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Xu YC, Fu DL, Yang F. Unraveling the enigma: A comprehensive review of solid pseudopapillary tumor of the pancreas. World J Gastrointest Oncol 2024; 16:614-629. [PMID: 38577449 PMCID: PMC10989376 DOI: 10.4251/wjgo.v16.i3.614] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 11/09/2023] [Revised: 12/28/2023] [Accepted: 01/16/2024] [Indexed: 03/12/2024] Open
Abstract
Solid pseudopapillary tumor of the pancreas (SPTP) is a rare neoplasm predominantly observed in young females. Pathologically, CTNNB1 mutations, β-catenin nuclear accumulation, and subsequent Wnt-signaling pathway activation are the leading molecular features. Accurate preoperative diagnosis often relies on imaging techniques and endoscopic biopsies. Surgical resection remains the mainstay treatment. Risk models, such as the Fudan Prognostic Index, show promise as predictive tools for assessing the prognosis of SPTP. Establishing three types of metachronous liver metastasis can be beneficial in tailoring individualized treatment and follow-up strategies. Despite advancements, challenges persist in understanding its etiology, establishing standardized treatments for unresectable or metastatic diseases, and developing a widely recognized grading system. This comprehensive review aims to elucidate the enigma by consolidating current knowledge on the epidemiology, clinical presentation, pathology, molecular characteristics, diagnostic methods, treatment options, and prognostic factors.
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Affiliation(s)
- Ye-Cheng Xu
- Department of Pancreatic Surgery, Pancreatic Disease Institute, Huashan Hospital, Fudan University, Shanghai 200040, China
| | - De-Liang Fu
- Department of Pancreatic Surgery, Pancreatic Disease Institute, Huashan Hospital, Fudan University, Shanghai 200040, China
| | - Feng Yang
- Department of Pancreatic Surgery, Pancreatic Disease Institute, Huashan Hospital, Fudan University, Shanghai 200040, China
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Wei G, Luo Q, Fang J, Li X, Shi Y, Li Y, Sun L. The Sex Features of Patients With Solid Pseudopapillary Neoplasms of the Pancreas: A Retrospective Study. Front Oncol 2022; 12:844182. [PMID: 35252013 PMCID: PMC8894654 DOI: 10.3389/fonc.2022.844182] [Citation(s) in RCA: 8] [Impact Index Per Article: 2.7] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/27/2021] [Accepted: 01/25/2022] [Indexed: 01/17/2023] Open
Abstract
Background 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. Methods 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. Results 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. Conclusion 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.
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Affiliation(s)
- Guangmin Wei
- Department of Oncology, Mengchao Hepatobiliary Hospital of Fujian Medical University, Fuzhou, China
| | - Qiong Luo
- Department of Oncology, Mengchao Hepatobiliary Hospital of Fujian Medical University, Fuzhou, China
| | - Jiankai Fang
- Department of Oncology, Mengchao Hepatobiliary Hospital of Fujian Medical University, Fuzhou, China
| | - Xiaolou Li
- Department of Oncology, Mengchao Hepatobiliary Hospital of Fujian Medical University, Fuzhou, China
| | - Yanhong Shi
- Department of Oncology, Mengchao Hepatobiliary Hospital of Fujian Medical University, Fuzhou, China
| | - Yuqiong Li
- Department of Gastroenterology, Changhai Hospital, Second Military Medical University, Shanghai, China
- *Correspondence: Yuqiong Li, ; Liqi Sun,
| | - Liqi Sun
- Department of Gastroenterology, Changhai Hospital, Second Military Medical University, Shanghai, China
- Department of Gastroenterology, 72 Group Army Hospital, Huzhou University, Huzhou, China
- *Correspondence: Yuqiong Li, ; Liqi Sun,
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Abudalou M, Vega EA, Dhingra R, Holzwanger E, Krishnan S, Kondratiev S, Niakosari A, Conrad C, Stallwood CG. Solid pseudopapillary neoplasm-diagnostic approach and post-surgical follow up: Three case reports and review of literature. World J Clin Cases 2021; 9:1682-1695. [PMID: 33728313 PMCID: PMC7942041 DOI: 10.12998/wjcc.v9.i7.1682] [Citation(s) in RCA: 6] [Impact Index Per Article: 1.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 10/07/2020] [Revised: 11/23/2020] [Accepted: 01/23/2021] [Indexed: 02/06/2023] Open
Abstract
BACKGROUND Solid pseudopapillary neoplasm (SPN) is a rare tumor that was first described by Frantz in 1959. Although this tumor is benign, some may have malignant potential that can be predicted based on demographics, imaging characteristics, and pathologic evaluation. This case series presents 3 SPN cases with discussion on gender differences, preoperative predictors of malignancy, and a suggested algorithm for diagnostic approach as well as post-surgical follow up.
