Dealing a Rare Case with Common Methods

Solid Pseudopapillary Tumour (SPT) of the pancreas is a very rare entity with a reported incidence of 0.13% to 2.7% of all pancreatic tumours, which was once described in many other terms, such as Frantz’s Tumour, Solid and Cystic Tumour, Papillary Cystic Neoplasm, Solid and Papillary Epithelial Neoplasm. It has a distinct female preponderance, low malignant potential and with an increasing rate of the disease being reported, there are higher chances of pre-operative mis-diagnosis. Due to the rarity and scarce literature about SPT there is uncertainty in the strategy for its treatment. Although pancreatic SPNs are uncommon, clinicians should consider the diagnosis in young women with typical lesions because such patients should have positive outcomes with appropriate surgical treatment.


A 34-year-old man was incidentally detected to have a pancreatic lesion during a routine health check-up. An abdominal ultrasound revealed a discrete hypoechoic lesion in the epigastric region - 4 cm x 3.5 cm in diameter. There were no calcifications or liquefaction within it.


The radiological appearance with sharp definitions and without local infiltration suggested towards a benign etiology. EUS-FNA revealed solid and cystic pseudopapillary neoplasm of pancreas. Tumour markers were within normal limits. A pylorus preserving pancreaticoduodenectomy was performed. Immunohistochemistry staining was negative for Ck7, neuron specific enolase and synaptophysin but positive for Cd10, CD56 and PR. A diagnosis of pancreatic SPEN was made. The resection margins were microscopically clear.


A year after resection, there is no evidence of local recurrence and the patient is doing quite well.

Radical surgical resection is established as the standard treatment protocol for the disease. It is also recommended to perform metastasectomy, vascular resections and/or resections of other compromised organs in order to ensure therapeutic success in 95% of the cases. A post-operative follow up of at least 5 years is required to identify the possibility of relapses.

Dr. Prashant B. Kerkar

Dr. Prashant B. Kerkar
Sr. Consultant
Surgical Oncology
Nanavati Super Speciality
Hospital, Mumbai