Solid pseudopapillary tumor of the pancreas: Experience at a tertiary care centre of Northern India

Abstract

Introduction: Solid pseudopapillary tumor (SPT) of the pancreas is rare, accounting for 0.13-2.7% of all pancreatic tumors. It is unique, has low malignant potential and predominantly affects young women. Radiological and pathological studies have revealed that the tumor is quite different from other pancreatic tumors. But the cell origin of SPT and tumorigenesis are still enigmatic. Abdominal mass is the most common presenting symptom. Due to the paucity of the number of cases, the natural history of the disease is not fully understood. This study was undertaken to examine the clinico-pathological characteristics of the disease and to evaluate the outcome of surgical intervention in a tertiary referral care centre.

Materials and methods: A retrospective analysis of all patients diagnosed and treated for SPN in our hospital over a period of 10 years (2005-2015) was carried out. A database of the characteristics of these patients was developed. In all, 11 patients were identified. A CT scan of the abdomen was performed in all the patients and the findings revealed a mass in the pancreas. The investigations performed included routine blood investigations, chest X-ray, CA-19-9 level and either an ultrasound or a CT Scan of the abdomen.

Results: During the time period of 10 years, of 349 patients with pancreatic malignancy admitted to our department, only 11 were diagnosed as having SPN (3.15%). Ten patients were women (90%) and one patient was a man (10%). The patients had a median age of 27.6 years (range 17-41). The most common symptoms were abdominal pain and dullness. Eight patients (72.7%) presented with abdominal pain or abdominal dullness and three patient (27%) were asymptomatic. All the 11 patients were taken up for surgery. Three patients underwent distal pancreatectomy with splenectomy, three patients underwent the total mass excision and one patient underwent total pancreatic resection. Three required extended distal pancreatectomy with splenectomy. One underwent spleen-preserving distal pancreatectomy.

Conclusion: SPT is rare, but treatable pancreatic tumor. While clinical signs and symptoms are relatively nonspecific, characteristic findings on imaging and histology separate these tumors from the more malignant pancreatic tumors. The prognosis is favorable even in the presence of distant metastasis. Although surgical resection is generally curative, a close follow-up is advised in order to diagnose a local recurrence or distant metastasis.

Keywords: Case series; Female; Pancreas; Solid pseudopapillary tumor (SPT); Young.

Fig. 1

(A&B) CECT abdomen revealed a 9.4 × 9.2 × 10.2 cm lesion arising from body and tail of pancreas.

Fig. 2

(A, B & C) ON GROSS: an encapsulated, well demarcated tumor in body and tail of pancreas.

Fig. 3

(A&B) On Microscopy: a well circumscribed tumor mass separated from pancreas by a fibrous capsule. The tumor cells were arranged in sheets, nests, pseudopillary formations. (C) The tumor cells were positive for PR (progesterone receptor). (D) CD10 & CD20.