Sudden rupture of a giant solid pseudopapillary tumour of the pancreas during caesarean delivery in a pregnant woman with severe preeclampsia: A case report and literature review.

INTRODUCTION AND IMPORTANCE: Nonspecific presentations during pregnancy can mask early signs and symptoms of upper abdominal tumours, making the preoperative diagnosis of upper abdominal tumours difficult. Solid pseudopapillary neoplasm of the pancreas (SPN) is a rare exocrine tumour of the pancreas, and SPN in combination with preeclampsia during pregnancy is even rarer. CASE PRESENTATION: In this paper, we report a case of SPN combined with preeclampsia during pregnancy and sudden rupture of a giant retroperitoneal SPN during a caesarean section, which resulted in life-threatening intra-abdominal haemorrhage. After exclusion of obstetric factors, a rapid response team was activated, multidisciplinary treatment (MDT) was carried out, and the patient was treated promptly and appropriately by resection of the giant retroperitoneal tumour, partial resection of the body and tail of the pancreas, and abdominal drainage. CLINICAL DISCUSSION: To our knowledge, this is the first reported case of SPN combined with preeclampsia during pregnancy, and a rapid and timely MDT could have ensured the patient's life. CONCLUSION: When dealing with a pregnant woman with an acute abdomen, the obstetrician should communicate fully with the woman to ensure that the most likely diagnosis is obtained. In the event of an unexpected accident during a caesarean section, it is important to remain calm, activate the Rapid Response Team and seek an MDT to ensure the life of the mother.

Pediatric Solid Pseudopapillary Neoplasm of the Pancreas.

Background: Solid pseudopapillary neoplasm (SPN) of the pancreas in children is a rare tumor with low malignant potential. Some tumors, however, behave aggressively. There is very little literature on managing these variants, especially in children. We share our experience of managing large and recurrent SPN and explore the clinicopathological findings correlating to the risk of recurrence. Methods: This is a retrospective study of children treated for SPN between 2012 and 2022 at a tertiary care center in India. The clinicopathological features and management strategies in these children were evaluated. Results: Sixteen children with SPN were treated during this period (88% of girls). The median age of presentation was 12 years (interquartile range [IQR]: 9-14). All children presented with abdominal pain. Computed tomography gave a definitive diagnosis in 81% of cases. The tumor predominantly involved the head of the pancreas (n = 9, 56%). Eight of nine children classified as high-grade (HG) malignant had a benign course. One child had a recurrence of the tumor 4 years after the initial resection and further recurrence on chemotherapy. She required radiation therapy in addition to reoperation following which she was disease free for 77 months. The overall median follow-up was 46 months (IQR: 18-72 months). Conclusion: Complete resection of the tumor provides a cure in most patients with SPN. Recurrent tumors require a multi-modality approach. Long-term survival is good. There is a need for clear definitions of the components within the WHO criteria for HG malignancy.

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.

Single-cell RNA sequencing of solid pseudopapillary neoplasms of the pancreas in children.

Solid pseudopapillary neoplasm (SPN) of the pancreas is a rare pancreatic tumor in children. Its origin remains elusive, along with its pathogenesis. Heterogeneity within SPN has not been previously described. In addition, low malignant but recurrent cases have occasionally been reported. To comprehensively unravel these profiles, single-cell RNA sequencing was performed using surgical specimens. We identified the cell types and suggested the origin of pancreatic endocrine progenitors. The Wnt/ß-catenin pathway may be involved in tumorigenesis, while the epithelial-to-mesenchymal transition may be responsible for SPN recurrence. Furthermore, NOV, DCN were nominated as primary and S100A10, MGP as recurrent SPN marker genes, respectively. Our results provide insight into the pathogenesis of SPN.

Hepatocellular carcinoma and solid pseudopapillary neoplasm of the pancreas complicating familial adenomatous polyposis: two cases and review of the literature.

Familial adenomatous polyposis (FAP) is characterized by colorectal polyposis and extracolonic tumors. Adenocarcinoma of the pancreas and hepatocellular carcinoma are rare in FAP. In this case series, we describe a mother and daughter with FAP who developed a hepatocellular carcinoma and solid pseudopapillary neoplasm of the pancreas, respectively.

Solid pseudopapillary neoplasm of the pancreas.

