Comparison between solid pseudopapillary neoplasms of the pancreas and pancreatic ductal adenocarcinoma with cystic changes using computed tomography.

BACKGROUND: Solid pseudopapillary neoplasms of the pancreas (SPN) share similar imaging findings with pancreatic ductal adenocarcinoma with cystic changes (PDAC with cystic changes), which may result in unnecessary surgery. AIM: To investigate the value of computed tomography (CT) in differentiation of SPN from PDAC with cystic changes. METHODS: This study retrospectively analyzed the clinical and imaging findings of 32 patients diagnosed with SPN and 14 patients diagnosed with PDAC exhibiting cystic changes, confirmed through pathological diagnosis. Quantitative and qualitative analysis was performed, including assessment of age, sex, tumor size, shape, margin, density, enhancement pattern, CT values of tumors, CT contrast enhancement ratios, "floating cloud sign," calcification, main pancreatic duct dilatation, pancreatic atrophy, and peripancreatic invasion or distal metastasis. Multivariate logistic regression analysis was used to identify relevant features to differentiate between SPN and PDAC with cystic changes, and receiver operating characteristic curves were obtained to evaluate the diagnostic performance of each variable and their combination. RESULTS: When compared to PDAC with cystic changes, SPN had a lower age (32 years vs 64 years, P < 0.05) and a slightly larger size (5.41 cm vs 3.90 cm, P < 0.05). SPN had a higher frequency of "floating cloud sign" and peripancreatic invasion or distal metastasis than PDAC with cystic changes (both P < 0.05). No significant difference was found with respect to sex, tumor location, shape, margin, density, main pancreatic duct dilatation, calcification, pancreatic atrophy, enhancement pattern, CT values of tumors, or CT contrast enhancement ratios between the two groups (all P > 0.05). The area under the receiver operating characteristic curve of the combination was 0.833 (95% confidence interval: 0.708-0.957) with 78.6% sensitivity, 81.3% specificity, and 80.4% accuracy in differentiation of SPN from PDAC with cystic changes. CONCLUSION: A larger tumor size, "floating cloud sign," and peripancreatic invasion or distal metastasis are useful CT imaging features that are more common in SPN and may help discriminate SPN from PDAC with cystic changes.

Imaging differentiation of solid pseudopapillary neoplasms and neuroendocrine neoplasms of the pancreas.

Purpose: The present study aimed to compare the computed tomography (CT) and magnetic resonance imaging (MRI) features of solid pseudopapillary neoplasms (SPNs) and pancreatic neuroendocrine neoplasms (pNENs). Method: Lesion imaging features of 39 patients with SPNs and 127 patients with pNENs were retrospectively extracted from 104 CT and 91 MRI scans. Results: Compared to pNEN patients, SPN patients were significantly younger (mean age 51.8 yrs versus 32.7 yrs) and more often female (female: male ratio, 5.50:1 versus 1.19:1). Most SPNs and pNENs presented as well-defined lesions with an expansive growth pattern. SPNs more often appeared as round or ovoid lesions, compared to pNENs which showed a lobulated or irregular shape in more than half of cases (p<0.01). A surrounding capsule was detected in the majority of SPNs, but only in a minority of pNENs (<0.01). Hemorrhage occurred non-significantly more often in SPNs (p=0.09). Signal inhomogeneity in T1-fat-saturated (p<0.01) and T2-weighted imaging (p=0.046) as well as cystic degeneration (p<0.01) were more often observed in SPNs. Hyperenhancement in the arterial and portal-venous phase was more common in pNENs (p<0.01). Enlargement of locoregional lymph nodes (p<0.01) and liver metastases (p=0.03) were observed in some pNEN patients, but not in SPN patients. Multivariate logistic regression identified the presence of a capsule (p<0.01), absence of arterial hyperenhancement (p<0.01), and low patient age (p<0.01), as independent predictors for SPN. Conclusions: The present study provides three key features for differentiating SPNs from pNENs extracted from a large patient cohort: presence of a capsule, absence of arterial hyperenhancement, and low patient age.

