• cancer genomics
• pancreatic cancer
• oncogenes

• CTNNB1 gene mutation
• Immunohistochemical marker
• LEF1
• WNT signaling pathway tumors
• Β-catenin

• Humans
• Lymphoid Enhancer-Binding Factor 1/genetics
• Lymphoid Enhancer-Binding Factor 1/metabolism
• Lymphoid Enhancer-Binding Factor 1/analysis
• beta Catenin/genetics
• beta Catenin/metabolism
• Female
• Mutation
• Male
• Biomarkers, Tumor/genetics
• Biomarkers, Tumor/metabolism
• Middle Aged
• Wnt Signaling Pathway/genetics
• Adult
• Prognosis
• Young Adult
• Desmoid Tumors/genetics
• Desmoid Tumors/diagnosis
• Desmoid Tumors/metabolism
• Desmoid Tumors/pathology
• Aged
• Adolescent
• Adenoma/genetics
• Adenoma/diagnosis
• Adenoma/metabolism
• Adenoma/pathology
• Pancreatic Neoplasms/genetics
• Pancreatic Neoplasms/diagnosis
• Pancreatic Neoplasms/pathology
• Pancreatic Neoplasms/metabolism
• Follow-Up Studies
• Child

• Beta-catenin
• LEF-1 IHC
• Pancreatic cancer
• Pancreatic neoplasm
• Solid pseudopapillary neoplasm

• Humans
• Pancreatic Neoplasms/pathology
• Pancreatic Neoplasms/diagnosis
• Pancreatic Neoplasms/metabolism
• Lymphoid Enhancer-Binding Factor 1/analysis
• Female
• Male
• Adult
• Immunohistochemistry
• Biomarkers, Tumor/analysis
• Middle Aged
• Young Adult
• Adolescent
• beta Catenin/analysis
• beta Catenin/metabolism
• Aged
• Child
• Carcinoma, Acinar Cell/pathology
• Carcinoma, Acinar Cell/diagnosis
• Carcinoma, Acinar Cell/metabolism
• Carcinoma, Papillary/pathology
• Carcinoma, Papillary/diagnosis
• Carcinoma, Papillary/metabolism
• Carcinoma, Pancreatic Ductal/diagnosis
• Carcinoma, Pancreatic Ductal/pathology
• Carcinoma, Pancreatic Ductal/metabolism

• diagnosis
• hepatic metastases
• prognosis
• solid pseudopapillary neoplasm
• treatment

• Childhood
• malignant solid pseudopapillary neoplasm of the pancreas
• pediatric
• solid pseudopapillary neoplasm of the pancreas

• Identification and assessment
• Malignant potential
• Management
• Prognosis
• Solid pseudopapillary neoplasm

• 82203158 National Natural Science Foundation of China
• 81972258 National Natural Science Foundation of China
• 2021-1-I2M-002 CAMS Innovation Fund for Medical Sciences
• 2022-PUMCH-D-001 National High Level Hospital Clinical Research Funding
• National Multidisciplinary Cooperative Diagnosis and Treatment Capacity Building Project for Major Diseases

• Endoscopic ultrasound
• Liver metastasis
• Pancreas
• Prognostic prediction
• Recurrence
• Solid pseudopapillary tumor
• Surgery
• β-catenin

• Adult
• Female
• Humans
• Male
• Abdominal Pain/etiology
• Pancreas/pathology
• Pancreatic Neoplasms/diagnosis
• Pancreatic Neoplasms/surgery
• Pancreatic Neoplasms/metabolism
• Retrospective Studies

Solid pseudopapillary neoplasm (SPN) is a rare tumor with low malignant potential, which typically occurs in the pancreas. Extrapancreatic SPN is also extremely rare worldwide.

We report a case of a 70-year-old woman hospitalized with abdominal pain and bloating. The patient did not have any underlying diseases, such as diabetes, coronary heart disease, or hypertension. More than 30 years ago, the patient underwent surgery for “ectopic pregnancy”. The patient had no family history of hereditary disease, nor did any immediate family members have a history of cancer. Laboratory tests showed that her hemoglobin and albumin levels were low and she had a high level of cancer antigen 125 (CA125). Enhanced computed tomography (CT) showed a large tumor in the abdomen and pelvis. The patient subsequently underwent surgery, and it was found that the tumor was attached to the terminal ileum. Pathological findings suggested that the tumor was an extrapancreatic SPN, with an ectopic pancreas found in the tumor tissue. The patient did not receive chemotherapy or radiotherapy after surgery. After 13 months of follow-up, the patient was admitted again with abdominal pain. CT showed tumor recurrence with extensive systemic metastases. The patient and her family refused reoperation and biopsy, and the patient was discharged after the abdominal pain and anemia resolved.

We report a rare case of extrapancreatic SPN of ileal origin, which could be the first report worldwide. It had aggressive biological features, with recurrence and metastasis 13 months after surgery. For extrapancreatic SPN, the risk of recurrence should be assessed, and for tumors suspected of malignant behavior, a longer follow-up after discharge may be needed. Although SPN generally has a good prognosis after surgery, there is no consensus on whether postoperative chemotherapy and other treatments are needed for patients with high recurrence risk.

