Solid Pseudopapillary Tumor Research Articles

Treatment with long term follow-up of solid pseudopapillary tumor of pancreas - A single center experience from India

Treatment with long term follow-up of solid pseudopapillary tumor of pancreas - A single center experience from India

Surgical outcomes of resected cystic neoplasms of pancreas: Experience from a tertiary care centre in India.

Cystic neoplasms of the pancreas form a rare heterogeneous group of pancreatic tumors with variable clinical and diagnostic characteristics. Its incidence has increased in recent years due to improvements in cross-sectional imaging methods and awareness amongst surgeons. This study aimed to study the demographic, clinical, imaging, and histopathologic characteristics, incidence of malignancy and outcome of surgical resection of pancreatic cystic neoplasms. Retrospective analysis of 91 patients who underwent surgical resection for cystic neoplasm of the pancreas between 2006 to 2017 at a tertiary care institute was done. There was a female preponderance in the study with a mean age of 47.2 years. Abdominal pain (46.1%) and jaundice (23.1%) were the most common symptoms. Computed tomography and endoultrasound (EUS) were the most commonly used imaging methods in the study and demonstrated good surgical correlation. Pancreaticoduodenectomy (37.1%) was the most commonly performed procedure followed by distal pancreaticosplenectomy (31.8%). Of the lesions, 9.8% were found malignant. Solid pseudopapillary epithelial neoplasm (SPEN) (37.3%) was the most common neoplasm followed by serous (21.9%), intraductal papillary mucinous neoplasm (IPMN) (15.3%) and mucinous neoplasm (14.3%). Preoperative radiological diagnostic correlation was found to be 75-100% implying the importance of imaging in cystic neoplasms of the pancreas. Morbidity and mortality in the study group were 28.5% and 2.1%, respectively. Pancreatic cystic neoplasms were mostly benign with female preponderance and presented in the younger age group with prevalence of SPEN higher than IPMN in our subcontinent. These can be reliably diagnosed on preoperative cross-sectional imaging, and surgical resection is associated with favourable outcome and acceptable morbidity.

ВОЗМОЖНОСТЬ ВЫПОЛНЕНИЯ ГАСТРО/ПАНКРЕАТОДУОДЕНАЛЬНОЙ РЕЗЕКЦИИ У ДЕТЕЙ С ОПУХОЛЯМИ ПОДЖЕЛУДОЧНОЙ ЖЕЛЕЗЫ

The article is about the experience in treating of pediatric patients with tumors of the pancreas who have undergone the gastro-pancreatoduodenal resection (GPDR) and pancreatoduodenal resection (PDR) at the Research Institute of Pediatric Oncology and Hematology named after Academician L.A. Durnov with the N.N. Blokhin Russian Cancer Research Center (Moscow, Russia). These kinds of surgical interventions are performed both with local neoplasms of the pancreatic head and with the spread of the tumor process to the duodenum, distal part of the stomach, and parapancreatic tissue. Materials and methods used: 13 GPDR, PDR surgical interventions were performed in children aged 5 to 16 y/o in 2010-2022. The results of treatment of these patients were assessed as satisfactory despite the complications that arose in some patients in the early and late postoperative periods. All patients are alive. GPDR surgical interventions were performed in 6 patients, and PDR in 7. The most frequently verified were solid pseudopapillary tumor of the pancreatic head (in 10 cases), neuroendocrine tumor (in 2 cases) and paraganglioma in a single case. Discussion and conclusions: bleeding from the pancreatic branch of the splenic artery and acute pancreatitis were noted among the early postoperative complications. Recurrent phenomenon of enzyme evasion and syndrome of excessive bacterial growth were revealed in the late postoperative period. Despite the complications arisen all patients are alive without signs of recurrence of the disease. The prognosis for this category of patients with radically performed surgical intervention is favorable.

