Case Of Solid Pseudopapillary Tumor Research Articles

Tomographic characteristics of solid-pseudopapillary neoplasms (Frantz tumor).

Solid-pseudopapillary neoplasms (SPN), or Frantz tumor, is a rare, low-grade neoplasm that occurs mainly in young women. It was described in 1959 by Virginia Frantz and constitutes 0.2-2.7 % of all pancreatic tumors. Computed tomography (CT) plays an important role in the diagnosis of this pathology of scarce reporting. The objective of the present study is to describe the epidemiological and tomographic characteristics of the SPN in the Instituto Nacional de Enfermedades Neoplásicas (INEN) of Peru. Descriptive cross-sectional study performed with medical records of all patients diagnosed with SPN between 2004 and 2014. The variables described were tumor size, location, shape, borders, thickness of the capsule, composition, calcifications and uptake of contrast. The categorical variables were expressed in absolute and relative frequencies; while the numerical variables were described with median and interquartile deviation (ID). Statistical support STATA Version 12.0 was used. Of all pancreatic cystic tumors (PCT), 51.9% corresponded to confirmed cases of TSP. The median age was 23.5 years. The isolated location in the head predominated (33.3%); the most frequent mixed location was body and tail (16.7%); diameter was more frequent between 5.1-10 cm and the content of the majority was predominantly solid. 30.0% of the NSP presented calcifications. Most cases of INP SPN (2004-2014) have similar characteristics to those reported in the international literature.

Utility of Contrast-Enhanced Harmonic Endoscopic Ultrasound for the Guidance and Monitoring of Endoscopic Radiofrequency Ablation.

Background/AimsInterventional endoscopists may utilize contrast-enhanced harmonic endoscopic ultrasound (CEH-EUS) for image guidance during radiofrequency ablation (RFA) because of its capability to delineate real-time tumor perfusion dynamics. The purpose of this study was to assess the utility of CEH-EUS for the guidance and monitoring of endoscopic RFA.MethodsNineteen consecutive patients with solid abdominal tumors who underwent CEH-EUS and endoscopic RFA were included. The extent of the ablation was assessed by CEH-EUS at 5 to 7 days after RFA. Additional RFAs were performed under CEH-EUS guidance.ResultsThe diagnoses were as follows nonfunctioning neuroendocrine tumor (n=13), solid pseudopapillary neoplasm (SPN) (n=2), insulinoma (n=1), left adrenal adenoma (n=2), and left adrenal oligometastasis (n=1). Pre-CEH-EUS findings revealed that 17 cases showed hyperenhanced patterns and two cases of SPN showed isoenhanced patterns. CEH-EUS-assisted RFA was technically feasible in all 19 patients. After the first RFA session, seven patients of the treated tumors showed the disappearance of intratumoral enhancement on CEH-EUS, whereas 12 showed residual contrast enhancement. Twelve patients with incomplete ablation were further treated with additional RFA under real-time CEH-EUS guidance. Radiologic complete response was observed in 13 patients (68.4%). Among the 35 ablation procedures, the only adverse events were two episodes of pancreatitis (5.7%; 1 moderate and 1 mild). During the median follow-up of 28 months, the local recurrence rate was 7.7%.ConclusionsThe application of CEH-EUS for RFA could be helpful in assessing early treatment response after ablation and targeting residual viable tumors during additional ablation sessions.

Solid pseudopapillary tumor of the pancreas

Solid pseudopapillary neoplasia of the pancreas is a rare tumor. It accounts for <1%–2% of exocrine pancreatic tumors. This relatively indolent tumor has the propensity to affect young women. We herein report a case of solid pseudopapillary tumor of the pancreas in a 24-year-old woman; for which curative distal pancreatectomy with splenectomy was performed. Postoperative period was uneventful, and during a follow-up of 2 years, she remains asymptomatic.

Investigation of Fluorodeoxyglucose Positron Emission Tomography for the Diagnosis of Solid Pseudopapillary Neoplasm of the Pancreas: A Study Associated With a National Survey of Solid Pseudopapillary Neoplasms.

