FOLFOX regimen in pancreatic acinar cell carcinoma: Case report and review of the literature

Abstract

Pancreatic acinar cell carcinoma (PACC) is a rare tumor with a poor prognosis in the absence of surgery. Moreover, there is currently no standard chemotherapy for metastatic PACC.We report the case of a patient treated by the FOlFOX 4 regimen with an objective response and a prolonged tumor control and we provide a review of chemotherapy regimens reported for PACC in the literature. Case reportA 79-year-old man, whose main medical history was a prostatic cancer (2004) treated by hormone therapy and radiotherapy with complete remission, was admitted in March 2009 following three months of painful erythematous subcutaneous nodules mainly located on both lower limbs. Performance Status was good (ECOg 1) with, however, a 4 kg weight loss within the past three months. The patient described also for several months slight transitory pain in the left side of the abdomen. The skin lesions evolved typically from erythematous and painful infiltrated plaques, secondly ulcerated with emission of subcutaneous fat, resolving into hyperpigmented scars. Skin biopsy showed focal subcutaneous fat necrosis, pathognomonic of pancreatic panniculitis, suggesting an underlying pancreatic disease. The serum lipase level was markedly elevated (5000 Ui/l). As for tumor markers, levels of CEA and CA 19-9 were low. A computed tomography (CT)-scan showed an infiltrating mass in the tail of the pancreas with spleen invasion and four suspect lesions in the liver (Figure 1). The biopsy of liver metastasis revealed a secondary localization of a pancreatic acinar cell carcinoma.A weekly chemotherapy with gemcitabine (1000 mg/m² weekly) was introduced. in addition, considering the disabling skin lesions, a systemic corticotherapy was initiated in the same time (prednisone 1 mg/kg/day for three days and then 30 mg per day).After two months of treatment, the patient presented a marked weakness with a 7 kg weight loss and a worsening of the skin eruption, without any improvement after corticosteroids were increased. On imaging, liver metastases had increased in size and number (unequivocal progression  50% by rECiST), although the pancreatic mass was stable in size (Figure 1).Then second-line chemotherapy was begun with the FOlFOX4 regimen. A clinical benefit was quickly observed as after only two courses skin lesions gradually improved and after four courses all lesions were almost healed, and the patient had recovered 5 kg and a good Performance Status. The clinical impression was reinforced by morphological assessment: the CT-scan showed a dramatic decrease of the pancreatic mass and of the liver metastases ( 50% by rECiST). After eight courses, the clinical good evolution was confirmed by an additional decrease of the pancreatic lesion and of the liver metastases (25% by rECiST), and serum lipase level was 130 Ui/l. The treatment had to be stopped after 12 courses in December 2009 because of a grade 3 neurotoxicity related to oxaliplatin. The patient was under medical surveillance for the following seven months without any clinical or morphologi...

Efficacy of Chemotherapy in Patients with Unresectable or Metastatic Pancreatic Acinar Cell Carcinoma: Potentially Improved Efficacy with Oxaliplatin-Containing Regimen

“…KRAS , TP53 , CDKN2A (p16), and SMAD4 gene mutations were not typically found in pancreatic ACC , whereas the frequency of mutations in the adenomatous polyposis coli–β catenin pathway, which is rarely detected in PDAC, was similar to those found in colorectal cancer (7%-24%) [17-19]. These findings suggest that the chemotherapeutic approaches for ACC patients include agents known to have activity in colorectal cancer [14,20-22]. …”

DNA Mismatch Repair Abnormalities in Acinar Cell Carcinoma of the Pancreas

“…This assumption has indeed been supported by isolated case reports and small series. 10,11,13,14 At the current time, however, there have been no clinical trials and there are no established patient selection criteria for the use of the various chemotherapeutic regimens. It is therefore worth directing future efforts toward molecular typing of the tumor and correlating the resulting data with the tumor's response to specific therapeutic agents.…”

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

“…Although no standard chemotherapy for advanced, unrespectable PACC has been established to date, some studies suggested a more favorable efficacy for 5-FU compare with gemcitabine (8). Considering the promising data for FOLFIRINOX against advanced pancreatic cancer (18), the present patient was treated with this regimen.…”

“…In cases where curative resection is not applicable due to advanced disease, chemotherapy may be considered. Although no standard therapy for PACC has been established to date due to the rarity of this disease, 5-fluorouracil (5-FU) is considered to be more effective compared with gemcitabine (8). …”