Prognostic factors of pancreatic tumors in children and adolescents: a population study based on the surveillance, epidemiology, and end results database.

PURPOSE: Pancreatic tumors in children are uncommon, and data is scarce. The purpose of this study is to examine the prognostic factors of pediatric pancreatic tumors in a population-based cohort. METHODS: The Surveillance, Epidemiology, and End Results (SEER) database was used to identify all pediatric patients with pancreatic tumors diagnosed between 1975 and 2018. The overall survival (OS) rates were determined using a Kaplan-Meier analysis. The log-rank test was used for univariate survival analysis. Cox proportional-hazards regression was used to determine the variables related to OS. RESULTS: We identified 195 children with pancreatic tumors, with a median age at diagnosis of 16 years. Tumors were classified as neuroendocrine tumors (33.8%), solid pseudopapillary tumors (SPTs) (32.3%), pancreatoblastoma (11.3%), and others (22.6%). Of the patients, 30.3% had distant metastases, and 69.7% had surgery. Pancreatoblastomas were more common in younger children, whereas solid pseudopapillary tumors were more common in female patients. Overall 1-year, 3-year, and 5-year survival rates for all patients were 90.3%, 79.2%, and 77.7%, respectively. The Cox proportional hazard regression revealed that SEER stage and surgery were significant independent predictors of overall survival. CONCLUSIONS: Pancreatic tumors are rare in children, and overall survival is grim except for SPTs. SEER stage and surgery were determined to be the most relevant determinants of OS in our study.

Analysis of invasiveness and tumor-associated macrophages infiltration in solid pseudopapillary tumors of pancreas.

BACKGROUND: Solid pseudopapillary tumor (SPT) is a rare pancreatic tumor. Considering its malignant behaviors, SPT has been classified as a low-grade malignant tumor. Indeed, only 9.2% of all SPT patients are initially diagnosed as malignant with invasion or metastasis. Thus, one of the challenges in managing SPT patients is predicting malignant behavior. AIM: To investigate the malignant behavior and tumor-associated macrophage (TAM) infiltration between different histopathologic features of SPT patients. METHODS: Twenty-five formalin-fixed paraffin-embedded tissue samples from 22 patients pathologically diagnosed with an SPT between 2009 and 2019 at West China Hospital were included in this retrospective study. Integrity of the capsule and growth pattern of the tumor cells was assessed microscopically in hematoxylin-eosin (HE)-stained sections. Based on the histopathological features, the SPT patients were divided into two groups: capsule or invasion. Clinical features, malignant behavior, and TAM infiltration were compared between the two groups. RESULTS: Among the 22 SPT patients, 11 were identified for each group, having either a capsule or invasion histopathologic feature. Malignant behavior was more frequent in the invasion group, including 2 patients who had peripheral organ invasion, 3 with liver metastasis, and 1 with both lymph node and spleen metastases (P= 0.045). Ki-67 index of more than 3% was also more frequent in the invasion group (P = 0.045). Immunohistochemical analysis showed that the invasion group had a significant increase of CD68-positive TAMs in intratumor and peritumor sites in comparison with the capsule group (all P < 0.0001). Similarly, CD163-positive M2-like macrophages were also markedly increased in the intratumor and peritumor sites in the invasion group (all P < 0.0001). At the liver metastasis site, both intratumor and peritumor tissues showed relatively high-level CD68-positive TAMs and CD163-positive M2-like macrophages infiltration. However, the differences between the intratumor, peritumor and normal hepatic tissues did not reach statistical significance (all P > 0.05). CONCLUSION: SPT patients with invasion evident under microscope were more likely to exhibit malignant behavior and TAM infiltration, especially M2-like macrophages. This finding can help in future investigations of the underlying mechanism of TAM-mediated SPT malignant behavior.

Risk Factors of the Recurrences of Pancreatic Solid Pseudopapillary Tumors: A Systematic Review and Meta-analysis.