Surgical Outcomes of Pancreatic Solid Pseudopapillary Neoplasm: Experiences of 24 Patients in a Single Institute.

Background and Objectives: The pancreatic solid pseudopapillary neoplasm (SPN), a rare tumor predominantly affecting young women, has seen an increased incidence due to improved imaging and epidemiological knowledge. This study aimed to understand the outcomes of different interventions, possible complications, and associated risk factors. Materials and Methods: This study retrospectively analyzed 24 patients who underwent pancreatic surgery for SPNs between September 1998 and July 2020. Results: Surgical intervention, typically required for symptomatic cases or pathological confirmation, yielded favorable outcomes with a 5-year survival rate of up to 97%. Despite challenges in standardizing preoperative evaluation and follow-up protocols, aggressive complete resection showed promising long-term survival and good oncological outcomes. Notably, no significant differences were found between conventional and minimally invasive (MI) surgery in perioperative outcomes. Histopathological correlations were lacking in prognosis and locations. Among the patients, one developed diffuse liver metastases 41 months postoperatively but responded well to chemotherapy and transcatheter arterial chemoembolization, with disease stability observed at 159 postoperative months. Another patient developed nonalcoholic steatohepatitis after surgery and underwent liver transplantation, succumbing to poor medication adherence 115 months after surgery. Conclusions: These findings underscore the importance of surgical intervention in managing SPNs and suggest the MI approach as a viable option with comparable outcomes to conventional surgery.

Laparoscopic Warshaw procedure in pediatric solid pseudopapillary neoplasm of the pancreas: Technical feasibility and oncological outcomes.

Achieving margin-negative resection is crucial in treatment of solid pseudopapillary neoplasm (SPN) of the pancreas, while preserving the spleen during distal pancreatectomy is highly desirable in pediatric cases. Laparoscopic Warshaw procedure (Lap-WT) is invaluable when tumor involvement in splenic vessels complicates preservation. However, the feasibility of Lap-WT in pediatric patients remains contentious. This study presents the clinical outcomes of three pediatric SPN cases who underwent Lap-WT. The median age was 10 years, with a median tumor size of 50 mm. Lap-WT demonstrated successful outcomes with a median operation time of 311 min and blood loss of 12 mL. No postoperative complications occurred, with a median length of hospital stay of 8 days. Long-term follow-up showed mild thrombocytopenia and increased spleen volume in two cases, perigastric varices in one, with no bleeding complications. No instances of tumor recurrence were observed. Lap-WT emerges as a feasible approach for pediatric SPN, ensuring spleen preservation without compromising oncological outcomes.

Curative Resection for Metastatic Solid Pseudopapillary Neoplasm of Pancreas-a Case Series.

Solid pseudopapillary neoplasm (SPN) is an unusual tumor of the pancreas. Unlike ductal adenocarcinoma, SPN is commoner in young women and is indolent with better prognosis. Fifteen percent of patients, however, develop metastases, often synchronous and involving the liver or peritoneum. Owing to the paucity of cases, management of the metastatic disease is not clearly defined. Retrospective review of case notes of patients treated between 2006 and 2019. There were 53 patients with SPN of which 4 had hepatic metastases-3 synchronous and 1 metachronous. Two patients underwent simultaneous distal pancreatectomy and splenectomy with liver resection (right posterior sectionectomy and metastasectomy). One required right hepatectomy with metastasectomy for metachronous liver metastases. The other underwent a staged operation-remnant tumor excision with metastasectomy followed by right hepatectomy after portal vein embolization. All four patients are recurrence free on median follow-up of 38.5 months. In view of the excellent prognosis, we recommend radical resection of both the primary lesion and metastases for SPN.

Solid pseudopapillary neoplasm (SPN) of the pancreas: current understanding on its malignant potential and management.