ВОЗМОЖНОСТЬ ВЫПОЛНЕНИЯ ГАСТРО/ ПАНКРЕАТОДУОДЕНАЛЬНОЙ РЕЗЕКЦИИ У ДЕТЕЙ С ОПУХОЛЯМИ ПОДЖЕЛУДОЧНОЙ ЖЕЛЕЗЫ

The article is about the experience in treating of pediatric patients with tumors of the pancreas who have undergone the gastro-pancreatoduodenal resection (GPDR) and pancreatoduodenal resection (PDR) at the Research Institute of Pediatric Oncology and Hematology named after Academician L.A. Durnov with the N.N. Blokhin Russian Cancer Research Center (Moscow, Russia). These kinds of surgical interventions are performed both with local neoplasms of the pancreatic head and with the spread of the tumor process to the duodenum, distal part of the stomach, and parapancreatic tissue. Materials and methods used: 13 GPDR, PDR surgical interventions were performed in children aged 5 to 16 y/o in 2010-2022. The results of treatment of these patients were assessed as satisfactory despite the complications that arose in some patients in the early and late postoperative periods. All patients are alive. GPDR surgical interventions were performed in 6 patients, and PDR in 7. The most frequently verified were solid pseudopapillary tumor of the pancreatic head (in 10 cases), neuroendocrine tumor (in 2 cases) and paraganglioma in a single case. Discussion and conclusions: bleeding from the pancreatic branch of the splenic artery and acute pancreatitis were noted among the early postoperative complications. Recurrent phenomenon of enzyme evasion and syndrome of excessive bacterial growth were revealed in the late postoperative period. Despite the complications arisen all patients are alive without signs of recurrence of the disease. The prognosis for this category of patients with radically performed surgical intervention is favorable.

Potential Role for Observation in Small Solid Pseudopapillary Neoplasm (SPN).

Solid pseudopapillaryneoplasms(SPN)arerare tumorsof the pancreas, typically affecting young women.Resection is the mainstay of treatment but is associated with significant morbidity and potential mortality. Weexplore the ideathat small, localized SPN could be safely observed. This retrospective review of the Pancreas National Cancer Database from 2004 to 2018 identified SPN via histology code8452. A total of 994 SPNs were identified. Mean age was 36.8 ± 0.5 years, 84.9% (n = 844) were female, and most had a Charlson-Deyo Comorbidity Coefficient (CDCC) of 0-1 (96.6%, n = 960). Patients were most often staged clinically as cT2 (69.5%, n = 457) followed by cT3 (17.6%, n = 116), cT1 (11.2%, n = 74), and cT4 (1.7%, n = 11). Clinical lymph node and distant metastasis rates were 3.0 and 4.0%, respectively. Surgical resection was performed in 96.6% of patients (n = 960), most commonly partial pancreatectomy (44.3%) followed by pancreatoduodenectomy (31.3%) and total pancreatectomy (8.1%). In patients clinically staged as node (N0) and distant metastasis (M0) negative, occult pathologic lymph node involvement was found in 0% (n = 28) of patients with stage cT1 and 0.5% (n = 185) of patients with cT2 disease. The risk of occult nodal metastasis significantly increased to 8.9% (n = 61) for patients with cT3 disease. The risk further increased to 50% (n = 2) in patients with cT4 disease. Herein, the specificity of excluding nodal involvement clinically is 99.5% in tumors ≤ 4 cm and 100% in tumors ≤ 2 cm. Therefore, there may be a role for close observation in patients with cT1N0 lesions to mitigate morbidity from major pancreatic resection.

Solid pseudopapillary tumor of the pancreas: a "female" tumor in a male adolescent

Die solid-pseudopapilläre Neoplasie (SPN), Synonym „Frantz-Tumor“, ist eine seltene Pankreasneoplasie niedrig-malignen Potenzials. Sie weist eine deutliche Gynäkotropie auf (w : m = 9 : 1) und findet sich typischerweise bei jungen Frauen in einem Alter von 20–35 Jahren. Bei Kindern und Jugendlichen tritt die Erkrankung zwar eher selten auf, stellt aber eine der 3 wichtigsten Differenzialdiagnosen des Pankreastumors im pädiatrischen Kollektiv dar.

ASO Visual Abstract: Potential Role for Observation in Small Solid Pseudopapillary Neoplasm (SPN).

ASO Visual Abstract: Potential Role for Observation in Small Solid Pseudopapillary Neoplasm (SPN).