Background: Pancreatic solid pseudopapillary tumors (SPTs) are rare neoplasms with low-grade malignancy. The main treatment for them is surgical resection. However, some SPTs relapse after resection. The risk factors associated with the recurrences of resected SPTs remain controversial to date. We performed a systematic review and meta-analysis to identify the risk factors of the recurrences of pancreatic SPTs. Materials and Methods: We searched PubMed, EMBASE, and the Cochrane Library from their inception to December 2017. Studies that focused on the risk factors of postoperative relapses of pancreatic SPTs were enrolled. Combined ORs with 95% CIs were calculated to evaluate the effects of relevant factors investigated in eligible studies. Heterogeneity among combined results was assessed by Cochran's Q test and by the degree of inconsistency (I2). Statistical analyses were performed by Review Manager (version 5.3) using random effects models. Results: We included 10 studies, which enrolled 1091 patients. The pooled results suggested that patients with larger tumors (diameter > 5cm), lymphovascular invasion, lymph node metastasis, synchronous metastasis and positive margin were prone to suffer from the recurrences of SPTs. In addition, some factors like gender, location of tumors, perineural invasion, calcification and capsular invasion did not show any correlation with the relapses of resected SPTs. Conclusion: Factors including a larger tumor size (diameter > 5cm), lymphovascular invasion, lymph node metastasis, synchronous metastasis and positive margin may increase the risk of recurrences of resected pancreatic SPTs. All SPTs should be excised and patients with high-risk features should undergo a long-term follow-up.

Solid pseudopapillary and malignant pancreatic tumors in childhood: A systematic review and evidence quality assessment.

A systematic review of 32 studies reporting on 489 children with pancreatic tumors was performed. The most prevalent histologic subtype was solid pseudopapillary tumor (SPT) (61.3%). Pancreaticoduodenectomy was the most commonly performed operation (48%). Neoadjuvant chemo/radiotherapy was utilized in 3.9, 36.2, 25, and 27.8% of patients with SPTs, pancreatoblastomas (PBLs), neuroendocrine tumors, and exocrine carcinomas, respectively. Adjuvant chemotherapy (75.6%) and radiation (34%) were most commonly utilized in PBLs. All-cause mortality was highest in exocrine carcinomas (50%). Overall, 98.8% of patients with SPTs survived. PBL exhibited the highest recurrence rate (14.7%) within a mean of 23.5 months.

A population-based analysis of a rare oncologic entity: Malignant pancreatic tumors in children.

PURPOSE: To examine the clinicopathological characteristics and prognosis of pediatric patients with malignant pancreatic tumors in a population-based cohort. METHODS: The Surveillance, Epidemiology, and End Results (SEER) database was utilized to identify all pediatric patients with malignant pancreatic tumors, diagnosed between 1973 and 2013. Kaplan-Meier analysis was performed to determine median and five-year overall survival (OS) rates. Univariate survival analysis was executed using the log-rank test. Cox proportional hazards model was used to identify variables independently associated with mortality. RESULTS: A total of 114 patients with pancreatic malignancies were identified. Median patient age was 16years and the majority of patients were white (64%) females (61.4%). The most prevalent histologic subtype was neuroendocrine tumors (35.1%), whereas pancreatoblastoma was more common during the first decade of life (P<0.001). Distant metastases were noted in 41.7% of the patients, while 33.3% and 25% had localized and regional disease respectively. Five-year OS rates were 77%, 66.4% and 64.8% for patients with pancreatoblastoma, neuroendocrine and epithelial tumors respectively. No death was observed in the solid pseudopapillary tumor group. Only history of having cancer-directed surgery (CDS) was significantly associated with lower overall mortality (HR: 5.1, 95% CI: 2.1, 12.4). CONCLUSION: Pancreatic malignancies are rare in children. Their prognosis is variable and only CDS was independently associated with superior survival. EVIDENCE RATING/CLASSIFICATION: Prognosis study, Level II.

Pancreatic Calcifications and Calcified Pancreatic Masses: Pattern Recognition Approach on CT.

OBJECTIVE: The purpose of this article is to review a spectrum of calcified pancreatic masses and propose an algorithm for diagnostic radiologic evaluation. CONCLUSION: Pancreatic calcifications are being detected more frequently because of the widespread use of imaging, particularly CT. Pancreatic calcifications are most commonly associated with chronic pancreatitis related to alcohol abuse. Several other pathologic entities, however, can cause pancreatic calcifications. Familiarity with these entities and their CT appearance is helpful in making an accurate diagnosis.

