The work has been reported in line with the CARE criteria.
A 75-year-old female patient with a medical history of hypertension was referred to our unit with the diagnosis of a giant cell tumor at the head of pancreas. Three years ago she presented to her local hospital with acute idiopathic pancreatitis. Receiving conservative treatment, she was asymptomatic for the subsequent 12 months, before she started suffering from abdominal discomfort, steatorrhea and weight loss. Computed tomography (CT) abdominal scan and magnetic resonance imaging (MRI) showed a cystic mass (max. diameter 4 cm) in the head of pancreas, which was thought to be a pancreatic pseudo-cyst, but due to increasing size and ensuing of symptoms, a CT-guided biopsy was performed. Histopathology showed the presence of multinuclear OGCs and she was referred to our center.
Our CT scan showed a 9 cm large head of pancreas mass, with solid and cystic components, in close relation (but no invasion) to the mesenteric vessels and the common hepatic artery (Fig. 1). The main pancreatic (but not the common bile duct) was dilated; the body and tail of pancreas were atrophic with no evidence of acute inflammation or lymphadenopathy. Chest CT scan was normal. Preoperative blood tests (TBL, ALP, gamma-GT, CEA) were normal, except for CA 19-9 at 110 U/ml (normal values: 0-37).
At theater a large encapsulated mass was discovered, in direct contact with the common hepatic artery and with the superior mesenteric vein. During dissection of the tumor from the common hepatic artery, it was realized that the common hepatic artery was invaded by the tumor. Although it was not clear if a resection would be beneficial for the patient it was decided the resection to go ahead as the common hepatic artery was partially injured during the dissection and a reconstruction was needed. For this reason a Whipple procedure was performed with excision of the involved part of the common hepatic artery and blood flow was restored with a 3 cm long venous graft from the left major saphenous vein. Postoperative course was uneventful. The patient denied having chemotherapy. Follow-up at 3 and 6 months found her in good health, but unfortunately she developed local recurrence and died 10 months after surgery.
Pathology revealed a round, circumscribed neoplastic mass measuring 8.5 cm in maximum dimension, extending to the ampulla of Vater. Microscopic examination showed a malignant neoplasm composed of undifferentiated cells with great pleomorphism and nuclear atypia, as well as of scattered OGCs (Fig. 2). Moreover, a minor well-differentiated adenocarcinoma component was also seen. Immunohistochemically, the great majority of undifferentiated neoplastic cells expressed vimentin and at least some of them were stained for cytokeratin and p53. OGCs reacted strongly for CD68, KP1 and PGM1, and were negative for all other markers. None of the seven peripancreatic lymph nodes found showed evidence of metastatic disease.