Solid pseudopapillary neoplasms (SPN) of the pancreas are presently recognized as low-grade malignant tumors that are frequently observed in young females. This tumor has a low incidence and is associated with an excellent prognosis following surgical resection. Typical SPNs primarily affect the pancreas and tend to have moderate or asymptomatic manifestations. Based on retrospective research, it is anticipated that patients with SPN can achieve disease-free survival, even in cases when metastasis is detected during inspection. However, the incidence of malignant SPN has been consistently underestimated, as evidenced by recent research findings. Malignancy of SPN primarily encompasses invasion and infiltration, metastasis, and recurrence after R0 resection. Imaging technologies such as Ultrasound, Computed Tomography, Magnetic Resonance Imaging, and Position Emission Tomography are capable of preliminarily identifying malignant SPN, which is primarily based on its invasive clinical features. Research on risk factors of malignant SPN revealed that larger tumor size, Ki-67 index, and several other parameters had significant correlations with invasive tumor behavior. Pathologic features of malignant SPNs overlay other pancreatic tumors, nevertheless they can provide valuable assistance in the process of diagnosis. Several confirmed specific pathologic biomarkers are related to its cellular origin, characteristic gene mutation, and cell proliferation. Considering the invasiveness of malignant SPN, it is imperative to enhance the comprehensiveness of its therapy. Tumor resection remains a suggested course of action in line with typical SPN, and additional lymph node dissection is seen as reasonable. Compared to benign SPNs, malignant SPNs have worse prognosis, underscoring the necessity of early identification and treatment in comprehensive medical centers to get improved clinical outcomes.

Outcome and survival were similar with laparoscopic and open pancreatectomy in 102 solid pseudopapillary neoplasms.

BACKGROUND: Solid 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). METHOD: Between 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. RESULTS: Population 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. CONCLUSIONS: The LA for SPNP appears to be safe, should be applied cautiously in case of PD for large lesion, and was not associated with recurrence.

Solid pseudopapillary neoplasm of the pancreas: a retrospective study of 195 cases.

Objective: Solid pseudopapillary neoplasm of the pancreas (SPN) is a rare exocrine tumor of the pancreas. The aim of our study is to summarize the clinical features of SPN and to analyze the risk factors for malignant SPN. Methods: From May 2013 to September 2022, patients who were pathologically confirmed to have SPN were retrospectively reviewed. Demographic data, clinical and pathological features, follow-up data were collected and analyzed. To investigate the factors influencing the benign or malignant nature of SPN, we employed logistic regression. Additionally, we utilized Kaplan-Meier curves to depict and analyze the overall prognosis. Results: A total of 195 patients were included, 163 of whom were female and the average age of all patients was 31.7 years old. Among 195 patients, 101 patients (51.8%) had no obvious clinical symptoms and their pancreatic lesions were detected during routine examination. The primary symptom was abdominal pain and distension in 64 cases (32.8%). The maximum diameter of SPN tumors ranged from 1-17 cm (mean 6.19 cm). Forty-eight postoperative complications developed in 43 (22.1%) patients. After a median follow-up duration of 44.5 months, the overall 5-year survival rate was 98.8% and the recurrence rate was 1.5%. Furthermore, we observed a statistically significant difference in the completeness of the tumor capsule between benign and malignant SPN. Conclusion: SPN is associated with a favorable long-term survival after surgery in our large sample size cohort. For malignant SPN, tumor capsule incompleteness is an independent risk factor.

Twenty-one years of experience with resected solid pseudopapillary neoplasm: a retrospective single-institutional cohort study.