CASE SUMMARY Three adult patients in a tertiary hospital found to have SPN, one elderly male and two young females. Each of the cases presented with abdominal pain and were discovered incidentally. Two cases underwent endoscopic ultrasound with fine needle aspiration and biopsy to assess tumor markers and immuno-histochemical staining (which were consistent with SPN before undergoing surgery), and one case underwent surgery directly after imaging. The average tumor size was 5 cm. Diagnosis was confirmed by histology. Two patients had post-surgical complications requiring intervention.
CONCLUSION Demographic and imaging characteristics can be sufficient to establish diagnosis for SPN, while malignant cases require pre-operative evaluation with endoscopic ultrasound fine needle aspiration/fine needle biopsy.
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Affiliation(s)
- Mohammad Abudalou
- Department of Internal Medicine, St. Elizabeth Medical Center, Brighton, MA 02135, United States
| | - Eduardo A Vega
- Department of General Surgery, St. Elizabeth Medical Center, Brighton, MA 02135, United States
| | - Rohit Dhingra
- Department of Gastroenterology, Tufts Medical Center, Boston, MA 02111, United States
| | - Erik Holzwanger
- Department of Gastroenterology, Tufts Medical Center, Boston, MA 02111, United States
| | - Sandeep Krishnan
- Department of Gastroenterology, St. Elizabeth Medical Center, Brighton, MA 02135, United States
| | - Svetlana Kondratiev
- Department of Pathology, St. Elizabeth Medical Center, Brighton, MA 02135, United States
| | - Ali Niakosari
- Department of Radiology, St. Elizabeth Medical Center, Brighton, MA 02135, United States
| | - Claudius Conrad
- Department of General Surgery, St. Elizabeth Medical Center, Brighton, MA 02135, United States
| | - Christopher G Stallwood
- Department of Gastroenterology, St. Elizabeth Medical Center, Brighton, MA 02135, United States
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Rodriguez-Matta E, Hemmerich A, Starr J, Mody K, Severson EA, Colon-Otero G. Molecular genetic changes in solid pseudopapillary neoplasms (SPN) of the pancreas. Acta Oncol 2020; 59:1024-1027. [PMID: 32672484 PMCID: PMC8081262 DOI: 10.1080/0284186x.2020.1792549] [Citation(s) in RCA: 10] [Impact Index Per Article: 2.0] [Reference Citation Analysis] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/23/2020] [Accepted: 07/02/2020] [Indexed: 10/23/2022]
Affiliation(s)
- Elisa Rodriguez-Matta
- MS-2, Mayo Clinic CCATS Research Scholar, University of Puerto Rico School of Medicine, San Juan, Puerto Rico
| | | | - Jason Starr
- Division of Hematology Oncology, Mayo Clinic, Jacksonville, FL, USA
| | - Kabir Mody
- Division of Hematology Oncology, Mayo Clinic, Jacksonville, FL, USA
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A Review of Clinicopathological Characteristics and Treatment of Solid Pseudopapillary Tumor of the Pancreas with 2450 Cases in Chinese Population. BIOMED RESEARCH INTERNATIONAL 2020; 2020:2829647. [PMID: 32685461 PMCID: PMC7352122 DOI: 10.1155/2020/2829647] [Citation(s) in RCA: 24] [Impact Index Per Article: 4.8] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Subscribe] [Scholar Register] [Received: 03/30/2020] [Accepted: 06/20/2020] [Indexed: 02/07/2023]
Abstract
Background Solid pseudopapillary tumor of the pancreas (SPTP) has been reported as a rare disease with low malignant potential. The aim of this study was to summarize experiences of the diagnosis and treatment for the patients reported in the Chinese population. Method 2450 SPTP cases reported in English and Chinese literature before Jan 2020 were for our review and analysis retrospectively. Result There are 389 male cases and 2061 female cases, and the ratio of male/female was 1 : 5.3. The average age was 29.3 years. The main clinical symptoms were upper abdominal pain and bloating discomfort in 51.6% of the cases and epigastric mass. 38.6% of the tumor was located at the head of the pancreas and 55.4% at the body and tail of the pancreas. The most frequent operative styles were tumor enucleation (38.4%). Pathology showed that the average diameter of the tumor was 8.2 cm and 12.3% of SPTP was malignant. 98.3% of cases had favorable survival. Conclusions SPTP is a rare indolent tumor occurring mainly in young women, and the main clinical performances are abdominal mass and abdominal pain; most tumors are distributed at the head and the tail of the pancreas; the prognosis after complete resection is excellent.
