SPT of the pancreas is extremely rare in children. It is usually found incidentally on radiologic examination for other reasons and there are no typical clinical signs and symptoms. In some patients the tumor is noticed because of chronic or acute abdominal pain. SPT is usually localized in the pancreatic head. Exocrine or endocrine insufficiencies have not yet been described. No specific tumor markers are known [
4‐
9]. In one of our cases SPT was discovered incidentally and in the other because of acute abdominal pain. The localization of the tumors was head or trunk of the pancreas. In both cases there were no characteristic biochemical abnormalities, which is in accordance with reports from other authors. Awareness of the radiologic features of Frantz’s tumor is very important to establish an accurate diagnosis before operation and planned further surgery. The typical tools used to diagnose are abdominal USG and CT scan. SPT appears as a solid well-demarcated mass, usually heterogenous in echo texture, sometimes containing hypoechoic fluid-filled cystic areas in USG. CT scans show a heterogenous mass, often with peripheral contrast enhancement corresponding to the fibrous pseudocapsule [
4‐
10]. In one of our cases the tumor was solid and in the other it was cystic and solid. Histological evaluation of SPT shows typically solid and pseudopapillary structures, intensive vascularization, or hemorrhagic pseudocystic structures in various proportions [
11]. This picture was also seen in the pathohistological examination in our patients. Some authors advocate preoperative fine-needle biopsy (FNB) for distinguishing between benign and malignant lesions. It can guide surgical management, because a potentially benign tumor (for example, neuroendocrine, SPT) may be treated by local excision, in contrast to an adenocarcinoma, which would require a more extensive resection [
12]. Other authors may not accept this because of the uncertainty in diagnosis and possible tumor cell spread. FNB, for instance, may not differentiate between pancreatoblastoma, the typical pancreatic tumor of young age, and Frantz’s tumor [
9]. We performed a preoperative biopsy in Case 1 because of a suspected additional mass in her right kidney, which was not typical for SPT and could indicate non-Hodgkin lymphoma (NHL). In Case 2 the characteristic radiological appearance of Frantz’s tumor was sufficient for qualification for laparotomy and a preceding biopsy was not necessary. The low grade of malignancy of this tumor, and because the mass is usually surrounded by a dense fibrous capsule, led to complete resection with preservation of as much pancreatic tissue as possible. Local resection is therefore the therapy of choice. Distal pancreatectomy with or without splenic preservation can be performed for tumors in the trunk or tail of the pancreas, and pancreatoduodenectomy for tumors of the pancreatic head. Our first patient underwent a pancreatoduodenectomy (Traverso-Longmire) because of the tumor localization in the head and the trunk of her pancreas. In our second case, we could perform a left pancreatectomy because the tumor arose from the trunk and the tail of her pancreas. Control MRI showed tissue similar to remnants of the pancreatic tail which might explain normal levels of glucose. In this patient postoperative complication made the preservation of the pancreatic head impossible. Descriptions from case reports concerning incomplete resection of Frantz’s tumor between 1985 and 2008 show that despite the low malignant potential of SPT, median survival in patients who underwent incomplete resection was only 5.7 years. That is why complete resection of Frantz’s tumor is always justified, even at the price of difficult mutilating surgery [
13]. This is the reason we should be searching for less radical options with complete resection of the tumor as described in literature central pancreatectomy of a 5 cm tumor located in the body of pancreas [
14]. Up to now there has been no clear role for chemotherapy or radiotherapy in cases of malignancy, inoperable tumors or relapse. Reported cases and reviews from the literature where unresectable SPT was treated with chemotherapy and radiotherapy show benefits in a limited number of patients, such as the report of a case of a 14-year-old girl with unresectable Frantz’s tumor with a 7 × 7 cm diameter mass compressing her superior mesenteric vein with acute pancreatitis. After two cycles of gemcitabine a repeated CT scan showed significant interval decrease in the size of the pancreatic mass 1.5 × 1.0 cm without compression on her superior mesenteric vein and no evidence of pancreatitis. She was able to successfully undergo a pyloric-sparing Whipple procedure [
15,
16]. Our two patients were not considered for preoperative chemotherapy or radiotherapy. In both cases the tumor was qualified as radically resectable. At present both patients remain without tumor recurrence.