Diagnosis and surgical treatment of solid pseudopapillary neoplasm of the pancreas: analysis of 24 cases

Background: Our aim was to summarize our experience with the diagnosis and surgical treatment of solid pseudopapillary neoplasm (SPN) of the pancreas to provide a reference for the management of this rare condition. Methods: We collected and analyzed retrospective data on the clinical presentation, laboratory investigations, radiologic imaging, pathology and operative details of patients with SPN of the pancreas diagnosed between February 2001 and December 2009. Results: In all, 23 of 24 patients were women, and the mean age of all patients was 31 years. The most common clinical presentation was vague abdominal pain. Abdominal imaging showed solid or solid cystic masses in the pancreas, mostly in the tail or head of the gland. All patients were treated surgically. There were no postoperative deaths. After follow-up ranging from 4 to 109 months (median 68 mo), 20 of 22 patients who underwent curative resection were alive with no evidence of disease recurrence. Of the 2 patients with R1 resections, 1 died 42 months after surgery, whereas the other underwent a second operation and was alive after 36 months' follow-up. Conclusion: Solid pseudopapillary neoplasm of the pancreas is a relatively indolent tumour. The initial diagnosis of SPN of the pancreas is suggested by radiologic imaging findings but should be considered in the context of clinical and histopathologic characteristics. We advocate for complete surgical resection once SPN is diagnosed. Contexte : Nous voulions resumer notre experience du diagnostic et du traitement chirurgical du neoplasme pseudo-papillaire solide (NPS) du pancreas afin d'etablir un point de reference pour la prise en charge de ce probleme rare. Methodes : Nous avons reuni et analyse des donnees retrospectives sur la manifestation clinique, les examens de laboratoire, l'imagerie radiologique, la pathologie et les details operatoires de patients atteints de NPS du pancreas diagnostique entre fevrier 2001 et decembre 2009. Resultats : Au total, 23 des 24 patients etaient des femmes et l'age moyen de tous les patients s'etablissait a 31 ans. La manifestation clinique la plus courante etait une vague douleur abdominale. L'imagerie abdominale a revele la presence de masses solides ou kystiques solides au pancreas, surtout a la queue ou a la tete de la glande. Tous les patients ont ete traites par chirurgie. Il n'y a pas eu de deces apres l'intervention. Apres un suivi echelonne sur 4 a 109 mois (mediane de 68 mois), 20 des 22 patients qui ont subi une resection curative etaient vivants et ne presentaient aucun signe de reapparition de la maladie. Des 2 patients qui ont subi une resection R1, 1 est decede 42 mois apres l'intervention tandis que l'autre a subi une deuxieme intervention et etait vivant apres un suivi de 36 mois. Conclusion : Le neoplasme pseudo-papillaire solide du pancreas est une tumeur relativement indolente. Le diagnostic initial de NPS du pancreas est indique par les resultats d'imagerie radiologique, mais il faudrait l'envisager en presence de caracteristiques cliniques et histopathologiques. Nous preconisons la resection chirurgicale complete apres le diagnostic.
A solid pseudopapillary neoplasm (SPN) of the pancreas is a rare pancreatic tumour. It was first described by Dr. Frantz in 1959,1 and was defined by the World Health Organization [...]