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Wu J, Mao Y, Jiang Y, Song Y, Yu P, Sun S, Li S. Sex differences in solid pseudopapillary neoplasm of the pancreas: A population-based study. Cancer Med 2020; 9:6030-6041. [PMID: 32578384 PMCID: PMC7433837 DOI: 10.1002/cam4.3180] [Citation(s) in RCA: 27] [Impact Index Per Article: 5.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/18/2020] [Revised: 04/24/2020] [Accepted: 05/06/2020] [Indexed: 12/14/2022] Open
Abstract
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.
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Affiliation(s)
- Jiali Wu
- Department of Pancreatobiliary Surgery, Sun Yat-Sen University Cancer Center, State Key Laboratory of Oncology in South China, Collaborative Innovation Center for Cancer Medicine, Guangzhou, China
| | - Yize Mao
- Department of Pancreatobiliary Surgery, Sun Yat-Sen University Cancer Center, State Key Laboratory of Oncology in South China, Collaborative Innovation Center for Cancer Medicine, Guangzhou, China
| | - Yiquan Jiang
- Department of Minimally Invasive Intervention, Sun Yat-Sen University Cancer Center, State Key Laboratory of Oncology in South China, Collaborative Innovation Center for Cancer Medicine, Guangzhou, China
| | - Yunda Song
- Department of Pancreatobiliary Surgery, Sun Yat-Sen University Cancer Center, State Key Laboratory of Oncology in South China, Collaborative Innovation Center for Cancer Medicine, Guangzhou, China
| | - Ping Yu
- Department of Anesthesiology, Sun Yat-Sen University Cancer Center, State Key Laboratory of Oncology in South China, Collaborative Innovation Center for Cancer Medicine, Guangzhou, China
| | - Shuxin Sun
- Department of Pancreatobiliary Surgery, Sun Yat-Sen University Cancer Center, State Key Laboratory of Oncology in South China, Collaborative Innovation Center for Cancer Medicine, Guangzhou, China
| | - Shengping Li
- Department of Pancreatobiliary Surgery, Sun Yat-Sen University Cancer Center, State Key Laboratory of Oncology in South China, Collaborative Innovation Center for Cancer Medicine, Guangzhou, China
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Shi S, Zhou Y, Hu C. Clinical manifestations and multi-slice computed tomography characteristics of solid pseudopapillary neoplasms of the pancreas between males and females. BMC Med Imaging 2019; 19:87. [PMID: 31718581 PMCID: PMC6852907 DOI: 10.1186/s12880-019-0390-9] [Citation(s) in RCA: 8] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/08/2019] [Accepted: 10/23/2019] [Indexed: 02/07/2023] Open
Abstract
Background Solid-pseudo papillary neoplasms of pancreas (SPNP) are rare in men and are often misdiagnosed. This study aimed to analyze the clinical and multi-slice computer tomography (MSCT) features of patients with SPNP, and examine the differences between males and females. Methods In this retrospective cohort study, the clinical and imaging data of 29 patients with histolopathologically confirmed SPNP (seven males and 22 females) that underwent radical resection, and underwent preoperative MSCT at the First People’s Hospital of Lianyungang between August 2010 and December 2018 were collected. All MSCT images were reviewed by two radiologists; disagreements were ruled by a third one. Results The median age of the 29 patients with SPNP was 30 (range, 12–70) years. The male patients were older than the female patients [median, 56 (28–66) vs. 29 (12–70), P = 0.012]. The median tumor size was 3.9 (range, 2.0–6.4) cm in the male SPNP patients, which was significantly lower than the 7.0 (range, 4.6–14.6) cm in the female patients (P < 0.001). The calcification rate of the SPNP was significantly higher in male than in female patients (P = 0.013). The percentage of solid tumor was higher in males than in females (P = 0.036). Capsule, bleeding, and enhancement in the arterial and venous phases were not significantly different between the male and female patients (all P > 0.05). Conclusion The imaging features of male SPNP are distinct from those of female patients. In males with pancreatic lesions, MSCT generally shows relatively small lesions with higher percentages of solid components and calcification, with typical enhancement suggesting SPNP.
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Affiliation(s)
- Shuguang Shi
- Medical Imaging Center, the First Affiliated Hospital of Soochow University, No.188 Shizi Street, Suzhou, 215006, Jiangsu, China.,Department of Radiology, First people's Hospital of Lianyungang, Lianyungang, China
| | - Ying Zhou
- Department of Radiology, First people's Hospital of Lianyungang, Lianyungang, China
| | - Chunhong Hu
- Medical Imaging Center, the First Affiliated Hospital of Soochow University, No.188 Shizi Street, Suzhou, 215006, Jiangsu, China.
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