A 61-year-old French Canadian female presented to hospital with a two-week history of obstructive jaundice, pruritus, tea-coloured urine, acholic stools, fatigue, anorexia, and unintentional weight loss. Her history also included vague abdominal pain that had been ongoing for years prior to her visit, although the pattern of pain did not change with the onset of jaundice. Her past medical history included type 2 diabetes mellitus and hypertension. She did not have a personal history of cancer, but her family history included two aunts who had pancreatic cancer in their sixth decades of life. She denied any tobacco, alcohol, or recreational drug use.\nAbdominal ultrasound revealed a lesion in the uncinate process of the pancreas. A subsequent CT scan of the abdomen confirmed a fairly well circumscribed hypovascular mass in the head and uncinate process of the pancreas, radiographically consistent with pancreatic adenocarcinoma (Fig. ). The mass encircled the first jejunal branch of the superior mesenteric artery by approximately 180°. Endoscopic retrograde cholangiopancreatography (ERCP) was ultimately unsuccessful due to inability to cannulate the common bile duct. The pancreatic duct was patent and could be cannulated but seemed to end abruptly.\nThe patient underwent an endoscopic ultrasound, which revealed a 4.8 × 3.5 cm hypoechoic mass in the pancreatic head/uncinate process along with numerous cystic changes in the body, tail, and distal tail of the pancreas, measuring 2.4 mm, 3.9 mm, and 4.3 mm, respectively. Fine-needle aspiration (FNA) biopsy at the time of endoscopic ultrasound demonstrated cytology consistent with adenocarcinoma (Fig. ).\nAt the time of diagnosis, the patient’s functionality was consistent with Eastern Cooperative Oncology Group (ECOG) grade 1: restricted in physically strenuous activity, but ambulatory and able to carry out light work. The CA19-9 levels were ordered but deferred by the patient. Her total bilirubin at the time of diagnosis was 160 μmol/L. The patient underwent placement of a percutaneous transhepatic biliary drainage catheter, with resolution of her jaundice. The patient was reviewed at a weekly hepatobiliary and pancreatic cancer multidisciplinary conference. Consensus was that her tumor was borderline resectable on the basis of partial encasement of the superior mesenteric artery. Although there was encasement of the first jejunal branch, the tumor was staged based on abutment of less than ≤ 180° of the SMA. Radiologically, the patient was staged as T3 N0 M0. It was recommended that the patient begin systemic chemotherapy with four cycles of FOLFIRINOX (Irinotecan 180 mg/m2; Oxaliplatin 85 mg/m2; 5-Fluorouracil 400 mg/m2; Folinic acid 400 mg/m2), after which she would be re-imaged for assessment of treatment efficacy.\nThe patient tolerated the four cycles without undue toxicity, after which she underwent re-staging with CT scans of the chest, abdomen, and pelvis. This revealed multiple non-calcified small pulmonary nodules detected bilaterally in the lower lobes, the largest of which measured 4 mm. These nodules were previously not present and were thus considered suspicious for metastatic disease. As a result of the equivocal pulmonary nodules, resection was not indicated and the decision was made to continue FOLFIRINOX to further assess both local response, and the response of the potential metastatic nodules.\nThe patient received a further nine cycles of FOLFIRINOX. Follow up MRI revealed that the pancreatic lesion had impressively decreased in size from 3.9 × 3.2 cm to 2.7 × 2.3 cm compared to the previous CT (Fig. ). Furthermore, the lung lesions had remained stable since the last CT scan, raising the possibility that they were not metastatic. Given these results, and after discussion at multidisciplinary cancer conference, a resection was offered to the patient as a potentially curative treatment. She continued two more cycles of therapy before undergoing a pancreaticoduodenectomy, six weeks following discontinuation of FOLFIRINOX and 33 weeks from the date of diagnosis. No major technical difficulties were encountered in the procedure, although there was considerable tissue edema as expected from the chemotherapy. No vascular resection or reconstruction was required. Her postoperative course was complicated by pneumonia. She was discharged home on day 11. She was readmitted 9 days later with acute kidney injury, secondary to vomiting, poor intake and diarrhea, requiring one round of hemodialysis (peak creatinine 847 μmol/L or 9.58 mg/dL, Dindo-Clavien grade 4a).\nHistological examination of the surgical specimen revealed no evidence of residual adenocarcinoma (Fig. ), consistent with a complete response to treatment (College of American Pathologists grade 0) []. As is required in this situation, the entire specimen was submitted for histological assessment, confirming the complete pathological response (ypT0). Severe acute and chronic pancreatitis and areas of fibrosis were noted, together with scattered foci of grade 1 pancreatic intraepithelial neoplasia. All 23 resected lymph nodes were negative for malignancy (ypN0).\nFollowing surgery, adjuvant therapy was not given. Her CT scans at 3 and 6 months postoperatively show no evidence of recurrence, with no appreciable change in her previously noted lung nodules. Her CA 19–9 is normal at 20 kU/L. She remains disease-free 15 months after surgery, and 24 months after diagnosis. Given her strong family history of pancreatic cancer, she was referred for genetic testing. She was found to be a carrier of a BRCA2 mutation that is not uncommon among French Canadians (BRCA2: E3002K).
A 63-year-old woman was referred to emergency department with sudden epigastria pain reporting that pain had started 1 month ago. Abdominal ultrasound (US) revealed a mass in pancreas. On CT scans, a lesion of 30 mm in the pancreatic neck was found. PET-CT showed an increased uptake in the head of the pancreas (SUV: 7.45) (). Eco-endoscopy identified a solid mass uT3N1, and the biopsy results were compatible with adenocarcinoma. From the family history, mother of the patient had endometrial cancer, sister suffered from breast cancer, and aunt also had breast cancer, all of them were BRCA-positive. The patient underwent the BRCA test, and the BRCA2 mutation was identified. Neoadjuvant chemotherapy with FOLFIRINOX was started with excellent tolerance after 6 cycles and subsequent radiotherapy was applied. PET-MRI visualization revealed the absence of pathological uptake and ruled out any metastatic lesions (). A standard distal pancreatectomy with splenectomy was performed. The pathology results showed complete response to therapy with no signs of malignancy. Currently the patient () is asymptomatic with no evidence of recurrence at 9-month follow-up.