Solid pseudopapillary neoplasms are rare. This article reviews the clinical and pathologic features of solid pseudopapillary neoplasm of the pancreas, including the epidemiology, cytology, molecular pathology, differential diagnosis, treatment, and prognosis. Solid pseudopapillary neoplasms are low-grade malignant tumours of the pancreas characterized by poorly cohesive epithelial cells with solid and pseudopapillary patterns. Solid pseudopapillary neoplasms occur predominantly in young women. Although solid pseudopapillary neoplasms can occur throughout the pancreas, they arise slightly more frequently in the tail of the pancreas. The aetiology is unknown. Extremely rare cases have been reported in the setting of familial adenomatous polyposis. There are no symptoms unique to solid pseudopapillary neoplasms, however, the most common symptom is abdominal pain or discomfort. The features of solid pseudopapillary neoplasms on computed tomography imaging are indicative of the pathologic changes within the tumour. Typically, well-demarcated masses with variably solid and cystic appearances. Microscopically, these tumours are composed of epithelial cells forming solid and pseudopapillary structures, frequently undergoing haemorrhagic cystic degeneration. Typically, these tumours express nuclear and/or cytoplasmic ß-catenin. Almost all solid pseudopapillary neoplasms harbour mutations in exon 3 of CTNNB1, the gene encoding ß-catenin. The overall prognosis is excellent, and most patients are cured by complete surgical resection.

Combined Laparoscopic spleen-preserving distal pancreatectomy and autologous islet transplantation: report of one case and literature review / 中华器官移植杂志

Objective:To Eveluate the safty and clinical efficacy of combined laparoscopic spleen-preserving distal pancreatectomy and autologous islet transplantation in the treatment of solid pseudopapillary neoplasm.Methods:A 22 years old solid pseudopapillary neoplasm female patient who underwent distal pancreatectomy and an autologous islet transplantation at Tianjin First Central Hospital, clinical date for 6 months follow up was collected and analyzed.Results:The patient was well recovered after surgery, and during the post-operative follow up, the fasting blood glucose was 5.72 mmol/L, HbA1c was 6.1%, remained insulin independent, the liver function was kept well.Conclusions:Combined Laparoscopic spleen-preserving distal pancreatectomy and autologous islet transplantation can effectively prevent diabetes after distal pancreatectomy.

Neoplasia Pseudopapilar sólida del Páncreas. A Propósito de un Caso / Solid Pseudopapillary Neoplasia of the Pancreas. Case Report

Introducción: La neoplasia pseudopapilarsólida del páncreas es una patología poco frecuente, que se presenta en mujeres jóvenes y es de comportamiento incierto. La prevalencia es del 1-2% y el tratamiento es quirúrgico. En el presente caso se describe a una paciente de sexo femenino de 10 años de edad que muestra esta patología y su correspondiente manejo. Caso clínico:Paciente de sexo femenino, de 10 años de edad, con antecedentes de una nefrectomía hace 6 años por hidronefrosis, refiere dolor abdominal localizado en egigastrio tipo cólico y pérdida de peso. En los estudio de imagen a nivel peritoneal se observa lesión de aspecto ocupativo de tipo sólido neoplásico con área central de probable necrosis y que desplaza en sentido posterior al páncreas así como al estómago y se asocia con adenomegalias peritoneales y retroperitoneales. Evolución:Lapaciente fue sometido a una espleno-pancreatectomía distal.El estudio anatomo-patológico concluyó en neuplasia pseudopapilar sólida del páncreas.Continúa en valoración por consulta externa. Conclusión:Neoplasia Pseudopapilar sólida del Páncreasdebe ser sospechada en pacientes con una masa sólida o quística pancreática con un estudio de imágenes compatibles. Palabras clave:Neoplasia pseudopapilar sólida del páncreas, prevalencia, inmunohistoquímica, receptores progestágenos,Informe de caso

Introduction:Solid pseudopapillary neoplasia of the pancreas is a rare pathology that occurs in young women and has an uncertain behavior. The prevalence is 1-2% and the treatment is surgical. In the present case, a 10-year-old female patient with this pathology and its corresponding management is described. Clinical case: A 10-year-old femalepatient with a history of nephrectomy 6 years ago due to hydronephrosis, complains of colicky localized abdominal pain in the egigastrium and weight loss. In peritoneal imaging studies, a neoplastic solid type occupational lesion with a central area of probable necrosis is observed, which displaces the pancreas as well as the stomach posteriorly and is associated with peritoneal and retroperitoneal adenomegaly. Evolution: The patient underwent a distal spleno-pancreatectomy. The anatomopathological studyconcluded in solid pseudopapillary neuplasia of the pancreas. He continues to be evaluated by external consultation. Conclusion: Solid Pseudopapillary Neoplasia of the Pancreas should be suspected in patients with a solid or cystic pancreatic mass with a compatible imaging study. Key words:Solid pseudopapillary neoplasm of the pancreas, prevalence, immunohistochemistry, progestin receptors, Case Report.

