Solid pseudopapillary neoplasm of pancreas: Two case reports

Medicine (Baltimore). 2019 Jul;98(29):e16455. doi: 10.1097/MD.0000000000016455.


Rationale: About 8384 cases of solid pseudopapillary neoplasms (SPN) of pancreas have been published in English literature, from 1933 to 2018. This is a low-grade tumor that usually occurs in children but is rare in adults and, in exceptional cases, can show extrapancreatic localization. In this paper we present 2 unusual cases of SPNs, 1 with retroperitoneal location (case 1) and 1 that was firstly diagnosed as a G1 neuroendocrine tumor (NET) and showed hepatic metastases after 13 years (case 2).

Patient concerns: No symptoms in first case. The tumor was incidentally diagnosed, during ultrasound examination. In the second case, the metastasis was observed during regular follow-up.

Diagnoses: The diagnosis was established based on the histological features and immunohistochemical profile that showed positivity for vimentin, nuclear β-catenin, cyclin D1, CD10, and SRY-related high-mobility group box 11 and negativity for maspin.

Interventions: Surgical excision, in both cases.

Outcomes: No recurrences in first case, at 5 months after diagnosis. Hepatic metastases in the second case, at 13 years after diagnosis, with portal invasion after another 15 months.

Lessons: Without a complex immunoprofile, SPN can be misdiagnosed as NET. SPN can be a low-grade tumor but long-time follow-up is mandatory to detect delayed metastases. A correct diagnosis is necessary for a proper therapeutic management.

Publication types

  • Case Reports

MeSH terms

  • Adenocarcinoma, Papillary* / immunology
  • Adenocarcinoma, Papillary* / pathology
  • Adenocarcinoma, Papillary* / physiopathology
  • Adenocarcinoma, Papillary* / therapy
  • Adult
  • Biomarkers, Tumor / analysis*
  • Cyclin D1 / analysis
  • Diagnosis, Differential
  • Female
  • Humans
  • Immunohistochemistry
  • Middle Aged
  • Neoplasms, Cystic, Mucinous, and Serous* / immunology
  • Neoplasms, Cystic, Mucinous, and Serous* / pathology
  • Neoplasms, Cystic, Mucinous, and Serous* / physiopathology
  • Neoplasms, Cystic, Mucinous, and Serous* / therapy
  • Neprilysin / analysis
  • Neuroendocrine Tumors / diagnosis*
  • Pancreas / pathology*
  • Pancreatectomy / adverse effects
  • Pancreatectomy / methods
  • Pancreatic Neoplasms* / immunology
  • Pancreatic Neoplasms* / pathology
  • Pancreatic Neoplasms* / physiopathology
  • Pancreatic Neoplasms* / therapy
  • Prognosis
  • Treatment Outcome
  • Vimentin / analysis
  • beta Catenin / analysis


  • Biomarkers, Tumor
  • CTNNB1 protein, human
  • Vimentin
  • beta Catenin
  • Cyclin D1
  • Neprilysin