BACKGROUND: Although the incidence of solid pseudopapillary neoplasm (SPN) is <2% of the incidence of pancreatic tumor, the prevalence seems to be increasing. SPNs are mostly benign. However, they also show malignant features. This study aimed to identify the clinical outcomes of patients who underwent surgery for SPN at a single center. METHODS: Data on 217 patients with SPN who underwent surgery in Samsung Medical Center between 2000 and 2020 were retrospectively analyzed. RESULTS: Herein, the mean age of the 217 patients was 40.0 ± 12.6 years, with a female predominance (80.6%). Most patients had no comorbidity. The mean tumor size was 4.4 ± 3.1 cm. The tumor was located at the pancreatic head in 36 patients (16.6%), the body of the pancreas in 69 patients (31.8%), and the pancreatic tail in 96 patients (44.2%). Of note, 35 patients (16.1%) underwent pancreaticoduodenectomies, 148 patients (68.2%) had distal pancreatectomies, and the other patients had subtotal /total pancreatectomy (9.7%) or enucleation/mass excision (6.0%). No patient had lymph node (LN) metastasis. Moreover, 6 patients (2.8%) had a recurrence in the liver or regional LNs. The 5-year recurrence-free survival rate was 96.8%. The only factor affecting recurrence was tumor size (P = .007). CONCLUSION: Because SPN predominates in relatively young women, patients often hesitate to undergo surgery. Nevertheless, as size is the prognostic factor, early resection is recommended for a better prognosis in the case of surgically feasible, young age, and healthy patients.

Case report: Retroperitoneal solid pseudopapillary neoplasm associated with multiple hepatic metastases.

Solid pseudopapillary neoplasm (SPN) is a rare tumor mostly occurring in the pancreas. They are low-grade malignant tumors of the exocrine pancreas that occasionally metastasize, usually to the liver or peritoneum. Additionally, multiple metastases of extrapancreatic SPN to the liver are extremely rare and have been reported before. This study presents a case of a 13-year-old male patient with retroperitoneal SPN and multiple hepatic metastases. The patient presented with abdominal trauma and underwent enhanced CT, which revealed upper pancreatic occupancy and three hypodense foci in the right lobe of the liver. Moreover, increased spleen size was noted. The patient's serum tumor marker CA125 was increased to 39.00 U/mL (N < 35.0 U/mL), and circulating tumor cells were elevated to 10.2 FU/3 mL (N < 8.7 FU/3 mL). The patient underwent retroperitoneal occupancy resection and splenectomy, followed by resection of liver metastases 7 months after the surgery. Furthermore, multiple liver metastases from retroperitoneal SPN were confirmed postoperatively. The patient recovered for 1 year without tumor recurrence. This case emphasizes the importance of evaluating serum tumor markers and medical imaging in young patients as well as the fact that surgery appears to be the preferred treatment option for multiple metastases in SPN.

Multicenter Study on the Performance of Imaging Tests Compared to Endosonography-Guided Fine-Needle Aspiration in the Diagnosis of Solid Pseudopapillary Neoplasms of the Pancreas.

Introduction: Imaging diagnosis of pancreatic solid-pseudopapillary neoplasms (SPNs) is difficult. Preoperative diagnosis by endosonography-guided fine-needle aspiration (EUS-FNA) is possible and has been reported in the literature in pancreatic tumors. However, its usefulness is still controversial. The aim of this study was to determine the accuracy of the EUS-FNA in the diagnosis of patients with SPN and describe the findings in computerized tomography (CT), magnetic resonance cholangiopancreatography imaging (MRI/MRCP), and EUS therefore comparing the imaging methods alone to the findings of microhistology (McH) obtained by EUS-FNA. Materials and Methods: We retrospectively reviewed the medical records of patients undergoing EUS-FNA with suspected SPN in imaging studies in 5 Brazilian high-volume hospitals (two university hospitals and three private hospitals). The demographic data; findings in CT, MRI/MRCP, and EUS; and McH results obtained by EUS-FNA were noted prospectively. The final diagnosis was obtained after the anatomopathological examination of the surgical specimen in all patients (gold standard), and we compared the results of CT, MRI/MRCP, EUS, and the McH with the gold standard. Results: Fifty-four patients were included in the study, of which 49 (90.7%) were women with an average age of 33.4 (range 11-78) years. The most common symptom presented was abdominal pain, present in 35.2% patients. SPN was detected incidentally in 32 (59%) patients. The average size of the tumors was 3.8 cm (SD: 2.26). The most common finding at EUS was a solid, solid/cystic, and cystic lesion in 52.9%, 41.1%, and 7.8% patients, respectively. The final diagnosis was 51 patients with SPN and 3 with nonfunctioning pancreatic neuroendocrine tumors (NF-NET). The correct diagnosis was made by CT, MRI/MRCP, EUS isolated, and EUS-FNA in 21.9%, 28.88%, 64.71%, and 88.24%, respectively. EUS-FNA associated with CT and MRI increased diagnostic performance from 22.72% to 94.11% and from 29.16% to 94.11%, respectively. Conclusions: SPN are rare, incidentally identified in most cases, and affect young women. Differential diagnosis between SPN, NF-NET, and other types of tumors with imaging tests can be difficult. EUS-FNA increases preoperative diagnosis in case of diagnostic doubt and should be used whenever necessary to rule out NF-NET or other type of solid/cystic nodular lesion of the pancreas.