Sex differences in solid pseudopapillary neoplasm of the pancreas: A population-based study.

OBJECTIVE: Solid pseudopapillary neoplasm (SPN) of the pancreas is a rare tumor. This study aims to examine the clinicopathological features and surgical treatments of SPN and compare the clinical behavior and prognosis between men and women with SPN. METHODS: We collected the population data of patients with SPN diagnosed between 2004 and 2017 from the SEER database. The Kaplan-Meier method was used to analyze overall survival (OS) and disease-specific survival (DSS), and log-rank tests were used to evaluate the differences between subgroups. Univariate and multivariate Cox regression analyses were performed to screen out prognostic risk factors of SPN. RESULTS: A total of 378 patients with SPN were included, with 246 (65.1%) female patients. 1-, 3-, and 5-year overall survival rates were 98.9%, 95.7%, and 93.7%, respectively. Survival analysis revealed that regardless of stage, patients with SPN who underwent surgical interventions still had a significantly better prognosis than those without surgical interventions (P < .001). The patients with lymphatic dissection had a significantly better prognosis than those without lymphatic dissection (P < .001). Moreover, compared with female patients, male patients had significantly poorer OS and DSS (P < .001). Female SPN showed a bimodal age-frequency distribution with early-onset incidence at 28 years and late-onset peak incidence at 62 years, while male SPN presented a unimodal distribution with peak incidence at approximately age 64 years. In female patients, the tumor size in premenopausal females (<65 years old) was significantly larger than that in postmenopausal females (≥65 years old) (P < .001). Clinicopathological characteristic profiles were different not only between male SPN and premenopausal female SPN but also between premenopausal and postmenopausal female SPN. CONCLUSION: SPN presents indolent behavior and predominantly occurs in young women. Regardless of stage, surgical intervention is recommended. Moreover, our study is the first large enough study to demonstrate sex-related discrepancies in SPN. Thus, different treatment strategies should be designed for patients of different sexes at different ages and hormone therapy is a promising approach for SPN.

Early Diagnosis of a Pediatric Pancreatic Tumor by Abdominal Physical Examination: A Case Report.

BACKGROUND: Detecting a neoplasm in pediatric patients with acute abdominal pain is difficult, especially when there is no palpable mass. Ihara's maneuver, which allows the physician to apply manual pressure directly on the pancreatic body, is a useful palpation method of diagnosing acute pancreatitis in children. We report a case of solid pseudopapillary neoplasm of the pancreas (SPNP) detected by Ihara's maneuver. CASE REPORT: An otherwise healthy, 15-year-old male visited our emergency department with acute abdominal pain and nausea. His vital signs were appropriate for his age. A physical examination denied peritoneal signs, but Ihara's maneuver induced strong tenderness. His serum amylase and lipase were normal. A contrast-enhanced computed tomography scan revealed a well-defined, 2.2-cm, nonenhanced mass in the pancreatic tail. Laparoscopic distal pancreatectomy was performed, and the diagnosis of SPNP was confirmed. The patient was well postoperatively without any metastasis. SPNP is a rare neoplasm with low malignant potential. Although it typically occurs in young females, it has also been reported in children. The early diagnosis of SPNP is usually challenging because most patients do not have specific symptoms or laboratory findings. In the present case, the SPNP was difficult to detect by palpation because of its size, but Ihara's maneuver induced strong tenderness of the pancreas and led to a diagnosis. WHY SHOULD AN EMERGENCY PHYSICIAN BE AWARE OF THIS?: Ihara's maneuver has the potential to enable early diagnosis not only of pancreatitis but also of pancreatic tumors, such as SPNP.

Solid pseudopapillary neoplasm of the pancreas in children: Hacettepe experience.

