Central Pancreatectomy - A Novel Surgical Technique Single Institution Experience.

Aims: Children evaluated for abdominal pain are increasingly recognized to have pancreatic lesions by imaging modalities. Malignant lesions of the pancreas have also been diagnosed at regular intervals, the most common being solid cystic pseudopapillary neoplasm (SPT) - Borderline (uncertain malignant potential). Surgical resection of this tumor should provide adequate tumor free margins and also should preserve pancreatic tissue. Radical resection of the pancreas will lead to pancreatic insufficiency. Herein, we describe the technique of central pancreatectomy wherein tumor excision gives adequate clearance but preserves the pancreatic tissue, thereby reducing significant morbidity. Materials and Methods: Three children ages ranging between 11 to 12 years diagnosed to have SPT were included in the study. Results: All children underwent successful central pancreatectomy and had an uneventful post operative recovery. Conclusion: Central pancreatectomy offers a good volume of remanant pancreas preserving near normal pancreatic function making it an ideal procedure for select cases.

Solid pseudopapillary tumor of the pancreas: A systematic review of clinical, surgical and oncological characteristics of 1384 patients underwent pancreatic surgery.

BACKGROUND: Pancreatic solid pseudopapillary tumors (SPTs) are rare clinical entity, with low malignancy and still unclear pathogenesis. They account for less than 2% of exocrine pancreatic neoplasms. This study aimed to perform a systematic review of the main clinical, surgical and oncological characteristics of pancreatic SPTs. DATA SOURCES: MEDLINE/PubMed, Web of Science and Scopus databases were systematically searched for the main clinical, surgical and oncological characteristics of pancreatic SPTs up to April 2021, in accordance with the preferred reporting items for systematic reviews and meta-analyses (PRISMA) standards. Primary endpoints were to analyze treatments and oncological outcomes. RESULTS: A total of 823 studies were recorded, 86 studies underwent full-text reviews and 28 met inclusion criteria. Overall, 1384 patients underwent pancreatic surgery. Mean age was 30 years and 1181 patients (85.3%) were female. The most common clinical presentation was non-specific abdominal pain (52.6% of cases). Mean overall survival was 98.1%. Mean recurrence rate was 2.8%. Mean follow-up was 4.2 years. CONCLUSIONS: Pancreatic SPTs are rare, and predominantly affect young women with unclear pathogenesis. Radical resection is the gold standard of treatment achieving good oncological impact and a favorable prognosis in a yearly life-long follow-up.

Outcome and prognosis after pancreatectomy in patients with solid pseudopapillary neoplasms.

BACKGROUND: Solid pseudopapillary neoplasms (SPN) are rare and represent approximately 4% of all cystic pancreatic tumors. The prognosis is excellent, although 10-15% of SPN patients show metastasis at the time of surgery or tumor recurrence during follow-up after pancreatectomy. Aim of the study was to analyze surgical management, risk factors for malignancy as well as long-term outcome and prognosis of this distinct tumor entity. METHOD: All patients with pancreatic resection for SPN between 10/2001 and 07/2018 in the authors' institution were identified from a prospective database. Clinicopathologic details, perioperative data and long-term follow-up results were retrospectively analyzed. RESULTS: Fifty-two patients were identified, 44 (85%) of them were female and the median age was 29 years (IQR 9-71). Seven (13%) patients showed a malignant behaviour of SPN with N1 (n = 2) or M1 (n = 1) disease at resection; 5 patients developed tumor recurrence, after a median of 21 months. During follow-up time (median 54 months) all patients were alive, the 5- and 10-year rates for disease-free survival were 89.0% and 81.6%, respectively. Significant risk factors for recurrence were age <18 years (p = 0.0087) and parenchyma-preserving surgical approaches (p = 0.0006). The postoperative long-term outcome showed ECOG = 0-1 in all patients, with resection related exocrine insufficiency in 20 (41%) and diabetes mellitus in 2 (4%) patients. CONCLUSIONS: Age < 18 years is a significant risk factor for malignancy in SPN, and parenchyma preserving resections harbor a significant risk for tumor recurrence. As recurrence may occur late, a systematic life-long follow-up should be performed.

Unresectable isolated hepatic metastases from solid pseudopapillary neoplasm of the pancreas: a case report of chemosaturation with high-dose melphalan.