Introdução: O diagnóstico por imagem da neoplasia pseudopapilar sólida do pâncreas (NPS) é difícil. O diagnóstico pré-operatório obtido pela endosonografia com punção aspirativa por agulha fina (USE-PAF) é possível e tem sido relatado na literatura em tumores do pâncreas. No entanto, sua indicação é controversa e merece discussão. O objetivo do estudo foi determinar a acurácia da USE-PAF no diagnóstico de pacientes com NPS, descrever os achados da tomografia computadorizada (TC), colangiopancreatografia por ressonância magnética (RM/ CPRM) e USE, comparando os métodos de imagem isolados aos achados da microhistologia (McH) obtida pela USE-PAF. Material e Métodos: Revisamos retrospectivamente os prontuários de pacientes submetidos à USE-PAF com suspeita de NPS em exames de imagem de 5 hospitais brasileiros de alto volume (dois universitários e três privados). Foram anotados prospectivamente os dados demográficos, os achados da TC, RM/CPRM e USE e o resultado da McH obtida pela USE-PAF. O diagnóstico final foi obtido após o anatomopatológico da peça operatória em todos os pacientes (padrão-ouro). Comparamos os resultados da TC, RM/CPRM, EUS isoladas e da McH obtida pela USE-PAF com o padrão-ouro. Resultados: Cinquenta e quatro pacientes foram incluídos no estudo, 49 (90.7%) eram mulheres com média de idade de 33.4 (11­78) anos. O sintoma mais frequente foi dor abdominal, presente em 35.2%. A NPS foi detectada acidentalmente em 32 (59%) pacientes. O tamanho médio da lesão foi de 3.8 cm (SD: 2.26). O achado mais comum à USE foi lesão sólida, sólida/ cística e cística em 52.9%, 41.1% e 7.8%, respectivamente. O diagnóstico final foi NPS (51) e tumor neuroendócrino pancreático não funcionante [NF-NET] (3). O diagnóstico correto feito pela TC, RM, USE e USE-PAF foi feito em 21.9%, 28.9%, 64.7% e 88.2%, respectivamente. A USEPAF associada a TC e a RM aumentou o desempenho diagnóstico de 21.9% para 94.1% e de 28.8% para 94.1%, respectivamente. Conclusões: NPS são raras, identificadas de forma acidental na maioria dos casos e afetam principalmente mulheres jovens. O diagnóstico diferencial entre NPS, NF-NET e outros tipos de lesões com exames de imagem isolados pode ser difícil. A USE-PAF aumenta a chance do diagnóstico pré-operatório em caso de dúvida diagnóstica e deve ser usado sempre que necessário para descartar NF-NET ou outro tipo de lesão nodular sólida ou sólido/cística do pâncreas.

Surgical outcome and prognosis of pediatric solid-pseudopapillary neoplasm.