Solid pseudopapillary tumors of the pancreas: Specific pathological features predict the likelihood of postoperative recurrence.

BACKGROUND: Since their introduction in the WHO classification, the incidence of solid pseudopapillary tumors (SPTs) of the pancreas has progressively increased, mainly because of the widespread use of cross-sectional imaging. Few recent studies have analyzed the biological behavior of SPTs, but reliable data on long-term follow-up are needed. METHODS: Retrospective analysis of two Institutions with high caseload, The Department of General Surgery-Pancreas Institute, University of Verona Hospital Trust and the Department of General Surgery, Massachusetts General Hospital, Harvard Medical School, was carried out. Data from 131 consecutive resections for SPT performed during the last three decades were collected and analyzed. RESULTS: The majority of patients were female (86.3%) with a median age of 33 (7-68) years. The prevalent location was the pancreatic tail (33.5%). Applying the WHO criteria, 16 (12.2%) SPTs were considered malignant due to the presence of at least pancreatic parenchyma (9.9%), perineural (4.6%), and/or angiovascular invasion (2.3%). After a median of 62 months after surgery, only two patients had a recurrence (1.5%). Both of them fulfilled the WHO criteria for malignant SPT (vs. 10.7% of those who did not recur, P = 0.01), had an infiltrative growth pattern (vs. 10.8%, P = 0.01), pancreatic parenchyma invasion (vs. 9.7%, P = 0.01) and capsular invasion (vs. 4.9%, P = 0.004). CONCLUSION: Overall, SPTs are associated with excellent survival results after surgical resection. Disease recurrence is extremely rare, and might occur if the primary tumor presents with either pancreatic parenchyma or capsule invasion. J. Surg. Oncol. 2016;114:597-601. © 2016 Wiley Periodicals, Inc.

Differential Diagnosis of Cystic Pancreatic Lesions Including the Usefulness of Biomarkers.

BACKGROUND: Cystic pancreatic lesions are more and more often found. Malignant risk ranges from nil to more than 60%. A precise diagnosis is required to adapt surveillance or therapeutic strategy. METHODS: We tried to identify the most difficult differential diagnoses encountered in a tertiary center of pancreatology and to guide the reader as how to reach the correct strategy and diagnosis in these situations. RESULTS: We identified eight clinically difficult situations: i) chronic pancreatitis versus intraductal papillary mucinous neoplasms, ii) serous versus mucinous cystic neoplasms, iii) serous cystic neoplasms versus branch-duct intraductal papillary mucinous neoplasms, iv) intraductal papillary mucinous neoplasms versus acinar cell cystadenoma, v) (pseudo-) solid serous cystic neoplasm versus neuroendocrine tumor, vi) pancreatic neuroendocrine tumors versus solid pseudopapillary tumors, vii) cystic forms of a solid tumor, and viii) rare pancreatic or peripancreatic cystic lesions. The work-up should rely on computed tomography scan, pancreatic magnetic resonance imaging, and, only if necessary, endoscopic ultrasound with or without fine needle aspiration. CONCLUSION: An expert analysis of imaging data allows a precise diagnosis in most of the cases. Pancreatic resection should no longer be performed in case of diagnostic doubt.

Extrapancreatic solid pseudopapillary neoplasm: Report of a case of primary retroperitoneal origin and review of the literature.

Solid pseudopapillary tumors (SPTs) occurring as primary tumors outside the pancreas are exceedingly rare. The present study reports such a case occurring as a non-functional adrenal tumor in a 22-year-old female. The tumor was completely removed from the retroperitoneum by laparoscopic surgery. A well-defined, encapsulated tumor measuring 6×6×5 cm was histologically characterized by a combination of the solid and pseudopapillary growth patterns of tumor cells with eosinophilic cytoplasm. Ectopic pancreatic tissue was also found histologically within the resected tumor. On immunostaining, the tumor was positive for progesterone receptor, CD56, cytokeratin and CD10. The morphological and immunohistochemical features were compatible with those of SPT. To the best of our knowledge, this is the first case report of extrapancreatic SPT with evidence of a pre-existing ectopic pancreas in the retroperitoneum. A review of the published English literature uncovered 12 cases of extrapancreatic SPTs, and revealed that extrapancreatic SPTs are likely to have a favorable clinical course and a clinical profile similar to their pancreatic counterparts.