BACKGROUND: Solid pseudopapillary neoplasm of the pancreas (SPNP) is mostly seen in young women in the second and third decades of life; it is quite uncommon in children. We aimed to review our institutional experience with SPNP in children. METHODS: Hospital charts of children <18 years of age diagnosed to have SPNP were reviewed for demographic characteristics, presenting symptoms, diagnostic interventions, physical examination findings, radiological data, extent of disease, diagnostic and management strategies and final outcome. RESULTS: Nineteen cases were diagnosed as SPNP between 1992 and 2017 (female: male, 16:3; median age 13 years, range 8.5-16.5). The most common symptom was abdominal pain. Physical examination was normal in 12/19 cases. Three cases were diagnosed incidentally. The most common tumour localization was the head of the pancreas. Median tumour diameter was 5 cm (1.4-15). One patient had abdominal disseminated disease, and others had localized disease. Surgical interventions were enucleations in nine, distal pancreatectomies in four and total resection in two patients; three underwent no surgery following diagnosis. Only one patient received adjuvant chemotherapy and radiotherapy. One patient died, one was lost to follow-up at 164 months and 17 were under follow-up with no events at a median of 60 months (20-308 months). CONCLUSION: In childen, SPNP demonstrates different clinical features. Complete resection is curative in most patients. In children, the optimal surgical strategy for SPNP is still debatable. Instead of radical resections, limited pancreatic resections, such as enucleations, with negative surgical margins should be attempted. For unresectable or recurrent tumours, cisplatin and 5-FU-based chemotherapy might be considered.

Solid pseudopapillary tumour of the pancreas: clinicopathological analysis.

BACKGROUND: We report on our experience of the surgical management and outcomes of 11 patients with solid pseudopapillary tumour of the pancreas (SPT). We sought to correlate the immunohistochemical staining of these tumours with that previously reported in the literature. METHODS: A retrospective analysis of the clinical presentation, radiological findings, surgical treatment, histopathological characteristics and outcomes for patients surgically managed with SPT at The Royal London Hospital. A literature search was performed to analyse the immunohistochemical stains commonly used to diagnose SPT. RESULTS: Between August 2006 and April 2016, 10 females and one male patient underwent surgery for SPT. The localization of the tumour was in the pancreatic head in two patients, one in the neck, three in the body and five in the tail. All 11 patients had localised disease. Six patients suffered post-surgical complications. Histopathology shows immunoreactivity for: ß-catenin, vimentin, CD-10, CD-56, α1-antitrypsin and negative staining for synaptophysin and chromogranin. At a median of 24 months of follow-up, the disease-free survival rate was 100% and no recurrence was noted. A literature review generated 38 suitable articles with 116 individual cases of SPT, with high expression of vimentin and neuron specific enolase throughout, and low rates of chromogranin and synatophysin positivity. CONCLUSION: SPT is rare and affects mostly young women. An accurate diagnosis is important as the relative indolent behaviour can be managed with surgical resection even when large in size, bringing excellent long-term outcomes.

Hyaline globules and papillary fragments in cytologic smears from two intra-abdominal tumors (ovarian and hepatic) in female patients: A diagnostic pitfall with histologic correlation.

Hyaline globules and papillary fragments in cytologic samples from two intra-abdominal tumors in young females are presented including the cytological features and the correlation with the histopathologic and immunohistochemical findings. In the first case a cytologic study from an ovarian mass showed papillary structures and isolated tumor cells with epithelioid morphology, irregular reniform-like nuclear contour, pale or vacuolated cytoplasm, abundant hyaline globules and occasional glomeruloid structures resembling Schiller-Duval bodies. Yolk sac tumor (YST) was the diagnosis on the histological slides. Tumor cells showed positivity for cytokeratin (AE1/AE3), epithelial membrane antigen (EMA), alpha-fetoprotein (AFP) and Sal-like protein 4 (SALL4). In case number two the cytologic study from a liver metastasis displayed papillary and rosette-like clusters composed of uniform and bland cells showing occasional long cytoplasmic tails, hyaline globules and nuclear grooves. A diagnosis of hepatic metastasis from solid pseudopapillary neoplasm of the pancreas (SPNP) was rendered from the histology. Tumor cells revealed immunoreactivity for cytokeratin (AE1/AE3), Vimentin, Galectin-1 (GAL-1), Neuron specific-enolase, CD10, progesterone and ß-catenin (nuclear stain). Regarding differential diagnosis, in the patient with the ovarian mass an ovarian clear cell carcinoma was considered, as well as other germ cell tumors or metastatic carcinoma, while in the patient with a liver metastasis a neuroendocrine carcinoma was taken into account. YST and SPNP share some cytological findings, including hyaline globules, papillary structures, clear cells and intercellular eosinophilic basement membrane deposits. Thus, a detailed study and careful interpretation of the cytological, histological and immunohistochemical findings may be worthwhile to avoid a potential misdiagnosis, particularly in the cytologic specimens of the ovarian and/or intra-abdominal mass, when involving young females. Diagn. Cytopathol. 2016;44:935-943. © 2016 Wiley Periodicals, Inc.