BACKGROUND: The aim of this study was to clarify the characteristics and outcomes of pediatric patients with solid pseudopapillary neoplasms (SPNs) who underwent pancreatectomy. METHODS: Pediatric patients with SPNs who underwent pancreatectomy at our institution between 1995 and 2020 were included in the study. RESULTS: During the period under review, 12 patients underwent pancreatectomy for SPNs (median age: 10 years; range: 6-15 years). The surgical procedures included pancreatoduodenectomy (n = 2; 16.6%), distal pancreatectomy (n = 3; 25%), and enucleation (n = 7; 58.3%). The most common postoperative complication was postoperative pancreatic fistula (n = 6; 50%). Patients who underwent enucleation tended to have higher postoperative complication rates compared with those who underwent other procedures. All patients were alive without recurrence at the end of the study period. CONCLUSIONS: SPN is associated with a good prognosis, regardless of the surgical procedure. If surgeons select enucleation for pediatric SPNs, they should bear in mind that it is associated with a higher complication rate.

Central pancreatectomy: A viable option for solid pseudopapillary neoplasm - A case report.

INTRODUCTION: Solid pseudopapillary neoplasms (SPNs) of the pancreas are rare tumors, comprising about 1 % of pancreatic tumors. They primarily affect females during their reproductive phase and have a favorable prognosis. SPNs are usually asymptomatic or present with mild symptoms. The exact histopathogenesis of SPNs remains unknown. Surgical resection is curative, and central pancreatectomy is a pancreas-sparing surgical technique. CASE PRESENTATION: A 33-year-old female presented with epigastric pain, vomiting, and infertility. Imaging revealed a mass in the pancreas. Exploration confirmed the mass, and central pancreatectomy was performed. Histopathology confirmed the diagnosis of SPN. The patient's recovery was uneventful, and follow-up CT scans showed no recurrence. DISCUSSION: This case involves a 33-year-old female presenting with epigastric pain and vomiting, revealing a cystic mass with a solid component in the pancreas. While generally benign, SPNs can become malignant in 15 % cases, with a favorable prognosis. Histopathologically, SPNs remain distinct, with CD99 and CD10 staining confirming the diagnosis. Diagnostic imaging, particularly CT scans, aids in identifying SPNs. Surgical resection, such as central pancreatectomy, is effective, preserving organ function. The case's positive outcome aligns with an overall 5-year survival rate of 95-97 %, highlighting the overall favorable prognosis of SPNs. The procedure's balance between tumor removal and organ preservation offers clinical advantages. CONCLUSION: This case underscores the successful management of an SPN using central pancreatectomy. It highlights the importance of early diagnosis and surgical intervention, as well as the favorable prognosis associated with SPNs, even in cases of metastasis. Central pancreatectomy offers organ preservation and reduces long-term complications. Continued reporting and research on such cases contribute to refining treatment strategies for SPNs.

Solid pseudopapillary neoplasm in a woman presenting with acute pancreatitis: a case report and review of literature.

Solid pseudopapillary neoplasm (SPN) is a rare pancreatic tumor that typically affects young women in the body and tail of the pancreas. SPN is often asymptomatic in the early stages, so it is initially discovered as a large tumor. In this report, we experienced a case of a relatively small SPN discovered in the setting of acute pancreatitis. Because there have been few reports of SPN being discovered in the situation like our case, we report this case based on a review of the literature.

Complete enucleation of a complicated solid pseudopapillary neoplasm of pancreas: A case report.