ObjectivesThis study aimed to investigate the utility of fluorodeoxyglucose (FDG) positron emission tomography for solid pseudopapillary neoplasm (SPN) diagnosis.MethodsThe subjects included 53 cases of SPN. We compared the maximal standardized uptake volume (SUVmax) with those of 25 cases of pancreatic duct cancer and 18 cases of pancreatic neuroendocrine neoplasm. In addition, immunopathological testing for SPN with regard to FDG uptake was undertaken.ResultsAn increase in SUVmax was observed in all tumors with increased tumor diameter. Among tumors of 20 mm or smaller, the SUVmax of SPN was significantly higher than those of pancreatic duct cancer and pancreatic neuroendocrine neoplasm. The results of a pathological study of FDG uptake in SPN revealed increased glucose transporter protein type 1 expression with tumor enlargement. Furthermore, increased hypoxia-inducible factor-1 and vascular endothelial growth factor expression under hypoxic conditions were observed in the areas of necrosis.ConclusionsIn cases in which high FDG uptake is observed in small pancreatic tumors, FDG positron emission tomography is potentially useful for SPN differentiation. The factors involved in FDG uptake in SPN include cell density and glucose transporter protein expression, as well as hypoxia-inducible factor and vascular endothelia growth factor expression in the hypoxic environment of necrotic areas.

Targeted next generation sequencing of pancreatic solid pseudopapillary neoplasms show mutations in Wnt signaling pathway genes.

Solid pseudopapillary neoplasms of the pancreas are rare neoplasms that have been shown to harbor recurrent somatic pathogenic variants in the beta-catenin gene, CTNNB1. Here, we used targeted next generation sequencing to analyze these tumors for other associated mutations. Six cases of solid pseudopapillary neoplasms were studied. DNA extracted from formalin-fixed paraffin embedded tissue blocks was analyzed using the Ion Torrent platform, with the 50-gene Ampliseq Cancer Hotspot Panel v2 (CHPv2), with further variant validation performed by Sanger sequencing. Four tumors (67%) were confirmed to harbor mutations within CTNNB1, two with c.109T > G p.(Ser37Ala) and two with c.94G > A p.(Asp32Asn). One case showed a frameshift deletion in the Adenomatous Polyposis Coli gene, APC c.3964delG p.(Glu1322Lysfs*93) with a variant allele frequency of 42.6%. Sanger sequencing on non-tumoral tissue confirmed the variant was somatic. The patient with the APC mutation developed metastasis and died. In addition to the four cases harboring CTNNB1 variants, we found a case characterized by poor outcome, showing a rare frameshift deletion in the APC gene. Since the APC product interacts with beta-catenin, APC variants may, in addition to CTNNB1, contribute to the pathogenesis of solid pseudopapillary neoplasms via the Wnt signaling pathway.

Solid Pseudopapillary Neoplasm – 12 years’ experience from tertiary center of Nepal

Introduction: Solid pseudopapillary neoplasm (SPN) are rare, relatively indolent tumor with potential malignant behavior. Incidence of these tumors is increasing due to widespread use of cross sectional imaging. Surgery remains the mainstay of treatment, and the only curative option. Aim of the study is to analyze the clinicopathological details, surgical management and outcomes of operated cases of SPN. Method: This is a retrospective review of diagnosed SPNs confirmed histopathologically after surgical resections at Tribhuvan University Teaching Hospital (TUTH), Kathmandu, Nepal from 2006 November to 2018 October. Patients clinical details, surgical procedures, perioperative outcomes and follow up were recorded. Data were analyzed using Statistical Package for the Social Sciences (SPSS) version 23. Result: Over the period of 12 years, total of 14 cases with median age of 22.5 years (11-52) had female predominance (13). Four cases were diagnosed incidentally while pain (5) was the most common presentation followed by lump (4). Location of tumor were head (4), uncinate process (2), neck (2), body (3) and tail (3). Pancreaticoduodenectomies were performed in 4 cases along with enucleation (3), central pancreatectomies (3), distal pancreatectomy (1) and distal pancreatico-splenectomies (3). Margin were negative in all cases with median tumor size of 6.5 cm (2.5 -18). Postoperatively, 21.4 % developed major complications with Clavien-Dindo (grade III & above) out of which 1 patient (7.1%) had mortality. Median hospital stay was 7 days (4-16). One patient was lost to follow up while remaining had median follow up of 42 months (1 month – 12 years) and revealed no issues. No recurrences were noted. Conclusion: SPNs are low grade tumor with good prognosis. Complete surgical resection is treatment of choice, with excellent survival. Keyword: SPN, Solid Pseudopapillary Neoplasm, TUTH, Nepal

