Pancreatic acinar cell carcinoma presenting with panniculitis, successfully treated with FOLFIRINOX: A case report

Abstract

Pancreatic acinar cell carcinoma (PACC) is a rare tumor of the exocrine pancreas, representing only 1% of all pancreatic malignancies. A 50-year-old man presented with edema of the thumb joints bilaterally, followed by an appearance of masses in the bilateral lower extremities and fever (38°C). The masses were diagnosed as panniculitis by skin biopsy, and multiple intraperitoneal masses were incidentally detected on pelvic magnetic resonance imaging performed to investigate the leg abnormalities. The patient was referred to the Kyushu University Hospital for further investigation, and fluorodeoxyglucose-positron emission tomography/computed tomography (CT) revealed high-uptake tumors in the pancreatic tail, in the periphery of the liver, and in the pelvis. Laboratory examinations revealed high serum concentrations of pancreatic exocrine enzymes, such as lipase, trypsin, elastase 1 and pancreatic phospholipase A2. Histological examination of a bioptic specimen obtained from a hepatic lesion revealed proliferation of atypical cells arranged in a tubular or glandular pattern. Immunohistochemical staining revealed that the atypical cells were positive for cytokeratin (CK)7, CK19 and lipase, but negative for CK20 and thyroid transcription factor-1, leading to a final diagnosis of acinar cell carcinoma of the pancreatic tail (T4bN0M1, stage IV according to the 7th edition of the TNM Classification of Malignant Tumors). Combined chemotherapy with oxaliplatin, irinotecan and fluorouracil (FOLFIRINOX) was administered and fever was soon alleviated. The serum levels of lipase also declined and panniculitis completely resolved. As of the start of the 8th course of chemotherapy, the levels of the pancreatic exocrine enzymes were within normal ranges and CT revealed partial response. Therefore, the severe lipase hypersecretion syndrome was well controlled by the FOLFIRINOX regimen and shrinkage of the mass was also achieved. Thus, the FOLFIRINOX regimen may represent an effective treatment option for advanced PACC.

Multicenter Retrospective Analysis of Chemotherapy for Advanced Pancreatic Acinar Cell Carcinoma

“…Thus, platinum and irinotecan might be among the key treatment agents for patients with unresectable PACC, and combination regimens, such as FOLFIRINOX, 29 might be promising regimens for unresectable or recurrent PACC, because FOLFIRINOX elicited favorable responses in some case reports. 13 , 14 , 16 , 19 …”

Pancreatic Acinar Cell Carcinoma with Germline BRCA2 Mutation and Severe Pancreatic Panniculitis: A Case Report

“…In a retrospective analysis, the use of 22 different regimens in 18 cases of ACC was described, with reported disease control in patients who received irinotecan, fluorouracil and leucovorin as well as cytarabine and cisplatin [8]. Recently, a case report described FOLFIRINOX as an effective first-line treatment to control tumor growth and symptoms of lipase hypersecretion syndrome in ACC [13]. In our case report, partial response was also achieved with FOLFIRINOX and FOLFIRI, but treatment was discontinued due to chemotherapy-induced neuropathic pain.…”

Optimizing Chemotherapy of Pancreatic Acinar Cell Carcinoma: Our Experiences and Pooled Analysis of Literatures

“…Brunetti O [23] / Butturini, G [5] 1999/2011 Ukei, T [43] / Jauch S [44] 2016/2018 GEM + CAP 1PR/2SD/1PD Yoo C [15] / Lowery MA [27] Sorscher SM [45] 2011/2017 PEXG 5SD Brunetti O [23] 2011 GTX 1PR/1SD Lowery MA [27] 2011 CAPE/ Temozolomide 1PD Callata-Carhuapoma, H. R [46] 2015 FOLFIRINOX 6PR/2SD Li M [47] / Yoshihiro T [48] Kryklyva V [49] /Schempf U [50] Kruger S [30] /Pfrommer S [51] 2013 AG/GEMOX 1PR/1SD/2PD Brunetti O [23] / Lowery MA [27] Kruger S [30] 2011/2016 2018 GEM 2SD/4PD Brunetti O [23] / Yoo C [15] / Kanemasa Y [34] 2013/2017/2018…”