Study on Cytomorphology of Solid Pseudopapillary Tumor (SPT) in pancreas and its Differential Diagnosis

Study on Cytomorphology of Solid Pseudopapillary Tumor (SPT) in pancreas and its Differential Diagnosis

Laparoscopic parenchyma-sparing resections for solid pseudopapillary tumors located in the head of pancreas

BackgroundSolid pseudopapillary tumor (SPT) of the pancreas is a rare low-grade malignant tumor. Here, we aimed to determine the safety and feasibility of laparoscopic parenchyma-sparing pancreatectomy for SPT located in the pancreatic head.MethodsFrom July 2014 to February 2022, 62 patients with SPT located in the pancreatic head were operated laparoscopically in two institutions. These patients were divided into two groups according to the operative strategy: laparoscopic parenchyma-sparing pancreatectomy (27 patients, group 1) and laparoscopic pancreaticoduodenectomy (35 patients, group 2). The clinical data were retrospectively collected and analyzed in terms of demographic characteristics, perioperative variables, and long-term follow-up outcomes.ResultsThe demographic characteristics of the patients in the two groups were comparable. Compared to the patients in group 2, those in group 1 required less operative time (263.4 ± 37.2 min vs. 332.7 ± 55.6 min, p < 0.001) and experienced less blood loss (105.1 ± 36.5mL vs. 188.3 ± 150.7 mL, p < 0.001). None of the patients in group 1 had tumor recurrence or metastasis. However, 1 (2.5%) patient in group 2 showed liver metastasis.ConclusionLaparoscopic parenchyma-sparing pancreatectomy is a safe and feasible approach for SPT located in the pancreatic head, with favorable long-term functional and oncological results.

Solid Pseudopapillary Neoplasm of the Pancreas: A Single-Center Experience of a Rare Neoplasm.

Solid pseudopapillary neoplasm (SPN) of the pancreas is an extremely rare pancreatic exocrine tumor. The study aims to report our experience with the SPN of the pancreas. A retrospective analysis of the prospectively maintained database was carried out of all the cases diagnosed and treated as SPN between January 2019 and January 2023. Patient characteristics including age, gender, clinical presentation, laboratory examinations, imaging features, surgical details, and histopathological and immunohistochemistry details were analyzed. During this period, eight cases were diagnosed with SPN. All patients were female with a median age of 25.75 years (range 14-55 years). All cases presented with pain in the abdomen, and four patients had a mass per abdomen. In all the cases, contrast-enhanced computed tomography (CECT) abdomen was done for the diagnosis and had preoperative suspicion of the pseudopapillary tumor. In four cases, the tumor was located in the head region, while in four cases, the tumor was in the body and tail of the pancreas. The median size of the tumor was 12 cm (range 3.5-15 cm). Three cases underwent Whipple's procedure and one patient was unresectable. Two out of four patients with body and tail tumors underwent distal pancreatectomy with splenectomy, one underwent spleen-preserving distal pancreatectomy, and one patient underwent central pancreatectomy. SPN is a rare neoplasm that primarily affects young women. Clinicopathologic and immunohistochemical features are diagnostic. Surgical resection is generally curative with a good long-term outcome.

A rare co-occurence of Turner syndrome and acromegaly complicated by the presence of a solid pseudopapillary neoplasm of pancreas-a case report

Searchable abstracts of presentations at key conferences in endocrinology ISSN 1470-3947 (print) | ISSN 1479-6848 (online)

Solid Pseudopapillary Neoplasm of the Pancreas: Case Series of a Rare Tumor

Solid pseudopapillary neoplasm is an infrequent type of pancreatic tumor that primarily occurs in young females. This retrospective case series analyzed three cases of SPN at Civil Service Hospital from 2012-2022. Patient’s median age was 18 years, and one of the patients presented with painless lump and other two had abdominal pain and dullness at presentation. Pre-operative investigations like CT/MRI and fine-needle aspiration cytology confirmed SPN in all cases. All patients received resection treatment, and no adjuvant therapy was administered. During follow-ups lasting 4-7 years, none of the patients experienced recurrence, indicating that surgical resection is the mainstay of treatment with excellent prognosis.

