecular Overlap.
Umetsu SE, Kakar S, Basturk O, Kim GE, Chatterjee D, Wen KW, Hale G, Shafizadeh N, Cho SJ, Whitman J, Gill RM, Jones KD, Navale P, Bergsland E, Klimstra D, Joseph NM
Mod Pathol 2023 Mar;36(3):100065. Epub 2023 Jan 10 doi: 10.1016/j.modpat.2022.100065. PMID: 36788102
The cytologic and immunohistochemical findings of pancreatic mixed acinar-endocrine carcinoma.
Whitehair R, Stelow EB
Diagn Cytopathol 2021 Feb;49(2):287-294. Epub 2020 Oct 31 doi: 10.1002/dc.24641. PMID: 33128511
Pancreatic acinar cell carcinoma: A review on molecular profiling of patient tumors.
Al-Hader A, Al-Rohil RN, Han H, Von Hoff D
World J Gastroenterol 2017 Dec 7;23(45):7945-7951. doi: 10.3748/wjg.v23.i45.7945. PMID: 29259370Free PMC Article
Acinar neoplasms of the pancreas-A summary of 25 years of research.
Klimstra DS, Adsay V
Semin Diagn Pathol 2016 Sep;33(5):307-18. Epub 2016 May 13 doi: 10.1053/j.semdp.2016.05.009. PMID: 27320062
Mixed acinar-neuroendocrine carcinoma of the pancreas: a case report and a review.
Jakobsen M, Klöppel G, Detlefsen S
Histol Histopathol 2016 Dec;31(12):1381-8. Epub 2016 Apr 15 doi: 10.14670/HH-11-767. PMID: 27081013

Therapy

Pancreatic acinar cell carcinoma: A review on molecular profiling of patient tumors.
Al-Hader A, Al-Rohil RN, Han H, Von Hoff D
World J Gastroenterol 2017 Dec 7;23(45):7945-7951. doi: 10.3748/wjg.v23.i45.7945. PMID: 29259370Free PMC Article
Clinical outcomes of patients with resectable pancreatic acinar cell carcinoma.
Seo S, Yoo C, Kim KP, Ryoo BY, Chang HM, Hong SM, Lee JH, Song KB, Hwang DW, Kim KH, Hwang S, Kim SC
J Dig Dis 2017 Aug;18(8):480-486. doi: 10.1111/1751-2980.12505. PMID: 28671770
Longterm follow-up of small pancreatic cystic lesions in liver transplant recipients.
Vidhyarkorn S, Siripongsakun S, Yu J, Sayre J, Agopian VG, Durazo F, Lu DS
Liver Transpl 2017 Mar;23(3):324-329. Epub 2017 Feb 8 doi: 10.1002/lt.24680. PMID: 27875639
A Rare Pancreatic Tumor That Underwent a Change in Morphology and Histopathologic Features During Chemotherapy.
Seino S, Tsuchiya A, Natsui M
Gastroenterology 2016 Feb;150(2):e11-3. Epub 2015 Dec 21 doi: 10.1053/j.gastro.2015.06.049. PMID: 26718170

Prognosis

Microsatellite instability in pancreatic and ampullary carcinomas: histology, molecular pathology, and clinical implications.
Luchini C, Scarpa A
Hum Pathol 2023 Feb;132:176-182. Epub 2022 Jun 14 doi: 10.1016/j.humpath.2022.06.009. PMID: 35714836
Paraneoplastic Cast Nephropathy Associated With Pancreatic Mixed Acinar-Neuroendocrine Carcinoma: A Case Report.
Nasr SH, Wehbe E, Said SM, Dasari S, Quoc T, Kurtin PJ
Am J Kidney Dis 2019 Oct;74(4):558-562. Epub 2019 Apr 2 doi: 10.1053/j.ajkd.2019.02.010. PMID: 30952487
Pancreatic acinar cell carcinoma: A review on molecular profiling of patient tumors.
Al-Hader A, Al-Rohil RN, Han H, Von Hoff D
World J Gastroenterol 2017 Dec 7;23(45):7945-7951. doi: 10.3748/wjg.v23.i45.7945. PMID: 29259370Free PMC Article
Clinical outcomes of patients with resectable pancreatic acinar cell carcinoma.
Seo S, Yoo C, Kim KP, Ryoo BY, Chang HM, Hong SM, Lee JH, Song KB, Hwang DW, Kim KH, Hwang S, Kim SC
J Dig Dis 2017 Aug;18(8):480-486. doi: 10.1111/1751-2980.12505. PMID: 28671770
Pathology and genetics of pancreatic neoplasms with acinar differentiation.
Wood LD, Klimstra DS
Semin Diagn Pathol 2014 Nov;31(6):491-497. Epub 2014 Oct 2 doi: 10.1053/j.semdp.2014.08.003. PMID: 25441307Free PMC Article

Clinical prediction guides

Cytomorphologic, immunophenotypical and molecular features of pancreatic acinar cell carcinoma.
Sun T, Gilani S, Jain D, Cai G
Diagn Cytopathol 2023 Nov;51(11):674-683. Epub 2023 Jul 19 doi: 10.1002/dc.25196. PMID: 37469257
Integrated Genomic and Clinicopathologic Approach Distinguishes Pancreatic Grade 3 Neuroendocrine Tumor From Neuroendocrine Carcinoma and Identifies a Subset With Molecular Overlap.
Umetsu SE, Kakar S, Basturk O, Kim GE, Chatterjee D, Wen KW, Hale G, Shafizadeh N, Cho SJ, Whitman J, Gill RM, Jones KD, Navale P, Bergsland E, Klimstra D, Joseph NM
Mod Pathol 2023 Mar;36(3):100065. Epub 2023 Jan 10 doi: 10.1016/j.modpat.2022.100065. PMID: 36788102
Microsatellite instability in pancreatic and ampullary carcinomas: histology, molecular pathology, and clinical implications.
Luchini C, Scarpa A
Hum Pathol 2023 Feb;132:176-182. Epub 2022 Jun 14 doi: 10.1016/j.humpath.2022.06.009. PMID: 35714836
Title: pancreatic-type mixed acinar neuroendocrine carcinoma of the stomach: a case report and review of the literature.
Ooe Y, Watanabe K, Hashimoto I, Takenaka S, Ojima T, Yamamoto S, Fujii H
Diagn Pathol 2021 Feb 2;16(1):11. doi: 10.1186/s13000-021-01070-x. PMID: 33531019Free PMC Article
Presentation and outcome of mixed neuroendocrine non-neuroendocrine neoplasms of the pancreas.
Nießen A, Schimmack S, Weber TF, Mayer P, Bergmann F, Hinz U, Büchler MW, Strobel O
Pancreatology 2021 Jan;21(1):224-235. Epub 2020 Dec 3 doi: 10.1016/j.pan.2020.11.020. PMID: 33309225

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