Solid pseudopapillary neoplasm of the pancreas in Sub-Saharan Africa: A case report and review of literature

Solid pseudopapillary neoplasm of the pancreas is a rare indolent malignant tumor mostly found in young women in the second and third decades of life. Only about 3000 cases have been reported in English literature, with none in the West African region. Here, we report to the best of our knowledge, the first histologically confirmed case of solid pseudopapillary neoplasm of the pancreas in West Africa. A 29-year-old woman with a slow-growing left upper abdominal mass of 10 months. Abdominal ultrasound and computed tomography scans revealed a cystic-solid mass arising from the tail of the pancreas. She underwent surgical excision of the mass, with post-operative histopathology findings in keeping with solid pseudopapillary neoplasm of the pancreas. This case report highlights the importance of improved abdominal imaging, histopathology and the need for increased awareness as a differential diagnosis of an intra-abdominal tumor, especially in young women.

Analysis of 50 cases of solid pseudopapillary tumor of pancreas: Aggressive surgical resection provides excellent outcomes

IntroductionThis study reports the clinicopathological characteristics and the perioperative and long-term treatment outcomes after aggressive surgical resection in solid pseudopapillary tumor (SPT) of the pancreas performed at a high volume center for pancreatic surgery in India. Materials and methodsWe analyzed a prospectively maintained database of the patients operated for SPT at Tata Memorial Hospital, India over a period of 11 years from February 2007 to February 2018. ResultsFifty consecutive patients operated for SPT, during the study period were included. The median age at presentation was 24 years. Majority of the patients (43/50) were female (86%). Disease was predominantly localized in the head and uncinate process of pancreas (66%). Median tumor size was 7.7 cm (Range 1.6–15 cm). Tumor extent was radiologically defined as borderline resectable or locally advanced in 48% (n = 24) patients. Forty-six major pancreatic resections were performed, which included 10 (21%) vascular resections, 2 synchronous liver metastasectomies, 1 multi visceral resection and 5 total pancreaticosplenectomies. Five of these resections were reoperations in patients deemed inoperable on exploration at other centers. R0 resection was achieved in 47 patients (98%). Postoperative major morbidity was 19% and there was no mortality. At a median follow-up of 29 months (Range, 1–121 months), all patients were alive without any recurrence. ConclusionAggressive complete surgical resection of SPT achieves excellent long-term survival. Surgery, especially for large and borderline resectable tumors, can be potentially complex and should be performed at high-volume centers to provide the best chance of cure.

Solid Pseudopapillary Neoplasm of Pancreas, Presentation and Management of a Rare Case

Solid Pseudopapillary Neoplasm (SPN) of the pancreas is a rare neoplasm that typically affects young females. It is a low-grade malignant tumor which is quite commonly misdiagnosed as pancreatic pseudo-cyst. We report a case of SPN in a young female who presented with epigastric pain. Computerized Tomographic (CT) scan of pancreas revealed well demarcated lesion in the head of pancreas. She underwent Whipple procedure. Histopathology confirmed the diagnosis. She did not need any further treatment.