BACKGROUND/OBJECTIVES: Solid pseudopapillary neoplasms of the pancreas (SPN) are rare tumors. For patients with unresectable liver metastases of SPN, no standard treatment has been defined so far. Here we report a case of a 40-year-old woman with SPN and metastases confirmed to the liver, and disease progression in the liver after primary tumor resection and chemotherapy with gemcitabine and cisplatin. METHODS: Chemosaturation with percutaneous hepatic perfusions is a minimally invasive, repeatable regional therapy which delivers chemotherapy directly to the liver while limiting systemic toxicity. As an individual treatment approach, the patient was treated with chemosaturation with percutaneous hepatic perfusions of melphalan. RESULTS: The procedure was performed twice within 8 weeks after which the liver metastases showed a marked reduction in size and vascularization (partial response). Grade 3 leukopenia after the second procedure was managed effectively with granulocyte colony-stimulating factor. No other toxicities were observed. Ten months after initiating treatment, the patient had a good performance status and remained stable. CONCLUSIONS: For SPN with unresectable liver metastases and progression despite systemic treatment, repeat chemosaturation with high-dose melphalan may also offer an effective regional treatment option.

Rare case of solid pseudopapillary neoplasm of the pancreas with liver and splenic metastasis in a 19-year-old girl.

INTRODUCTION: Solid pseudopapillary neoplasms (SPNs) of the pancreas are rare neoplasms, accounting for only 1 %-2 % of all pancreatic tumors, and predominantly affect female patients. CASE PRESENTATION: The present case report details a patient presenting to the emergency department with abdominal pain for 3 days who ultimately received a diagnosis of SPNs in the pancreatic body and tail. A contrast-enhanced computed tomography (CT) scan revealed a sizable mass arising from the pancreas, featuring an enhancing cystic component with involvement of the liver and spleen. The patient underwent subsequent exploratory laparotomy, a distal pancreatectomy, splenectomy, and partial hepatectomy. SPN diagnosis was confirmed by histopathology and immunohistochemistry with negative resection margins. CLINICAL DISCUSSION: Approximately 70 % of SPN cases are asymptomatic and are incidentally discovered. Despite advances in diagnostic modalities, preoperative diagnosis of SPNs remains a clinical challenge. Surgical management with negative resection margins remains the primary treatment approach. The recurrence rate after surgical resection has been reported to be 3 %-9 %. The prognosis for SPNs limited to the pancreas is generally favorable, with a cure rate exceeding 95 % after complete surgical resection. CONCLUSION: An SPN of the pancreas is a rare tumor observed in young female patients. Although it is classified as a malignant tumor, SPN has low malignant potential. Aggressive surgical resection, however, has proven effective in curing SPN for the majority of patients.

Frantz's tumor as incidental finding during Heller myotomy for achalasia: case report and review of literature.

Introduction: Solid pseudopapillary tumor is a low-grade malignancy of the pancreas and predominantly affects young women. This neoplasm is a rare pancreatic entity with vague clinical presentation. Diagnosis is often incidental through imaging or even during surgical approach for another condition. Case presentation: A 22-year-old Brazilian female with gastrointestinal symptoms was diagnosed with achalasia and underwent Heller myotomy. Intraoperatory findings included an enlarging mass in the distal pancreas. During follow-up for the surgical approach of achalasia, a hypothesis of Frantz's tumor was stated, and spleen-preserving distal pancreatectomy was performed. Discussion: The pathological pathways of Frantz's tumor is still unclear, and its connection with chromosomal abnormalities is under investigation. Although the tumor has been reclassified over the years to solid pseudopapillary tumor, surgical resection remains the standard treatment. Conclusion: Despite a surgical challenge, surgery presents a great prognosis in these patients and long-term survival. High suspicion and proper investigation are fundamental to diagnosis and early treatment.

Middle-Preserving Pancreatectomy for Multicentric Solid Pseudopapillary Neoplasm in a 10-Year-Old Female.

Solid pseudopapillary neoplasm (SPN) is a rare low-grade malignant tumor of the pancreas that occurs predominantly in young females. This tumor is occasionally multicentric, posing a unique surgical conundrum for resection. We present a case of a 10-year-old female with a history of multicystic dysplastic left kidney and persistent urogenital sinus who was diagnosed with biopsy-proven multicentric SPN of the pancreatic head and tail and underwent middle-preserving pancreatectomy. The patient tolerated the surgery very well. Our case is one of the few reported cases of multicentric SPN in a pediatric patient, and the only case treated with middle-preserving pancreatectomy, which is a novel surgical option for protecting pediatric patients from total endocrine and exocrine pancreatic insufficiency. With the increase in the incidence of SPN, there is an increasing need for pancreas-preserving surgical options, particularly in pediatric patients.

