ORIGINAL ARTICLE Hong Zhou á Wei Cheng á K. Y. Lam Godfrey C. F. Chan á P. L. Khong Paul K. H. Tam Solid-cystic papillary tumor of the pancreas in children Accepted: 30 October 2000 Abstract Solid-cystic papillary tumor SCPT) of the pancreas is a rare neoplasm in children. The current study attempted to estimate the incidence and possible pathological origin of the tumor. Clinicopathologic features of the children under the age of 16 years with pancreatic tumors managed in a single institution be- tween January 1993 and November 1999 were reviewed. Representative blocks of SCPT specimens were immunostained for neuron-speci®c enolase NSE) chromogrannin, synaptophysin, insulin, glucagon, so- matostatin, and gastrin. There were three SCPTs, two pancreatic endocrine tumors, and one acinar cell carci- noma during the study period. The estimated yearly incidence in the referral area of 2 million population is about 0.01 pediatric SCPT per year per 100,000 popu- lation. The children underwent surgical removal of the tumors. Postoperatively, they were followed up for 6 months to 4 years and were well. Immunostaining for NSE, chromogranin, synaptophysin, insulin, somatost- atin, and gastrin was negative in all cases. SCPT may thus be the most common pancreatic tumor in the Asian pediatric population. The pathological origin of the tu- mor remains unclear and requires further investigations. Keywords Solid-cystic papillary tumor of the pancreas á Children á Asian Introduction Solid-cystic papillary tumor SCPT) of the pancreas SCPT) is a rare neoplasm in children [1±7]. It was ®rst described by Frantz in 1959 [8]. To date, over 450 cases in patients of all ages have been reported in the English literature [9]. It has been misdiagnosed as adenocarci- noma, non-functioning islet-cell tumor, cystadenoma, papillary cystadenocarcinoma, and infantile pancreatic carcinoma [1, 9, 10]. The nomenclature for this tumor includes papillary epithelial neoplasm, papillary cystic neoplasm, solid-and-papillary epithelial neoplasm, solid-and-cystic acinar-cell tumor, papillary-and-solid neoplasm, papillary-cystic epithelial neoplasm, papil- lary-cystic carcinoma, solid-and-papillary neoplasm, papillary cystic tumor, solid-and-cystic tumor, solid- and-papillary neoplasm, low-grade papillary neoplasm, and Frantz's tumor [1, 9, 11, 12]. This has caused con- siderable confusion. Misclassi®cation may be one reason why this neoplasm has only recently been recognized as a distinct entity. The term SCPT) is used here, as it best describes the pathological features of the tumor. In this report, the clinico-pathologic features of three children with SCPT are described and the features of SCPT in children reported in the literature are reviewed. Materials and methods Clinicopathologic features of three children under the age of 16 years with SCPTs resected in Queen Mary Hospital, Hong Kong, between January 1983 and November 1999 were reviewed. Repre- sentative blocks of SCPT specimens were immunostained for neuron-speci®c enolase NSE) polyclonal, pre-diluted, BioGenex, Pediatr Surg Int 2001) 17: 614±620 Ó Springer-Verlag 2001 H. Zhou á W. Cheng á P. K. H. Tam Division of Pediatric Surgery, Department of Surgery, Queen Mary Hospital, The University of Hong Kong Medical Centre, Hong Kong, China K. Y. Lam Department of Pathology, Queen Mary Hospital, The University of Hong Kong Medical Centre, Hong Kong, China G. C. F. Chan Department of Pediatrics, Queen Mary Hospital, The University of Hong Kong Medical Centre, Hong Kong, China P. L. Khong Department of Radiology, Queen Mary Hospital, The University of Hong Kong Medical Centre, Hong Kong, China P. K. H. Tam &) Division of Pediatric Surgery, Department of Surgery, The University of Hong Kong Medical Centre, Queen Mary Hospital, Pok Fu Lam Road, Hong Kong, China