Therapeutic strategy of papillary cystic and solid neoplasm (PCSN): a rare non-endocrine tumor of the pancreas in children

Abstract

Papillary-cystic and solid neoplasm (PCSN) are rare tumors. Two personal observations and a review of the literature are presented with a total of 44 pediatric patients in addition to a total of 67 published cases in the review of Cohen (Pediatr. Surg. Int. 6 (1991) 128) and Snadjauf (Eur. J. Pediatr. Surg. 9 (1999) 416). Overall, PCSN shows a clear predominance in females and only occasionally occurs in males. Typically they grow to a large tumor mass with minimal symptoms. Their histologic and immunocytologic characteristics cause diagnostic difficulties, especially on frozen sections of small biopsies. The tumors are assumed to origin from pluripotent stem cells and present as tumors of low malignancy with a favorable prognosis. Nevertheless 10 children have been reported to develop metastases, 5 have demonstrated an invasive growth pattern and 4 local recurrence. But only two of the 111 pediatric cases have died from their tumor burden. Treatment of choice is a complete surgical resection, which is true for the primary tumor and for metastases as well as local recurrences. In our 2 patients one had spleen-conserving left pancreatic resection and one mesopancreatectomy with roux-en-y-reconstruction leading to long-term cure. Adjuvant therapy in curative resected patients is unnecessary and does not appear to improve prognosis.