Imaging differentiation of solid pseudopapillary neoplasms and neuroendocrine neoplasms of the pancreas.

Purpose: The present study aimed to compare the computed tomography (CT) and magnetic resonance imaging (MRI) features of solid pseudopapillary neoplasms (SPNs) and pancreatic neuroendocrine neoplasms (pNENs). Method: Lesion imaging features of 39 patients with SPNs and 127 patients with pNENs were retrospectively extracted from 104 CT and 91 MRI scans. Results: Compared to pNEN patients, SPN patients were significantly younger (mean age 51.8 yrs versus 32.7 yrs) and more often female (female: male ratio, 5.50:1 versus 1.19:1). Most SPNs and pNENs presented as well-defined lesions with an expansive growth pattern. SPNs more often appeared as round or ovoid lesions, compared to pNENs which showed a lobulated or irregular shape in more than half of cases (p<0.01). A surrounding capsule was detected in the majority of SPNs, but only in a minority of pNENs (<0.01). Hemorrhage occurred non-significantly more often in SPNs (p=0.09). Signal inhomogeneity in T1-fat-saturated (p<0.01) and T2-weighted imaging (p=0.046) as well as cystic degeneration (p<0.01) were more often observed in SPNs. Hyperenhancement in the arterial and portal-venous phase was more common in pNENs (p<0.01). Enlargement of locoregional lymph nodes (p<0.01) and liver metastases (p=0.03) were observed in some pNEN patients, but not in SPN patients. Multivariate logistic regression identified the presence of a capsule (p<0.01), absence of arterial hyperenhancement (p<0.01), and low patient age (p<0.01), as independent predictors for SPN. Conclusions: The present study provides three key features for differentiating SPNs from pNENs extracted from a large patient cohort: presence of a capsule, absence of arterial hyperenhancement, and low patient age.

Solid pseudo papillary tumor of the pancreas: An unusual tumor in children.

An 11-year-old girl with a pancreatic solid pseudo papillary tumor (SPT) is reported. Contrast enhanced computerized tomography (CECT) & Magnetic resonant imaging (MRI) abdomen revealed a well-defined inhomogenous mass arising from the retro-peritoneum in the left upper quadrant of the abdomen possibly from the tail of the pancreas. USG guided biopsy of tumor showed benign pseudo papillary tumor of pancreas. Complete excision of the tumor was carried out. Histopathology revealed pancreatic pseudo papillary tumor. Patient did well postoperatively & is on regular follow-up.

Frantz's Tumor in Focus: The Tale of a 34-Year-Old Yemeni Female Patient.

A solid pseudopapillary tumor (SPT) of the pancreas, which mainly occurs in young women, is an uncommon pancreatic tumor that often presents diagnostic and therapeutic dilemmas. This case study discusses the symptoms and treatment approach for a 34-year-old woman from Yemen diagnosed with SPT. The patient was diagnosed through abdominal and pelvis CT scan, followed by ultrasound-guided biopsy confirming the presence of SPPT. Management through the Whipple procedure and portal vein reconstruction proved successful, with no recurrence or metastasis noted in a year-long follow-up. The importance of comprehensive understanding and surgical expertise in handling SPT is emphasized.

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

BACKGROUND: 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. AIM: To determine the characteristic EUS features of SPN and define its role in preoperative assessment. METHODS: 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. RESULTS: 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. CONCLUSION: 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.

Multidisciplinary treatment of advanced or recurrent solid pseudopapillary neoplasm of the pancreas: three case reports.

BACKGROUND: Solid pseudopapillary neoplasm (SPN) is a rare pancreatic tumor that predominantly affects young females. Prognosis is excellent; however, 10-15% of patients show metastasis at the time of surgery or develop tumor recurrence after pancreatectomy. CASE PRESENTATION: We reviewed the clinical course of three patients with advanced or recurrent SPN and subsequently underwent multidisciplinary treatment at our institution between 2002 and 2019. The primary tumor was resected in all three patients, and metastases were also resected if indicated. Intensive combined therapy, including re-resection, chemotherapy, ablation, arterial chemoembolization, and radiation therapy, allowed all patients to survive for a long time. The literature review showed that resection seems to be more effective than other treatments for metastatic SPN. CONCLUSIONS: Multidisciplinary treatment, including resection, may improve the prognosis of patients with SPN with recurrence or metastasis.

Resection of a large Solid Pseudopapillary Neoplasm of the Pancreas: A multidisciplinary feat and review of literature.