Solid pseudopapillary tumors of the pancreas: A review of 1180 patients reported in chinese literature / 中华胰腺病杂志

Objective To summarize and analyze data of solid pseudopapillary tumor of pancreas (SPTP) in China,and investigate its epidemiology,clinical features,diagnosis and treatment.Methods Retrieval of Chinese Medical Current Contents and China Biology Medicine disc by the key words of solid pseudopapillary tumor of the pancreas,papillary cystic tumor of the pancreas,pancreatic papillary epithelial tumor,cystic solid tumor of pancreas and Frantz tumor were performed,and relevant literatures were included.Results A total of 1180 SPTP patients from 117 articles were involved.There were 1054 women and 126 men and the ratio of male to female was 1:8.37.The average age was 29 years old (range 9 ～83 years).Detailed clinical information was available for 1172 cases,and the main clinical manifestations included abdominal discomfort (n=526,44.88％),medical check-up (n=464,39.59％),abdominal mass (n=131,11.18％).Laboratory and imaging tests were non-specific.The tumors size was 1.3～ 30 cm with a mean value of 7.84 cm.Four handreds and seven (36.8％) cases were located in pancreatic head,96 (8.7％)were in pancreas neck and 587 (53.1％) were in the body and tail of pancreas.Eleven handreds and sixteen patients received treatment,and the resection rate was 99.2％ (n =1107).Pathological examination showed that 628(57.0％) cases were benign and 306 (27.8％) were presented as malignant behavior,mainly as infiltrative growth and invasion of the surrounding organs,vessels.Nine handreds and seventy-seven cases were followed up (ranging from 1 month to 13 years),and re-occurrence or metastasis were detected in 42 cases (4.3％) and 24 patients died.Conclusions Solid pseudopapillary tumor of pancreas is a rare pancreatic tumor with low-grade malignant potential,and part of this tumor may present as malignant behavior and it primarily affects young females.No characteristics in clinical manifestations,laboratory and imaging tests are found.Pathological examination can confirm the diagnosis.Surgical resection is the therapy of choice and the prognosis is good.

Diagnosis and surgical treatment of solid pseudopapillary tumor of the pancreas / 中华普通外科杂志

Objective To evaluate the diagnosis and therapy of solid pseudopapillary tumor of the pancreas (SPTP).Methods From January 2006 to October 2011,the clinicopathological data of 24 SPTP patients diagnosed and surgically treated at Tianjin Medical University Cancer Hospital were analyzed retrospectively.Results There were 22 women and 2 men,the median age was 27.5 years old,with a range from 12 to 51 years.The most common tumor localization were at the head of the pancreas ( 11 patients,45.7％ ),followed by the body (5 patients,20.8％ ),the tail (4 patients,16.7％ ),the body and the tail (3 patients,12.5％ ) and the neck (one patient,4.2％ ).Clinical symptoms were nonspecific and the preoperative diagnosis of SPTP depended on imaging study such as ultrasonography,CT and MR.All patients were treated surgically and the correct diagnosis was made according to pathology after surgery.In a follow-up ranging from 2 to 55 months( median 27.2 months),23 cases were alive with no evidence of disease recurrence,one patient was alive with tumor recurrence.Conclusions SPTP was a less common neoplasm with low-grade malignancy,occurring mainly in young women.Complete tumor resection results in satisfactory prognosis.