INTRODUCTION: Solid pseudopapillary neoplasm (SPN) of the pancreas is a rare pancreatic tumor, usually affecting young females. It is categorized as a low-grade malignant tumor without any specific epithelial differentiation, which can occur anywhere in the pancreas. CASE PRESENTATION: Here, we present the case of a 35-year-old lady who presented to us with abdominal pain and a pancreatic mass. She had a prior laparotomy at a different center without any specific intervention for the lump. After presenting to our center, she was managed through proper evaluation and adequate preparation for surgery. The diagnosis was challenging, and so was the surgery. We had enucleated the lesion completely. Histopathology confirmed the diagnosis of SPN. There are no signs of recurrence after two years. DISCUSSION: Patients are either asymptomatic or usually present with abdominal pain, a large abdominal lump, or some vague symptoms. A high index of suspicion is the key to diagnosis. Complete resection is the gold standard of treatment. Enucleation is also a good option in difficult cases. The prognosis after surgery is excellent. CONCLUSION: Total enucleation of the SPN of the pancreas is a reasonable alternative in selected cases when performed by experienced hepatobiliary-pancreatic surgeons.

Solid pseudopapillary neoplasms of the pancreas in childhood and adolescence-an analysis of the German Registry for Rare Pediatric Tumors (STEP).

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.

Solid pseudopapillary neoplasm: Report of a case of primary ovarian origin and review of the literature.

Background: Solid pseudopapillary neoplasms (SPNs) are uncommon tumors of low malignancy with a generally favorable prognosis, mostly originating from the pancreas. To date, 12 cases of SPNs with a primary ovarian origin (SPN-Os) have been reported globally, and their detailed characteristics have not been fully elucidated. Case description: We reported the 13th SPN-O case, which occurred in a 52-year-old woman with an 18.5 cm left ovarian mass. Four imaging methods, including ultrasound, computed tomography, magnetic resonance imaging and positron emission tomography, were utilized before surgery. An elevated level of serum cancer antigen 125 was detected and a total hysterectomy plus bilateral salpingo-oophorectomy was performed. Microscopic examination revealed a typical solid pseudopapillary structure. The tumor cells were stained focally for pan-cytokeratin, synaptophysin, CD99 and CD10, while ß-catenin, vimentin and CD56 were diffusely expressed. The Ki-67 proliferation index was 3%, and immunohistochemical (IHC) staining for chromogranin-A, inhibin-a, and E-cadherin was negative. No evidence of recurrence or metastasis was observed by clinical and imaging data during a 5-month postoperative follow-up. Conclusion: This is a report of an unusual case of a primary ovarian SPN with an up-to-date review of SPN-Os. A minimum combination of imaging methods and IHC stains was proposed for SPN-Os, which may prove beneficial in clinical practice.

Precision anatomy for minimally invasive spleen-preserving distal pancreatectomy in children: A case report of solid-pseudopapillary neoplasm in a 12-year-old girl.

INTRODUCTION: Solid pseudopapillary neoplasm (SPN) is a rare and low malignant tumor found mainly in young females. There is no standardized procedure for SPN of the pancreatic body and tail in children. In adults, an international consensus on precision anatomy for minimally invasive distal pancreatectomy (MIDP) was established recently (PAM-HBP Surgery Project). The aim of this study is to demonstrate that precision anatomy can also be safely and effectively implemented in the pediatric population. PRESENTATION OF CASE: A 12-year-old girl with an incidentally found SPN located in the pancreatic tail was referred to our hospital. She successfully underwent an R0 resection by laparoscopic spleen-preserving distal pancreatectomy (LSPDP) under the concept of precision anatomy. The patient recovered uneventfully and was discharged on day 7. DISCUSSION: This is the first successful report of LSPDP under the concept of precision anatomy in children. In accordance with the recommendations from the international consensus, the "anterior approach" was selected to dissect and encircle the splenic artery based on the vascular anatomy identified by preoperative imaging. The dorsal dissection border of the pancreas along the anterior layer above the Gerota's fascia was carefully maintained and the splenic vessels were preserved taking into consideration the low malignant potential of SPN and to decrease the risk of complications associated with splenectomy, which were also the essential issues of the consensus. CONCLUSION: The implementation of precision anatomy for pediatric pancreas surgery should facilitate the safe diffusion of MIDP for SPN and other benign or low-malignant tumors in children.