Laparoscopic pylorus preserving pancreaticoduodenectomy in paediatric age for solid pseudopapillary neoplasm of head of the pancreas - case report.

Solid pseudopapillary neoplasm (SPN) of the pancreas is a rare tumour commonly seen in young women without significant clinical features. SPN is usually a lowgrade malignant neoplasm which warrants resection. Recurrence and metastasis is seen rarely after complete resection. Pancreaticoduodenectomy is indicated for SPN situated in head of the pancreas which is generally performed by open approach. Laparoscopic pancreaticoduodenectomy (LPD) is difficult to perform for this condition because of smaller size of pancreatic and hepatic ducts more so in paediatric population. We report a case of 12 years old girl having SPN arising from head of the pancreas. She underwent laparoscopic pylorus preserving pancreaticoduodenectomy. Post-operative period was uneventful. Histological examination of resected specimen confirmed diagnosis of SPN. At 6 months follow up, she was doing well without any recurrence. To best of our knowledge, no case of LPD in paediatric patients is reported in literature available to us.

Preoperative diagnosis and surgical management for solid pseudopapillary neoplasm of the pancreas.

BACKGROUND: Preoperative diagnosis of solid pseudopapillary neoplasm of the pancreas (SPN) remains difficult and optimal surgical management for SPN has yet to be fully defined. METHODS: Retrospective analysis was undertaken of all 10 patients (six women, four men) who underwent surgery for SPN between 2001 and 2013. RESULTS: Mean age was 26 years (range, 16-33 years) for women, and 50 years (range, 35-76 years) for men. Although large SPN showed typical imaging findings, small SPN (≤ 3.0 cm) appears as almost entirely solid tumors. Endoscopic ultrasound-guided fine-needle aspiration (EUS-FNA) was performed in six patients with atypical findings of SPN for differentiation from other pancreatic neoplasms. Definitive preoperative cytological diagnosis was achieved in all patients who underwent EUS-FNA. All 10 patients underwent surgical exploration. One patient with portal vein invasion and multiple lung metastases underwent pancreaticoduodenectomy combined with portal vein resection and reconstruction, followed by two pulmonary resections. This patient remains alive as of 34 months after the initial operation. CONCLUSIONS: Endoscopic ultrasound-guided fine-needle aspiration is useful for definitive preoperative diagnosis of SPN. As long-term survival after surgical resection can be achieved even in patients with locally advanced and metastatic SPN, aggressive surgical resection should be performed.

Effect of Isotretionin for Refractory Solid-pseudopapillary Tumor of the Pancreas which Previously did not Respond to Anticancer Drugs and Radiation Therapy / 계명의대학술지

Solid pseudopapillary neoplasm (SPN) of the pancreas is an exocrine tumor with low malignant nature. Although it's treatment of choice is complete surgical resection, the patients who are not eligible for surgical therapy need other kinds of therapy and there are little reports. Vitamin A and its derivatives in some tumors are known to be prohibiting the tumors' growth by reducing the manifestation of transcription factors. We present one case of SPN of the pancreas, which was not eligible for resection and was not responding to chemotherapy and radiation therapy, but, responded with the administration of Isotretionin (Roaccutan(R)). Therefore, for inoperable SPN of the pancreas, we consider Isotretionin therapy can be used as treatment and, in the future, new studies with more of Isotretionin treatment implemented cases should be conducted to identify their anticancer mechanism.

Pancreatic mucinous cystic tumor in Turner syndrome: How a tumor bends to a genetic disease.

INTRODUCTION: Mucinous cystic neoplasms (MCN) are uncommon tumors of the pancreatic corpus/tail occurring mostly in middle-aged women, with a variable clinico-biological behavior. On histology, MCNs concurrently show an epithelial mucosecreting component with ovarian-type stromal cells. PRESENTATION OF CASE: This report describes the first case of a pancreatic MCN with no ovarian-type stroma in a patient with Turner syndrome (TS). DISCUSSION: The mesenchymal component of MCN presumably results from the intra-pancreatic entrapment of ovarian stroma during embryogenesis. In our case, the absence of such stromal component may relate to the "dysgenetic" changes in the ovary involved in TS. CONCLUSION: The present case of primary pancreatic MCN arising in a TS-patient triggers some original speculation on the morphogenesis of pancreatic MCN, also expanding the current clinico-pathological knowledge of this extremely rare entity.