Management of unresectable solid papillary cystic tumor of the pancreas. A case report and literature review Pietro Soloni a , Giovanni Cecchetto b , Patrizia Dall'Igna b , Modesto Carli a , Tiziana Toffolutti c , Gianni Bisogno a, ⁎ a Division of Hematology/Oncology, Department of Pediatrics, University Hospital of Padova, 35128 Padova, Italy b Division of Pediatric Surgery, Department of Pediatrics, University Hospital of Padova, 35128 Padova, Italy c Section of Pediatric Radiology, Institute of Radiology, University Hospital of Padova, 35128 Padova, Italy Received 14 October 2009; revised 9 February 2010; accepted 11 February 2010 Key words: Solid pseudopapillary tumor; Papillary cystic neoplasm; Pancreas; Cystic and solid tumor; Frantz tumor Abstract Pancreatic solid papillary cystic tumor is a rare neoplasm with an excellent prognosis if surgical excision is complete. We report on a case and review 47 more cases extracted from the published literature to assess the treatment options when solid papillary cystic tumor is considered unresectable. Chemotherapy and radiotherapy were beneficial in a limited number of patients, but therapeutic decisions must be made bearing in mind that patients may be long-term survivors without any treatment because of the tumor's slow growth. © 2010 Elsevier Inc. All rights reserved. Solid papillary cystic tumor (SPT) of the pancreas is an uncommon epithelial tumor accounting for 2% to 3% of all pancreatic tumors occurring at any age. It typically affects young women in their second or third decade of life, whereas it is exceptional in children [1,2]. It is characterized by a solid cystic appearance, often surrounded by a fibrous pseudocapsule. Microscopically, it consists of a combination of solid, pseudopapillary, and hemorrhagic pseudocystic structures; on immunohistochem- ical investigation, it expresses epithelial, mesenchymal, and endocrine markers [3,4]. Solid papillary cystic tumor appears to be an indolent neoplasm with a low, but often unpredictable, malignant potential. Metastases, especially in the liver, have been described in up to 20% of patients [4] and can be seen at diagnosis or appear after long disease-free periods [1]. The prognosis is excellent when surgical excision is complete, although the treatment for patients with inoperable or relapsing tumors is less clear. To help clarify the most suitable management for such patients, we reviewed the literature and discuss a case that we have recently treated. 1. Case report An 11-year-old girl with a previously unremarkable medical history underwent abdominal US after a mild abdominal trauma. A pancreatic lesion was discovered, and a computed tomographic (CT) scan confirmed the presence of a large mass arising from the pancreatic body. No evidence of distant metastases or increase in tumor markers (α-fetoprotein, β-subunit of human chorionic gonadotropin, ⁎ Corresponding author. Tel.: +39 049 8211481; fax: +39 049 8213510. E-mail address: gianni.bisogno@unipd.it (G. Bisogno). www.elsevier.com/locate/jpedsurg 0022-3468/$ – see front matter © 2010 Elsevier Inc. All rights reserved. doi:10.1016/j.jpedsurg.2010.02.045 Journal of Pediatric Surgery (2010) 45, E1–E6