Efficacy and predictive factors of endoscopic ultrasound-guided ethanol ablation in benign solid pancreatic tumors

Backgrounds and ObjectivesEndoscopic ultrasound-guided ethanol ablation (EUS-EA) has recently been introduced for the management of solid pancreatic tumors, including pancreatic neuroendocrine tumors (PNETs) and solid pseudopapillary tumors (SPTs). The study aims to evaluate the efficacy and predictive factors for response of EUS-EA in solid pancreatic tumors.MethodsBetween October 2015 and July 2021, 72 patients who underwent EUS-EA for solid pancreatic tumors were included. The study outcomes were to evaluate the efficacy of EUS-EA with complete remission (CR) and objective response, and their predictive factors.ResultsDuring follow-up, 47 patients were diagnosed with PNETs and 25 with SPTs. Eight cases reached CR and 48 reached objective response. When compared with SPTs, PNETs showed similar duration to reach CR (median not reached; p = 0.319), but shorter duration to reach objective response (PNETs: median 20.6 months, 95%CI 10.26–30.88; SPTs: median 47.7 months, 95%CI 18.14–77.20; p = 0.018). Ethanol dosage > 0.35 ml/cm3 shortened the duration to reach CR (median not reached; p = 0.026) and objective response (median 42.5 months, 95%CI 25.34–59.66 vs. 19.6 months, 95%CI 10.17–29.09; p = 0.006). CR had no significant predictive factors, but PNETs showed significant predictive factors for objective response (HR 3.34, 95%CI 1.07–10.43; p = 0.038). Twenty-seven patients experienced adverse events, and there were two severe cases.ConclusionEUS-EA for pancreatic solid lesions seems feasible as a local treatment for patients who refuse or are unfit for surgery. Additionally, PNETs seem to be the better candidate for EUS-EA.

Role of endoscopic ultrasound in the characterization of solid pseudopapillary neoplasm of the pancreas.

Solid pseudopapillary neoplasm (SPN) is an uncommon pathology of the pancreas with unpredictable malignant potential. Endoscopic ultrasound (EUS) assessment plays a vital role in lesion characterization and confirmation of the tissue diagnosis. However, there is a paucity of data regarding the imaging assessment of these lesions. To determine the characteristic EUS features of SPN and define its role in preoperative assessment. This was an international, multicenter, retrospective, observational study of prospective cohorts from 7 large hepatopancreaticobiliary centers. All cases with postoperative histology of SPN were included in the study. Data collected included clinical, biochemical, histological and EUS characteristics. One hundred and six patients with the diagnosis of SPN were included. The mean age was 26 years (range 9 to 70 years), with female predominance (89.6%). The most frequent clinical presentation was abdominal pain (80/106; 75.5%). The mean diameter of the lesion was 53.7 mm (range 15 to 130 mm), with the slight predominant location in the head of the pancreas (44/106; 41.5%). The majority of lesions presented with solid imaging features (59/106; 55.7%) although 33.0% (35/106) had mixed solid/cystic characteristics and 11.3% (12/106) had cystic morphology. Calcification was observed in only 4 (3.8%) cases. Main pancreatic duct dilation was uncommon, evident in only 2 cases (1.9%), whilst common bile duct dilation was observed in 5 (11.3%) cases. One patient demonstrated a double duct sign at presentation. Elastography and Doppler evaluation demonstrated inconsistent appearances with no emergence of a predictable pattern. EUS guided biopsy was performed using three different types of needles: Fine needle aspiration (67/106; 63.2%), fine needle biopsy (37/106; 34.9%), and Sonar Trucut (2/106; 1.9%). The diagnosis was conclusive in 103 (97.2%) cases. Ninety-seven patients were treated surgically (91.5%) and the post-surgical SPN diagnosis was confirmed in all cases. During the 2-year follow-up period, no recurrence was observed. SPN presented primarily as a solid lesion on endosonographic assessment. The lesion tended to be located in the head or body of the pancreas. There was no consistent characteristic pattern apparent on either elastography or Doppler assessment. Similarly SPN did not frequently cause stricture of the pancreatic duct or common bile duct. Importantly, we confirmed that EUS-guided biopsy was an efficient and safe diagnostic tool. The needle type used does not appear to have a significant impact on the diagnostic yield. Overall SPN remains a challenging diagnosis based on EUS imaging with no pathognomonic features. EUS guided biopsy remains the gold standard in establishing the diagnosis.

