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Introduction: Franz Tumor or the solid pseudopapillary neoplasm is a very rare form of pancreatic cancer, that can be held responsible for approximately 0.2-2% of the exocrine pancreatic tumors.Case report: We report a case of a 20-year-old woman, without any comorbidities, admitted to our department accusing mild abdominal pain at the level of the left hypochondrium and epigastrium, that started approximately half a year ago. Computed tomography and magnetic resonance imaging showed a voluminous encapsulated tumor, with mixed structure, apparently adherent to the tail of the pancreas, left kidney and adrenal gland, though being unable to certainly establish its visceral origin.Management and results: After analyzing all aspects of the case, we decide in favor of a left subcostal laparotomy approach; the intraoperative aspect is that of a relatively well delineated mass, adherent at the level of the pancreatic tail, therefore a complete excision of the tumor alongside with the pancreatic tissue that came in contact with it was performed. The pathological analysis reveals an encapsulated tumor with solid and pseudopapillary structure, with hemorrhagic and cystic degeneration regions; therefore, we reach the following final diagnosis: pT3 stage pseudopapillary-solid pancreatic neoplasm.Conclusions: In spite of its malignant behavior and impressive tumor volume, the surgical intervention was curative with favorable prognosis.

Solid pseudopapillary neoplasm (SPN) is a rare clinical entity. One third of the patients are asymptomatic and the diagnostic is based on imaging studies (abdominal ultrasound, computed tomography, magnetic resonance imaging) and histological examination of biopsies obtained most frequently by endoscopic ultrasound-guided fine-needle aspiration. In this article we made a short review of the literature and present the imaging and histological aspects found in our patients with SNP.

Solid pseudopapillary neoplasm (SPN) of the pancreas is a rare pancreatic tumor with low malignant potential. It occurs characteristically more often in young women. Radiological and pathological studies have revealed that the tumor is quite different from other pancreatic tumors. Limited information is available in the literature reporting their accumulation of fluorine-18 fluorodeoxyglucose (18F-FDG) in positron emission tomography/computed tomography (PET/CT). Here, we report a case of pancreatic SPN imaged with contrast-enhanced FDG PET/CT. A percutaneous fine needle aspiration from the metabolically active lesion revealed SPN, and it was confirmed with histopathological results. Recurrence or metastasis was not found after 7 months of follow-up.

Solid pseudopapillary neoplasm (SPN) of the pancreas is a rare, low-grade malignant epithelial tumor that may originate from embryonic multipotent stem cells. The prevalence of metastatic SPN is approximately 9% to 15%, most commonly in the liver and peritoneum. Delayed ovarian metastasis during pregnancy is exceedingly rare, pregnancy may promote the progression and pose a life-threatening risk. A 38-year-old female with an early intrauterine pregnancy was found to have a large mass in the right ovary. Following pregnancy termination and surgical resection, initial pathological assessment indicated a sex cord-stromal tumor, specifically a luteinized Sertoli-Leydig cell tumor. Upon reviewing her medical history, it was noted that a pancreatic tumor resection performed ten years prior. Further immunohistochemical analysis demonstrated diffuse nuclear and cytoplasmic expression of β-catenin, along with positivity for LEF-1, CD10, and TFE-3. Based on these findings, the final diagnosis was revised to pancreatic SPN with delayed ovarian metastasis. The patient showed no evidence of disease recurrence during the 2-year follow-up after treatment. This is a rare case of ovarian metastatic pancreatic SPN that occurred during pregnancy ten years after the initial diagnosis. Our findings underscore the diagnostic challenge in distinguishing metastatic lesions from primary ovarian tumors and highlight the clinical importance of excluding high-grade transformation during pathological assessment.

A 23-year-old woman presented to our hospital with nonspecific pain in the abdomen. She underwent radiological investigations, which revealed a solid and cystic mass in the tail end of the pancreas. The mass was diagnosed to be solid pseudopapillary neoplasm of the pancreas on intraoperative scrape cytology. This was further confirmed by histopathology. The cytological diagnosis enabled appropriate surgical treatment to be planned and carried out without undue delay. It is important to distinguish this rare tumor from other pancreatic tumors with similar cytohistologic features because, if diagnosed correctly and managed surgically, this neoplasm is associated with a good prognosis.

Solid pseudopapillary neoplasm (SPN) of the pancreas is an uncommon tumor, leading to the lack of a prognostic prediction model. This study aimed to develop and validate a nomogram for predicting overall survival (OS) in SPN patients. Data from patients diagnosed with SPN between 2000 and 2018 were obtained from the Surveillance, Epidemiology, and End Results (SEER) database and randomly split into training and validation cohorts (7:3). Independent prognostic factors for OS were identified by Cox regression analysis. A nomogram was constructed and internally validated using the concordance index (C-index), area under the curve (AUC), calibration curves, and decision curve analysis (DCA). External validation was performed using our institutional data from SPN patients between January 2008 and January 2026. The results showed that the total positive lymph nodes, age, surgery type, and SEER summary stage were all independent prognostic factors for OS chosen to develop a nomogram for SPN. C-indices of 0.927, 0.93, and 0.965 were found in the training, internal validation, and external verification cohorts respectively. The nomogram’s discriminative ability was proved by the AUC value (> 0.7), and the calibration curve showed alignment between the nomogram’s prediction and actual survival. Finally, the outcomes of DCA further demonstrated the nomogram’s clinical effectiveness. In conclusion, A nomogram was built and validated to help doctors to determine the prognosis of patients with SPN and tailor treatment options.