Solid pseudopapillary neoplasm (SPN) of the pancreas is a rare neoplasm that mainly affects young women.

To evaluate the impact of parenchyma-preserving surgical methods (PPMs, including enucleation and central pancreatectomy) in the treatment of SPN patients.

From 2013 to 2019, patients who underwent pancreatectomy for SPNs were retrospectively reviewed. The baseline characteristics, intraoperative index, pathological outcomes, short-term complications and long-term follow-up data were compared between the PPM group and the conventional method (CM) group.

In total, 166 patients were included in this study. Of them, 33 patients (19.9%) underwent PPM. Most of the tumors (104/166, 62.7%) were found accidentally. Comparing the parameters between groups, the hospital stay d (12.35 vs 13.5 d, P = 0.49), total expense (44213 vs 54084 yuan, P = 0.21), operation duration (135 vs 120 min, P = 0.71), and intraoperative bleeding volume (200 vs 100 mL, P = 0.49) did not differ between groups. Regarding pathological outcomes, tumor size (45 vs 32 mm, P = 0.07), Ki67 index (P = 0.53), peripheral tissue invasion (11.3% vs 9.1%, P = 0.43) and positive margin status (7.5% vs 6%, P = 0.28) also did not differ between groups. Moreover, PPM did not increase the risk of severe postoperative pancreatic fistula (3.8% vs 3.0%, P = 0.85) or tumor recurrence (3.0% vs 6.0%, P = 0.39). However, the number of patients who had exocrine insufficiency during follow-up was significantly lower in the PPM group (21.8% vs 3%, P = 0.024). CM was identified as an independent risk factor for pancreatic exocrine insufficiency (odds ratio = 8.195, 95% confident interval: 1.067-62.93).

PPM for SPN appears to be feasible and safe for preserving the exocrine function of the pancreas.

• Solid pseudopapillary neoplasm
• Surgical resection
• Parenchyma-preserving method
• Pancreatic exocrine insufficiency

Solid pseudopapillary neoplasms of the pancreas (SPNs) in male patients are more frequently reported. The aim of the study was to evaluate the sex features of SPN and the risk factors that predict tumor recurrence.

From 2013 to 2019, patients who were pathologically confirmed to have SPNs were retrospectively reviewed. The baseline study parameters were compared between males and females. A logistic regression model was established to identify the independent risk factors for tumor recurrence.

In total, 221 patients were included in this study. Of them, 53 patients (24.0%) were males. Male patients were older than female patients (39.1 vs 31.6 years, P=0.001), and the tumor size in male patients was smaller than that in female patients (50.38 vs 39.65 mm, P=0.038). The preoperative imaging diagnostic accuracy was significantly higher in females than in males (70.5% vs 54%, P=0.02). SPNs in male patients tended to be misdiagnosed with other malignant tumors (37.7% vs 10.7%, P<0.0001), with a more solid component observed in images (66.8% vs 24.7%, P<0.0001). For immunohistochemical staining, the expression of beta catenin was significantly lower in male patients (P=0.002), and the expression of vimentin was the opposite (P=0.01). The overall survival rate and disease-free survival were not different. Based on multivariate analysis, older age [hazard ratio (HR)= 1.094, 95% confidence interval (CI): 1.005-1.190] and KI 67 index grade III (HR=12.029, 95% CI: 2.399-60.311) were independent risk factors for tumor recurrence.

The clinical and imaging features of SPN in males were not in full accord with those in females; however, the differences did not influence prognosis.

Solid pseudopapillary neoplasm of the pancreas is a low-grade malignant tumor that predominantly affects young women in their third and fourth decade. Etiology and risk factors are unknown. Clinical symptoms are aspecific and most commonly due to mass effect. Diagnosis is made by computed tomography scan or magnetic resonance imaging and histological characterization is obtained by endoscopic ultrasound-guided fine needle biopsy. Microscopically, these lesions are composed by both solid and pseudopapillary structures with necrotic and hemorrhagic areas. Occasionally, the biological behavior is aggressive with tumor recurrence and distant metastasis. Usually, curative R0 surgical resection is the best option able to provide long term survival even in advanced disease. Unresectable disease is the main predictor of poor prognosis. Chemotherapy and radiotherapy regimens are not well standardized. However, they could be effective in reducing tumor size as neoadjuvant treatment or disease control in palliative setting. Although complete surgical resection provides a cure rate of > 95%, considering young age of the patients and morbidity associated to pancreatic surgery, further studies are needed to better investigate risk factors and responsiveness to hormones in order to allow early diagnosis and follow up strategies that could avoid unnecessary surgery in less aggressive disease.

• Pseudopapillary neoplasm
• Pancreatic tumor
• Pancreaticoduodenectomy
• Distal pancreatectomy
• Pancreas
• Surgery

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 β-catenin. Almost all solid pseudopapillary neoplasms harbour mutations in exon 3 of CTNNB1, the gene encoding β-catenin. The overall prognosis is excellent, and most patients are cured by complete surgical resection.

• Cancer of pancreas
• Pancreatic neoplasms
• Solid pseudopapillary neoplasm of the pancreas
• Non-ductal pancreatic tumours
• Pancreas