The first case reported in Southeast Asia of Huge Frantz’s Tumor

Introduction: Solid pseudopapillary pancreatic tumor (Frantz’s Tumor) is a low-grade malignancy, that lacks clinical symptomatology. It was treatable with a favorable prognosis. Case presentation: A young female patient presented with increasing abdominal pain three years ago. Abdominal examination revealed a lump palpable. Contrast-enhanced Computed Tomography abdomen reported a solid mass measuring 13.9 cm x 13.2 cm x 8.9 cm from the pancreas with an enhancing cystic component. Fine-needle aspiration biopsy yielded a cellular sample comprising pseudopapillary neoplasm of the pancreas. A distal pancreatectomy was performed; she was discharged in satisfactory condition.

Long-term Survival, Quality of Life, and Molecular Features of the Patients With Solid Pseudopapillary Neoplasm of the Pancreas: A Retrospective Study of 454 Cases.

To present comprehensive information on the clinicopathological, molecular, survival characteristics, and quality of life (QOL) after surgery for solid pseudopapillary neoplasm (SPN) of the pancreas in a large cohort after long-term follow-up. SPN is a rare tumor with an uncertain malignant potential, and solid information on long-term prognosis and QOL remains limited. All hospitalized patients with SPNs who underwent surgery between 2001 and 2021 at the Peking Union Medical College Hospital were retrospectively reviewed. The clinicopathological characteristics of the patients were retrieved. A cross-sectional telephone questionnaire was administered to inquire about the QOL. Molecular analyses were performed using whole-exome sequencing. Exactly 454 patients with SPN were enrolled, of whom 18.5% were males and 81.5% were females. The mean patient age was 31 ± 12 years. In total, 61.3% of the patients had no symptoms. The size of the tumors was 5.38 ± 3.70 cm; 83.4% were solid cystic tumors, and 40.1% had calcifications. The proportions of local resection, distal pancreatectomy with or without splenectomy, and pancreaticoduodenectomy with or without pylorus preservation were 29.7%, 28.9% or 22.9%, and 11% or 6.8%, respectively. Over the years, there has been a significant shift from open to minimally invasive surgery. Among all surgical procedures, pylorus-preserving pancreaticoduodenectomy (PPPD) had the highest incidence of grade 2 to 4 complications (up to 32.3%), compared with 6.7% in distal pancreatectomy ( P < 0.001). Regarding histopathology, tissue invasion, perineural invasion, cancerous microvascular emboli, lymph node metastasis, and distant metastasis were present in 16.5%, 2.2%, 0.7%, 2.0%, and 3.1% of patients, respectively. Sixty patients were lost to follow-up. Sixteen of the 390 patients who underwent resection (4.1%) experienced local recurrence or distant metastasis after surgery. In total, 361 patients responded to the telephone survey. Nearly 80% of patients claimed their QOL was not significantly affected after surgery; however, the remaining 20% complained of lower QOL during 3 to 6 years of follow-up after surgery. No clinicopathological factor could reliably predict clinical recurrence or metastasis after resection. A total of 28 driver genes were detected with mutations in at least 2 tumor samples and the top 3 frequently mutated genes were CTNNB1 , ATRNL1 , and MUC16 . This study presented the largest cohort of patients with SPN after surgery from a single center and reported the QOL of these patients. SPN is associated with extremely favorable long-term survival, even in patients with metastasis, and most patients have a good QOL after surgery.