Meta analysis of solid pseudopapillary tumors of the pancreas / 中华胰腺病杂志

Objective To summarize the experience of diagnosis, treatment and prognosis of solid pseudopapillary tumors of the pancreas (SPT) in China. Methods The Chinese literature from January 1992 to April 2009 in Chinese Journal Full-text Database was systematically searched and a total of 439 cases of SPT from 42 reports were found. Clinical data of these cases were retrospectively analyzed. Results Among 439 was 28 years old (range 8 ～76 yrs). The clinical symptoms were recorded in 377 cases, including abdominal pain (35. 3%), mass (31. 3%), discomfort (7. 7%). 101 patients (26. 8%) were completely asymptomatic, and the lesions were detected during routine check-up. All of the patients underwent surgical resection. The preoperative misdiagnosis rate was 65.4% ( 161/246 ). The rate of curative resection was 97.3% (427/439). Mean diameter of the tumor was 7.8 cm ( range 1.5 ～ 25.0 cm). Among 394 patients with information on metastases or invasions, 80 patients(20.3% ) were positive. In all patients, 418(95.2% )were followed up and the mean follow-up period was 34 months ( range 1 month to 25 years ). During the follow-up period, 403 ( 96. 4% ) patients were alive with no evidence of recurrence or metastases, local recurrence developed in 4 patients, the liver metastases developed in 6 patients, 4 patients died from the disease. Conclusions SPT is a rare and potentially low-grade malignant tumor, and predominantly affect young women. The correct diagnosis depends on the histopathological examination. Radical surgical resection is the only effective treatment for SPT, which usually has an excellent prognosis.

Solid pseudopapillary neoplasm of the pancreas / 中华普通外科杂志

Objective To investigate the clinical features,diagnosis and prognosis of solid pseudopapillary tumor of the pancreas(SPTP). Method The clinical data of SPTP cases that underwent surgical resection with a definitive histological diagnosis in Zhong Shan Hospital from 1999 to 2007 were retrospectively analyzed.Result There were 42 SPTP cases undergoing surgery in this period,including 37 females and 5 males:mean age was 37.6 years.Tumors located in the pancreatic head in 18 cases and in the body or tail in 24 cases.The most common symptom was abdominal discomfort(n=20),palpable mass (n=8).Preoperative diagnostic accuracy of MRI and CT were 93%and 31.8%,respectively.Ten cases underwent pancreaticoduodenectomy,20 cases received distal pancreatectomy,3 cases did spleen-preserving distal pancreatectomy.Tumors were simply enucleated in 8 cases.Average diameter of the tumors was 6.1 cm,34 lesions were solid,6 were solid-cystic,2 were cystic.The overall perioperative morbidity was 38.1%,10 cases developed postoperative pancreatic fistula.The mean follow-up time was 38.6 months.Except three cases that were lost to follow-up,all the other cases were alive.Four cases(2 cases had had tumor enucleation,2 had had distal pancreatectomy)developed hepatic metastases on 70、110、41、3 months after first surgery,respectively.A transarterial chemoembolization was employed in three cases,while right hemihepatectomy was performed in one case. Conclusion SPTP is an indolent tumor with low-grade biological aggressiveness.Patients had a favorable outcome after surgical treatment,but enucleation should be avoided.Patients with liver metastasis may benefit from chemoembolization and liver resection.

Clinical analysis of solid-pseudopapillary tumor of the pancreas in 27 cases / 中华普通外科杂志

Objective To summarize the experience in the diagnosis and treatment for solidpseudopapillary tumors of the pancreas (SPTP). Method In this study, 27 cases of SPTP from September 2000 to June 2007 were retrospectively analyzed. Results The median age of these 27 cases was 26 years. There were 26 females and 1 male. Epigastric pain was complained in 16 cases, abdominal aponia mass in 11 cases. Tumour marker level was within normal range. Ultrasonic manifestation usually shows cystic and solid lesion without blood signal. Lesions were typical with high blood supply in enhanced CT scan, cystic and solid component confounding in it. MRI revealed low-middle signal in T1 and middle-high signal in T2. Pancreaticoduodenectomy was performed in 9 cases, pancreatic local excision and pancreaticojejunostomy were performed in 4 cases, pancreatic local excision was performed in 6 cases, Distal pancreatectomy and spleen resection were performed in 8 cases. Post-operative pathology shows local lesion in 12 cases, invading the pancreatic parenchyma in 14 cases and intravascular tumor thrombi and lymphnode metastasis in 1 case. All cases were followed up with an average period of 26.1 months. There was no recurrence nor metastasis. Conclusion Solid-pseudopapillary tumor of the pancreas has a low malignant potential, affecting primarily young women. It has no typical clinical appearance. Enhanced CT scanning has the relative characteristic imaging. Complete resection usually results in long-term survival.