Solid pseudopapillary neoplasm (SPN) is an extremely rare tumor with low malignant potential which is generally located in the tail of the pancreas. The prevalence of SPN has increased with the recent advancement in radiological imaging. CECT abdomen and Endoscopic ultrasound-FNA are excellent modalities in preoperative diagnosis. Surgery is the main treatment modality of choice and a successful R0 resection is curative. We present a case of solid pseudopapillary neoplasm and included a summary of the current literature to provide a reference for the management of this rare clinical entity.

Clinical Course, Genetic, and Immunohistochemical Characterization of Solid Pseudopapillary Tumor of the Pancreas (Frantz Tumors) in a Brazilian Cohort.

Frantz tumors or solid pseudopapillary pancreatic neoplasm (SPN) are rare exocrine neoplasms that carry a favorable prognosis; they represent up to 3% of all tumors located in the region of the pancreas and have specific age and gender predispositions. In recent years, the rising curve of diagnosis is entitled to the evolution and access of diagnostic imaging. In this paper, we have retrospectively reviewed and described the clinical course of 40 patients with SPN from three institutions in Brazil, who had their diagnosis between 2005 and 2020, and analyzed the clinicopathological, genetic, and surgical aspects of these individuals. In accordance with the literature, most patients were women, 60% with unspecified symptoms at diagnosis, with tumors mainly located in the body and tail of the pancreas, of whom 70% underwent a distal pancreatectomy with sparing splenectomy as a standard procedure, and none of the cases have experienced recurrence to date. Surgery still remains the mainstay of treatment given the low metastatic potential, but more conservative approaches as observed in this cohort are evolving to become the standard of care. Herein, we present an in-depth analysis of cases focusing on the latest literature and report some of the smallest tumor cases in the literature. To our knowledge, this is the first report evaluating germline genetic testing and presenting a case of detected Li-Fraumeni syndrome.

A classic case of Solitary Pseudopapillary Epithelial Neoplasm of Pancreas - Case report with Review of Literature.

We report a case of a solid pseudopapillary epithelial neoplasm of the pancreas in a 19-year-old female who presented with abdominal pain. Computed tomography of the abdomen showed a heterogenous density mass in the tail of the pancreas. The mass had fluid density in the central region and soft tissue density in the peripheral region. Post-contrast evaluation showed enhancement in the peripheral solid component. Based on the radiological investigation carried out at our institute, a diagnosis of solid pseudopapillary epithelial neoplasm of the pancreas was established which was confirmed on histopathology.

Yield of surgery in solid pseudopapillary neoplasms of the pancreas: A case series and literature review.

BACKGROUND: Solid pseudopapillary neoplasms (SPN) of the pancreas represents approximately 2% of non-endocrine tumors of the pancreas. It is described in the literature as a rare and predominant tumor in young women. AIM: To report a case series with SPN and analyzing clinical, surgical, anatomopathological characteristics, as well as the prognosis and review of literature. METHODS: Retrospective analysis of patients undergoing surgery, with histological diagnosis of SPN between 1998 and 2018, using standardized and prospectively completed forms, performed at the Surgery Service of the Upper Digestive System at Hospital São Rafael/Rede D'Or in Salvador - BA. Review of literature through a database search in MEDLINE/PubMed of retrospective articles. RESULTS: Fourteen female patients with the average age of 31.6 years (range min-max) were selected. Twelve patients (85.7%) were asymptomatic, being an incidental diagnosis or due to screening for other reasons. One patient had abdominal pain due to gastric compression and another patient had jaundice. The 14 patients were staged with computerized tomography or magnetic resonance imaging. None had evidence of metastasis. In 8 patients (57.1%), the tumor was in the tail and body. The average size was 6.7 cm (range min-18). The type of surgery was according to the anatomical location of the tumor. There was no lymph node involvement. In two cases, vascular resection with the use of a prosthesis was required for reconstruction. The surgical margins were free. In all cases, postoperative immunohistochemistry confirmed that it was a solid pseudo-papillary neoplasia of the pancreas. There has been no disease recurrence in any case so far. CONCLUSION: The tumors had a benign, indolent and histopathological behavior compatible with the literature. Curative surgery is recommended in all cases.

Frantz tumor in a 58 year old woman; case report and literature review.

INTRODUCTION: Frantz tumor or solid pseudopapillary neoplasm is a very rare tumor with low malignant potential, it constitutes 1-2 % of exocrine tumors of the pancreas. DESCRIPTION OF THE CASE: We present the case of a 58-year-old female patient with a 4-month history of occasional abdominal pain in the epigastrium, the tomography detected a distal tumor of the pancreas. Is taken to distal pancreatectomy. DISCUSSION: It was first described by Franz in 1959 as a papillary tumor of the pancreas, in 2010 it was reclassified as a solid pseudopapillary tumor of low grade of malignancy. They appear in 8-16.6 % in patients before 13 years of age, in young women they present between the second and fourth decade of life. The treatment for excellence is surgical resection. CONCLUSION: Pancreatic tumors represent a surgical challenge in any place of presentation.