Rare Non-Neuroendocrine Pancreatic Tumours

The most common tumour of the pancreas is ductal adenocarcinoma (PDAC). It remains one of the most lethal non-neuroendocrine solid tumours despite the use of a multi-approach strategy. Other, less-common neoplasms, which are responsible for 15% of pancreatic lesions, differ in treatment and prognosis. Due to the low incidence rate, there is a lack of information about the rarest pancreatic tumours. In this review, we described six rare pancreatic tumours: intraductal papillary mucinous neoplasm (IPMN), mucinous cystadenoma (MCN), serous cystic neoplasm (SCN), acinar cell carcinoma (ACC), solid pseudopapillary neoplasm (SPN) and pancreatoblastoma (PB). We distinguished their epidemiology, clinical and gross features, covered the newest reports about courses of treatment and systematised differential diagnoses. Although the most common pancreatic tumour, PDAC, has the highest malignant potential, it is still essential to properly classify and differentiate less-common lesions. It is vital to continue the search for new biomarkers, genetic mutations and the development of more specific biochemical tests for determining malignancy in rare pancreatic neoplasms.

Solid Pseudopapillary Epithelial Neoplasm of the Pancreas: A Rare Entity with Diagnostic Dilemma

The solid pseudopapillary epithelial neoplasm (SPEN) of the pancreas is a relatively uncommon entity. The aim of the present study was to summarize our experiences with regard to diagnostic dilemma, surgery, postoperative follow-up, and management. This retrospective data were collected during the period from January 1, 2018 to December 31, 2020. A total of four patients (three females and one male) were identified within an age range of 13 to 25 years. All the patients were presented with nonspecific symptoms such as abdomen lumps, swelling in the abdomen, and abdominal pain. To reach a definite diagnosis, imaging studies were conducted along with endoscopic ultrasound fine-needle aspiration (EUS-FNA) and biopsy. After confirmation of SPEN on biopsy, all the patients underwent surgery without any complications. Patients are on follow-up, and to date, no metastasis has been detected. SPEN is a rare pancreatic tumor with unusual pathological features leading to a diagnostic dilemma. The pathologist should be familiar with SPEN and its salient histological characteristics that differentiate it from other look-alike pancreatic tumors and can help in timely surgery and management.

Perioperative safety and prognosis following parenchyma-preserving surgery for solid pseudopapillary neoplasm of the pancreas

Background/objectivesTo evaluate perioperative safety and outcome of parenchyma-preserving pancreatectomy and risk factors of metastasis and recurrence for patients with solid pseudopapillary neoplasm (SPN).MethodsDemographic data, operative and pathological parameter, follow-up data of patients with SPN undergoing their first operation were collected in our single center from May 2016 to October 2021 and compared between regular pancreatectomy group and parenchyma-preserving surgery group. Risk factors for metastasis and recurrence were investigated.ResultsA total of 194 patients were included, 154 of whom were female and the average age of all patients was 33 years old. Most patients were asymptomatic, with the most common complaint being abdominal pain or discomfort. Of them, 62 patients underwent parenchyma-preserving pancreatectomy including middle segment pancreatectomy and enucleation, and 132 patients underwent regular pancreatectomy including pancreaticoduodenectomy, distal pancreatectomy and total pancreatectomy. Patients in the parenchyma-preserving surgery group had a shorter duration of operation, less intraoperative bleeding, and decreased risk of combined organ removal and blood transfusion, with no statistical significance yet. The two groups exhibited a similar incidence of postoperative complications including grade B and C pancreatic fistula, delayed gastric emptying, postoperative pancreatic hemorrhage, and other complications, as well as radiological intervention, relaparotomy and the length of postoperative hospital stay. There were no perioperative deaths. All the patients, except 18 of those who discontinued follow-up, were alive with a median follow-up time of 31 months. Three patients in the regular pancreatectomy group were observed to have liver metastasis, and no metastasis was observed in the parenchyma-preserving surgery group. Significant risk factors for tumor metastasis and recurrence were tumor size, angioinvasion, and nerve infiltration.ConclusionsParenchyma-preserving surgery did not significantly increase the frequency of perioperative complications or recurrence and might be preferable if comprehensive conditions allow.

SOLID PSEUDOPAPILLARY TUMOR OF PANCREAS: A CASE REPORT AND REVIEW OF LITERATURE

We report a case of a 21-year-old female patient with a solid pseudopapillary tumor of the pancreas, who presented with abdominal pain and vomiting. Imaging studies revealed a well-defined mass in the body of the pancreas, which was confirmed by biopsy. The patient underwent central pancreatectomy and had an uneventful postoperative course. Follow-up imaging showed no signs of local recurrence.