Acinar cell carcinoma of pancreas

A very rare, malignant, epithelial tumor of the pancreas characterized, macroscopically, by a usually large, well-circumscribed, fully or partially encapsulated, solid mass, often with hemorrhage, necrosis and cystic changes, in any portion of the pancreas and, histologically, by neoplastic cells with variable degrees of differentiation and morphology, ranging from acinar structures similar to normal pancreatic acini to large sheets of poorly differentiated neoplastic cells. Presenting symptoms are typically non-specific and include abdominal pain, weight loss, vomiting, nausea, and/or, less commonly, jaundice. Immunohistochemical evidence of acinar-specific products is observed. Association with Lynch syndrome, familial adenomatous polyposis, and pancreatic panniculitis has been reported.



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Total: 70 (papers)

   


(per page)
Matched Phenotype  Gene  Mutation  MeSH
Rank
(Similarity)		 PMID
(PMCID)
1
(21.2%)		 27112929
 Pancreatic panniculitis as a paraneoplastic phenomenon of a pancreatic acinar cell carcinoma.
Naeyaert C, de Clerck F, De Wilde V.
Acta Clin Belg. 2016;71(6):448-450.
Arthritis
Acinar Cell Carcinoma Biopsy Differential Diagnosis Homo sapiens Male Middle Aged Pancreas Pancreatic Diseases Pancreatic Neoplasm Panniculitis Paraneoplastic Syndromes X-Ray Computed Tomography
1
(21.2%)		 25959244
 Pancreatic acinar cell carcinoma with bilateral ovarian metastases, panniculitis and polyarthritis treated with FOLFIRINOX chemotherapy regimen. A case report and review of the literature.
Callata-Carhuapoma HR, Pato Cour E, Garcia-Paredes B, Fernandez RM, Mendoza Fernandez ML, Fernandez AM, De La Rosa CA, Sotelo Lezama MJ, Cabezas-Camarero S, Sastre Varela J.
Pancreatology. 2015;15(4):440-4.
Arthritis
Acinar Cell Carcinoma Adult Antineoplastic Combined Chemotherapy Protocols Arthritis Camptothecin Fatal Outcome Females Homo sapiens Organoplatinum Compounds Pancreatic Neoplasm Panniculitis Peritoneal Neoplasms X-Ray Computed Tomography
3
(17.5%)		 31002019
 Pancreatic acinar cell carcinoma is associated with BRCA2 germline mutations: a case report and literature review.
Kryklyva V, Haj Mohammad N, Morsink FHM, Ligtenberg MJL, Offerhaus GJA, Nagtegaal ID, de Leng WWJ, Brosens LAA.
Cancer Biol Ther. 2019;20(7):949-955.
Neoplasm Panniculitis
ACCS BRCA1 BRCA2 CHEK2 PARP1
Acinar Cell Carcinoma BRCA1 Protein BRCA2 Protein Biomarkers, Tumor Biopsy Disease Management Genetic Association Studies Genetic Predisposition to Disease Germ-Line Mutation Homo sapiens Immunohistochemistry Male Middle Aged Nucleic Acid Amplification Techniques Pancreatic Neoplasm Precision Medicine
3
(17.5%)		 29527901
 Pancreatic panniculitis as a presentation symptom of acinar cell carcinoma.
de Frutos Rosa D, Espinosa Taranilla L, Gonzalez de Canales de Simon P, Velez Velazquez MD, Guirado Koch C.
Rev Esp Enferm Dig. 2018;110(5):329-331.
Panniculitis
Acinar Cell Carcinoma Fatal Outcome Females Homo sapiens Middle Aged Pancreatic Diseases Pancreatic Neoplasm Panniculitis
3
(17.5%)		 29082362
(5656292)
 An Unexpected Etiology of Pancreatic Panniculitis: A Case Report.
Evans AC, Singhi AD, Zeh HJ Rd, Bahary N, Brand RE.
J Pancreat Cancer. 2017;3(1):1-4.
Pancreatitis Panniculitis
3
(17.5%)		 28588779
(5451886)
 Pancreatic acinar cell carcinoma presenting with panniculitis, successfully treated with FOLFIRINOX: A case report.
Yoshihiro T, Nio K, Tsuchihashi K, Ariyama H, Kohashi K, Tsuruta N, Hanamura F, Inadomi K, Ito M, Sagara K, Okumura Y, Nakano M, Arita S, Kusaba H, Oda Y, Akashi K, Baba E.
Mol Clin Oncol. 2017;6(6):866-870.
Edema Panniculitis
CELA1 KRT19 KRT20 NKX2-1 PLA2G1B
3
(17.5%)		 26456669
 Multimodal approach and long-term survival in a patient with recurrent metastatic acinar cell carcinoma of the pancreas: A case report.
Jauch SF, Morris VK, Jensen CT, Kaseb AO.
Pancreatology. 2016;16(1):153-6.
Neoplasm Panniculitis
Acinar Cell Carcinoma Antineoplastic Agents Females Homo sapiens Middle Aged Neoplasm Recurrence, Local Organoplatinum Compounds Pancreatic Neoplasm
3
(17.5%)		 21546712
 Acinar cell carcinoma of the pancreas associated with subcutaneous panniculitis.
Moro M, Moletta L, Blandamura S, Sperti C.
JOP. 2011;12(3):292-6.
Subcutaneous nodule Panniculitis
Acinar Cell Carcinoma Differential Diagnosis Females Homo sapiens Pancreatic Neoplasm Panniculitis X-Ray Computed Tomography
3
(17.5%)		 20703831
 Functional pancreatic acinar cell carcinoma extending into the main pancreatic duct and splenic vein.
Iwatate M, Matsubayashi H, Sasaki K, Kishida N, Yoshikawa S, Ono H, Maitra A.
J Gastrointest Cancer. 2012;43(2):373-8.
Subcutaneous nodule Panniculitis
Acinar Cell Carcinoma Fatal Outcome Homo sapiens Male Pancreatic Neoplasm Panniculitis Paraneoplastic Syndromes Positron-Emission Tomography
3
(17.5%)		 9823560
 [Hepatic artery ligation for liver metastasis of a acinar cell carcinoma of the pancreas revealed by a nodular panniculitis].
Viguier J, D'Alteroche L, Regimbeau C, Picon L, Jan V, De Muret A, Benchellal ZA, Metman EH.
Gastroenterol Clin Biol. 1998;22(8-9):715-9.
Panniculitis
Acinar Cell Carcinoma Fatal Outcome Homo sapiens Ligation Male Pancreatic Neoplasm Panniculitis, Nodular Nonsuppurative
        

Phenotype(s) retrieved from Orphanet

    Total: 0

HPO ID Term Frequency


Phenotype(s) retrieved from case reports

    Total: 11

HPO ID Term # of case reports
HP:0002664 Neoplasm 4
HP:0002835 Aspiration 4
HP:0012490 Panniculitis 3
HP:0001482 Subcutaneous nodule 2
HP:0030731 Carcinoma 2
HP:0000112 Nephropathy 1
HP:0001733 Pancreatitis 1
HP:0001824 Weight loss 1
HP:0001919 Acute kidney injury 1
HP:0100003 Peritoneal mesothelioma 1
HP:0100570 Carcinoid tumor 1


Causative gene(s) retrieved from Orphanet

    Total: 0

Gene Symbol Gene Name Entrez Gene ID