Solid pseudopapillary epithelial neoplasm (SPEN) of the pancreas involving the distal body and proximal tail: A case report.

INTRODUCTION AND IMPORTANCE: Solid Pseudopapillary Epithelial Neoplasm (SPEN) of the pancreas is a rare cystic exocrine tumor of the pancreas most commonly occurring in women between 30 and 40 years of age. This case report aims to demonstrate the clinicopathological findings encountered and the management of a patient diagnosed with SPEN. CASE PRESENTATION: An 18-year-old woman with gradually progressive and intermittent abdominal pain in the epigastric region presented to our outpatient department. Physical examination elicited tenderness to palpation in the epigastric area, and imaging findings suggested SPEN of the pancreas involving distal body and proximal tail region of the pancreas. The tumor was resected, and the diagnosis was confirmed on histopathology examination. CLINICAL DISCUSSION: SPEN is a slow-growing tumor with a low-grade malignant potential, found incidentally in asymptomatic patients and symptomatic patients present with abdominal pain. The average tumor size is about 4 to 6 cm in diameter. Imaging is essential for diagnosis, and distal pancreatectomy with splenectomy was the most commonly reported procedure. CONCLUSION: It is crucial to consider a diagnosis of SPEN in women with abdominal pain in the epigastric region as early surgical resection of the tumor results in resolution and excellent prognosis.

Solid pseudopapilar neoplasm of the pancreas.

Solid pseudopapillary tumor of the pancreas is a rare entity, more frequent in women between the 2nd and 4th decades. The diagnosis is usually incidental and it can be reached by computed tomography or magnetic resonance imaging. Subsequent pathological confirmation is necessary for an adequate treatment. A retrospective study of six cases was carried out. All the patients were female, between 14 and 56 years of age, in which 50% the tumor were an incidental finding. We had three cases located in the head and three in the body of the pancreas. We performed three pancreaticoduodenectomies and three distal pancreatectomies with splenic preservation, without disease recurrence.

El tumor sólido-quístico de páncreas es poco frecuente y predomina en mujeres entre la segunda y la cuarta décadas de la vida. Los pacientes son generalmente asintomáticos. El diagnóstico se realiza por imágenes con tomografía o resonancia magnética, y con la posterior confirmación patológica para poder ofrecer un tratamiento adecuado. Presentamos una serie de seis casos. Todas las pacientes fueron de sexo femenino, de entre 14 y 56 años. El 50% fueron un hallazgo incidental. Tuvimos tres casos localizados en la cabeza y tres en el cuerpo del páncreas. Se realizaron tres duodenopancreatectomías cefálicas y tres pancreatectomías distales con preservación esplénica, con buena evolución y sin recidiva.

Hepatic metastasis in Frantz's tumor: A case report.

INTRODUCTION: Frantz's Tumor or Solid Pseudopapillary Neoplasm (SPN) is rare with a solid-cystic pattern, and most common in young women. PRESENTATION OF CASE: This study based on guidelines for case reports (SCARE) reports a case of SPN in a teenager aged 13 years at the diagnosis time, attended at a teaching public hospital in Brazil, which evolved into liver metastases. Clinical, laboratory, therapeutic and imaging data were collected from the physical chart and analyzed in light of current publications on the topic. The first consultation occurred in January 2012, where weight loss, fever, vomiting, left-sided hypochondrium and epigastric pain were reported. Imaging exams evidenced an expansive heterogeneous process in the pancreatic tail; however, laboratory exams and tumor markers did not present alteration in relation to reference values. In March of 2012, she underwent caudal body pancreatectomy, splenectomy, segmental colectomy and colo-coloanastomosis as a function of the intraoperative findings. After 63 months, right-sided hepatectomy was performed to resect metastases. Currently, she is undergoing outpatient monitoring, without complaint or alterations in imaging and laboratory exams, totaling 100 months of global survival. DISCUSSION: This is an interesting case report of a rare tumor, in so far as without any adjuvant chemotherapies, an 8-year long survival time could be achieved in this particular type of tumor despite initially large tumor expansion and later liver metastases. CONCLUSION: Additional epidemiological studies, molecular and clinical trials are required to increase knowledge on SPN.