Solid pseudopapillary tumor: a rare neoplasm of the pancreas

Abstract

Solid pseudopapillary tumor is a rare primary neoplasm of the pancreas that typically affects young women. It is a relatively a benign tumor, with a favorable prognosis. We here report a 27-year-old woman with solid pseudopapillary neoplasm, who presented with mild jaundice, mildly elevated liver function tests and right upper quadrant pain. Ultrasound was suggestive of hemorrhagic hepatic adenoma; however, on magnetic resonance imaging, a heterogenous mass was found in the head of pancreas. Endoscopic ultrasound-guided fine needle aspiration (FNA) revealed tumor cells with papillary architecture and immunohistochemical analysis showing cells positive for markers including beta-catenin, vimentin, alpha 1 antitrypsin etc. These findings were consistent with solid pseudopapillary neoplasm. The patient underwent pancreaticoduodenectomy. Post-surgical biopsy confirmed the FNA findings with tumor localized to the pancreas. The patient was not given any adjuvant therapy. She remained asymptomatic and showed no signs of disease after four months follow-up. It is important to differentiate this tumor from other pancreatic neoplasms, because this type is amenable to cure after complete surgical resection, even in cases with capsular invasion, unlike malignant tumors of the pancreas.

Pancreatectomía distal laparoscópica con preservación del bazo para el tratamiento del tumor pseudopapilar del páncreas: informe de un caso y revisión de la literatura

“…Su origen no está claro: según algunos investigadores, se originan de células primordiales pluripotenciales, mientras que otros sugieren un origen extrapancreá-tico (8,9). Constituyen el 5 % de los tumores quísticos del páncreas, el 1 % de las neoplasias pancreáticas exocrinas (10) y menos del 4 % de todas las lesiones quísticas resecadas del páncreas (3,11).…”

10-Year-Old Female with Acute Abdominal Pain with Pancreatic Mass

“…Cystic pancreatic lesions in general are less rare; an estimated 7% of patients who undergo imaging with MRI or CT will have a pancreatic cyst; however, less than 5% of these will have SPNs [ 1 ]. SPNs are rare tumors which are typically seen in females in their 20s and 30s [ 2 ]. The male to female ratio is 1 : 10 and the average age of presentation is 22 years old [ 2 ].…”

“…SPNs are rare tumors which are typically seen in females in their 20s and 30s [ 2 ]. The male to female ratio is 1 : 10 and the average age of presentation is 22 years old [ 2 ]. SPNs are even more uncommon in pediatrics, with an incidence rate of 0.005–0.01 cases per 100,000.…”

“…Weight loss can also be observed as well as gastrointestinal obstruction, anemia, jaundice, and, less commonly, pancreatitis, which was also present in our patient who had elevation of her lipase [ 4 ]. The most typical locations for SPNs are the body and the tail of the pancreas [ 2 ]. Ultrasound, CT, and MRI may demonstrate a well-demarcated mass that can range from cystic to primarily solid.…”

Diagnostic imaging of a solid pseudopapillary tumour of the pancreas in a 20-year-old woman – a case study

“…Based on the immunohistological profile, many researchers favour the theory that SPT originates from multipotent stem cells. Other authors, however, suggest that female sex hormones may play a role in tumorigenesis, but a definitive causal relationship has not been proven [ 3 , 4 ].…”