A 35 year-old man presented with gradual swelling of abdomen for last 10 years. This was associated with mild pain, anorexia and moderate weight loss. There was no history of alcoholism, jaundice, vomiting, obstipation or gastro intestinal bleeding.
Clinical examination revealed a bosselated, abdominal swelling with soft to firm consistency occupying the whole abdomen and almost reaching up to the pelvis. His complete blood count and liver function tests were normal. An ultrasound and CECT done showed a complex hepatic mass having cystic, solid and fatty areas and a similar complex mass inside the abdominal cavity (,). A fine needle aspiration cytology done in another hospital was inconclusive.
Figure 1 The contrast enhanced CT scan demonstrates a well circumscribed lobulated, multicystic lesion involving the head of pancreas. The cysts have thick walls with fine wall calcifications and mildly different levels of densities within the cyst cavities. Diffuse (more ...)
Figure 2 Contrast enhanced CT scan demonstrates a large, well-defined, heterogenously hypodense lesion in the right lobe of liver comprising of multiloculated cysts with fine calcifications in the wall, mildly enhancing soft tissue component and small amount of (more ...)
At laparotomy the peritoneal cavity was found full of a partly cystic and partly fatty mass, arising from the head of the pancreas (). There was abundant fat around the portal triad and beneath the gallbladder. There was no ascites or peritoneal nodules. During tumour mobililization splenic vein was transected for tumor clearance. Classical Whipple’s pancreatoduodenectomy, splenectomy alongwith the tumour excision was done.
Operative photograph showing the pancreatic tumour. Black arrow marks the fatty component of the tumour
Postoperatively patient had a pancreatic leak which was managed conservatively. After 3 months, he underwent right hepatectomy for the liver tumour. At this time rest of the visceras appeared normal and no areas of abnormal adoipose tissue proliferation was seen.
Two independent experienced pathologists examined the specimens and the microscopy.
The pancreatic specimen (approximately 37 cm × 25 cm × 8 cm in size) was smooth, glistening, and grayish in color with attached clumps of fat. On cutting open it was multiloculated and cystic, largest locule was of 18 cm diameter. Inner surface was also smooth, whitish and without any papillary projections (). Cysts contained mucoid, brown, inpissated fluid. Microscopic sections revealed mucinous neoplasm with a single layer of mucin secreting columnar epithelium. There was no cellular stratification, pleomorphism or mitotic activity (). Sections from fat showed mature adipose tissue.
Inner smooth surface of the cyst of the pancreas
Hematoxylin and eosin (H&E) stained photomicrograph from pancreatic cyst showing mucin secreting columnar epithelium without significant cellular atypia (× 400)
The liver specimen was of 15 cm × 9 cm × 6 cm in size, containing both solid and cystic areas of variable sizes with areas of adipose tissue (). Largest cyst was of 3 cm diameter. Cysts contained yellowish mucoid materal. Microscopic sections showed cystic tumor with a lining of columnar mucin secreting cells having minimal cytologic atypia. Intervening fibrocollagenous stroma showed biliary ducts, scant lymphocytic and plasma cell infiltrate ().
Cut surface of the liver tumour showing cystic, solid and fatty component
Hematoxylin and eosin (H&E) stained section from hepatic cyst showing lining mucin secreting columnar epithelium and underlying fibrocollagenous tissue containing biliary ducts (× 100)
The final histological diagnosis was of mucinous cystic neoplasms of the pancreas and the liver without ovarian-like stroma. The patient is doing well 24 months postoperatively. Followup CECT scan done after 24 months showed no recurrence of the tumours ().
Figure 8 Contrast enhanced CT scan 2 years after right hepatectomy shows hypertrophy of the left lobe of liver and a small area of post-operative fibrosis with no evidence of recurrence of the tumor. Axial section at the level of pancreas also demonstrates no (more ...)