Solid pseudopapillary neoplasms (SPNs) are the most common entity among pediatric pancreatic tumors. Still, these are rare tumors with an annual incidence of 0.1–0.2/1,000,000, and little is known about their optimal treatment. This analysis aimed to increase knowledge about the occurrence and treatment strategies of SPN in childhood. Data regarding diagnostics, treatment, and outcome of children aged 0–18 years with SPN recorded in the German Registry for Rare Pediatric Tumors (STEP) were analyzed. Thirty-eight patients were identified with a median age of 14.5 years at diagnosis (range: 8–18) and a female preponderance (81.6%). The most frequent location of the tumor was the pancreatic tail. In histopathological and immunohistochemical examination, pseudopapillary, solid, and cystic lesions as well as expression of beta-catenin, progesterone receptors, and cyclin D1 were the most common findings. All patients underwent surgical resection. Most patients underwent open resection, predominantly tail resection for tumors in the tail region and pylorus-preserving pancreaticoduodenectomy for tumors in the head region. The main postoperative sequela was exogenous pancreatic insufficiency (23.7%), especially with SPN in the pancreatic head. No recurrence occurred during follow-up, although two patients underwent resection with microscopic residue. Conclusion : SPN of the pancreas in childhood are low-grade malignancies with usually favorable treatment outcomes. However, therapy can lead to relevant long-term sequelae. To prevent recurrence, complete surgical resection is recommended, sparing as much healthy pancreatic tissue as possible. Interdisciplinary collaboration between specialists is essential to optimize treatment. Molecular genetic analysis of these tumors could improve understanding of their genesis. What is Known: • Solid pseudopapillary neoplasms (SPNs) of the pancreas are very rare tumors in childhood. • Little is known about tumorigenesis, and there are no specific guidelines for treatment and follow-up in pediatric patients. What is New: • Characteristics, treatment, and outcome were comprehensively assessed in a large cohort of pediatric patients with SPN. • We propose recommendations for diagnosis, treatment, and follow-up of children with SPN, based on our analysis and considering published experience.

BackgroundSolid pseudopapillary neoplasms of the pancreas (SPNP) are rare tumors predominantly in young women. We report the largest single-center cohort study comparing resection of SPNP by laparoscopic approach (LA) and the open approach (OA).MethodBetween 2001 and 2021, 102 patients (84% women, median age: 30) underwent pancreatectomy for SPNP and were retrospectively studied. Demographic, perioperative, pathological, early and the long-term results were evaluated between patients operated by LA and those by OA.ResultsPopulation included 40 LA and 62 OA. There were no significant differences in demographics data between the groups. A preoperative biopsy by endoscopic ultrasound was performed in 45 patients (44%) with no difference between the groups. Pancreatoduodenectomy (PD) was less frequently performed by LA (25 vs 53%, p = 0.004) and distal pancreatectomy (DP) was more frequently performed by LA (40 vs 16%, p = 0.003). In the subgroup analysis by surgical procedure, LA-PD was associated with one mortality, less median blood loss (180 vs 200 ml, p = 0.034) and fewer harvested lymph nodes (11 vs 15, p = 0.02). LA-DP was associated with smaller median tumor size on imaging (40 vs 80mm, p = 0.048), shorter surgery (135 vs 190 min, p = 0.028), and fewer complications according to the median comprehensive complication index score (0 vs 8.7, p = 0.048). LA-Central pancreatectomy was associated with shorter surgery (160 vs 240, p = 0.037), less median blood loss (60 vs 200, p = 0.043), and less harvested lymph nodes (5 vs 2, p = 0.025). After a median follow-up of 60 months, two recurrences (2%) were observed and were unrelated to the approach.ConclusionsThe LA for SPNP appears to be safe, should be applied cautiously in case of PD for large lesion, and was not associated with recurrence.

Insulinoma-associated protein 1 (INSM1) is an important biomarker of Achaete-scute homolog-like 1-driven pathways. For diagnosis of pancreatic neuroendocrine tumors (PanNET), chromogranin A (CGA), synaptophysin (SYP), and neural cell adhesion molecule (NCAM) were also considered as potential biomarkers. However, it is often difficult to diagnose it immunohistochemically. Hence, we examined the expression pattern of INSM1 in pancreatic solid tumors. We detected INSM1, CGA, SYP, and NCAM immunohistochemically, in 27 cases of NET [pure type: 25 cases, mixed adenoneuroendocrine carcinoma (MANEC): 2 cases]. We included 5 cases of solid-pseudopapillary neoplasm (SPN), 7 cases of acinar cell carcinoma (ACC), and 15 cases of pancreatic ductal adenocarcinoma (PDAC) as the control group. Nuclear expression of INSM1 was found in all PanNET pure type cases. However, expression of INSM1 was negative in PDAC, ACC, and SPN in all cases, whereas faint expression was seen in the cytoplasm from SPN. MANEC comprises of two components: neuroendocrine carcinoma and adenocarcinoma components. The NET component was positive for INSM1 expression, whereas the PDAC component does not express INSM1, which aids in distinguishing these components. Our results suggest that INSM1 is a useful immunohistochemical marker for diagnosing pancreatic neuroendocrine tumor.