Diagnostic Dilemma For Periampullary Tumour Resolved & Treated

A 65-year-old man presented with high coloured urine for the last three months to Max Super Speciality Hospital, Mohali. There were no known comorbidities. On evaluation, he was found to have features of obstructive jaundice (Total Bilirubin-16 mg/dl) and elevated CA 19.9 (1068 U/ml). He underwent MRCP, which showed cholelithiasis, and dilated CBD with stricture at the lower-end. He was subjected to ERCP and stenting. EUS and side view endoscopy were also done, suggesting EHBO - distal CBD stricture and carcinoma gall bladder. Biopsy from the periampullary region did not show any malignancy. Bilirubin and CA 19.9 started coming down, but the diagnosis was still a challenge. Following this, the patient underwent a PET CECT scan which showed FDG avid GB fundus wall (SUV max 3.9). There was a 3.9 x 3.8 cm metabolically active (SUV max 6.7) cystic/necrotic lesion in relation to segment 6 of the liver. Distal CBD thickening with mild FDG uptake (SUV max 2.5) was seen in the periampullary region, causing an abrupt cut-off. There were few periportal, portocaval and peripancreatic LNs, suspicious of metastatic disease. This really confused the matter. A multiphasic CECT was done to aid the diagnostic process. It further complicated the scenario by reporting two arterial enhancing tiny sub-centimetric lesions in segments 2 and 6 of the liver metastasis from the carcinoma gall bladder. GB showed mildly thickened shaggy walls, 3-3.5 mm in fundus/mid-body and 6-7 mm in the neck region. EUS and CECT scans were done at the Mohali centre, while MRCP and PET were done at two different centres. Therefore, it was really difficult to club all under one roof. We asked the patient to get the CDs of all the outside imaging done. The case was discussed in a multidisciplinary team approach, and the doctors decided to do a repeat biopsy from the periampullary region. The arrow hit the target this time and revealed well-differentiated adenocarcinoma. The patient’s bilirubin and CA 19.9 got normalised by this time and he was planned for Whipple’s surgery.

Well-differentiated periampullary adenocarcinoma was diagnosed. Pancreatoduodenectomy (Whipple’s surgery) was done. As the patient had a stent in the CBD and empyema of the gall bladder, considering the high incidence of surgical site infection, the wound was intermittently tagged with Ethilon sutures, followed by twice daily dressing in the post-operative period. There was a biochemical pancreatic fistula on post-operative day 3, which resolved by day 5. The patient was started on FJ feed first, followed by oral liquid and semisolids thereafter. There was not much of the component of delayed gastric emptying, although he had nausea sometimes and vomited once on post-operative day 5. Secondary closure of the wound was done on post-operative day 6, a day prior to discharge.

The patient was regularly followed up in OPD. Histopathology reported (as shown in figures), moderately differentiated adenocarcinoma of the periampullary region infiltrating muscularis propria of the duodenum. All margins were free of tumours. One peripancreatic lymph node was positive with extranodal extension turning the patient a candidate for adjuvant chemotherapy. Gall bladder showed xanthogranulomatous cholecystitis, realising the fact of inclination towards carcinoma gall bladder in the pre-operative period. The patient was doing well till the last follow-up and is being planned for adjuvant chemotherapy.

Dealing with diagnostic uncertainty is a major challenge for appropriate management. This case is a very clear example of the importance of a multidisciplinary team in managing such complicated cases. Periampullary carcinoma is a widely used term to define a heterogeneous group of neoplasms arising from the head of the pancreas, the distal common bile duct and the duodenum. This term should be distinguished from ampullary carcinoma. Duodenal cancer has the longest survival, at five years when compared with other periampullary tumours. The prognosis of pancreatic adenocarcinoma is one of the most dismal of all cancers.

In conclusion, in ampullary and periampullary tumours, resection margin status, resected lymph node statu and degree of tumour differentiation. significantly influence the outcome, and of course, the role of the multidisciplinary team in setting a diagnosis is never forgettable.