TFE3 is a diagnostic marker for solid pseudopapillary neoplasms of the pancreas

Aberrant Wnt signaling is a hallmark of solid pseudopapillary neoplasms (SPNs) of the pancreas. Transcription factor E3 (TFE3) plays a critical role in activation and regulation of the Wnt pathway and is predicted to be a candidate gene implicated in SPN by gene regulatory network analysis. The aim of this study was to evaluate TFE3 as a marker for SPN. Paraffin-embedded tissues of SPN (n = 75) and other primary pancreatic tumors were analyzed, including pancreatic neuroendocrine tumors (n = 17), pancreatic ductal adenocarcinomas (n = 14), pancreatic neuroendocrine carcinomas (n = 4), and acinar cell carcinomas (n = 3). The clinicopathological features were summarized as well. Differentiation of specific pancreatic duct or acinus was not found in any SPN tissue. Morphologic and immunohistochemical results indicated that SPN displays certain characteristics of neuroendocrine cells. Overall, 71 (94.67%) cases of SPN showed nuclear accumulation for TFE3, most of which displayed moderate to intense expression. The TFE3 positive rates in pancreatic neuroendocrine tumor, pancreatic ductal adenocarcinoma, and pancreatic neuroendocrine carcinoma were 23.53%, 14.29%, and 25%, respectively. All 3 cases of acinar cell carcinoma were negative for TFE3. We conclude that SPN may originate from primordial pancreatic cells and is accompanied by some characteristics of neuroendocrine tumors. TFE3, besides β-catenin, can be an additional diagnostic marker of SPN in differential diagnosis.

Solid Pseudopapillary Neoplasm of Pancreas In A 35 Year Old Lady

Solid pseudopapillary neoplasm of pancreas (SPN) is a rare entity; therefore proper diagnosis, evaluation and formulation of treatment protocols is difficult. The commonest presenting symptom is abdominal pain. This article presents a case of solid pseudopapillary neoplasm of pancreas in a 35 years old lady. A high index of clinical suspicion is necessary to suspect and diagnose SPN. This diagnosis should be borne in mind when young female patients present with a pancreatic mass.J Shaheed Suhrawardy Med Coll, June 2017, Vol.9(1); 31-34

急性膵炎を契機に診断したsolid pseudopapillary neoplasmの一例

急性膵炎を契機に診断したsolid pseudopapillary neoplasmの一例

Features and Treatment of Peritoneal Metastases from Solid Pseudopapillary Neoplasms of the Pancreas.

BackgroundSolid pseudopapillary neoplasms (SPNs) of the pancreas may present widespread peritoneal metastases, but the treatment of this malignancy has not been well described and requires further investigation.Material/MethodsFour cases of SPN with significant peritoneal metastases in our department were operated and retrospectively summarized after long-term follow-up. Eight more cases of peritoneal metastatic SPN from the PubMed database were also included in the analysis.ResultsPeritoneal metastases of SPNs have different gross features. The benign nodules were tenacious and well encapsulated, while the malignant nodules were soft and prone to slow bleeding. However, neither of these nodules invaded the small intestines or mesentery. Of the 12 disseminated cases, 7 had history of primary tumor rupture, whereas the others had tumors malignant in nature. A total of 14 surgical events were documented, including 3 complete cytoreductive surgeries (CCRS), 9 cytoreductive surgeries (CRS), and 2 debulking surgeries. After follow-up ranging from 0.3 to 6.1 years, the results of the Fisher’s exact test showed no difference between CCRS and CRS in treating either low-grade or high-grade malignant SPNs (P=0.257 and P=0.203, respectively). For all cases of SPN with peritoneal metastases, the CCRS procedure could significantly improve tumor-free survival (TFS) compared to the CRS procedure (P=0.046).ConclusionsSPN rupture could cause significant peritoneal metastases, and either disruption or biopsy of these lesions should be avoided. Peritoneal metastases from SPNs vary both in gross features and biological mechanisms. CCRS may offer optimal therapeutic outcomes and longer TFS for individuals with significant peritoneal metastases of SPNs.

A rare case report of solid pseudopapillary tumor of the pancreas

A solid pseudopapillary tumor is a low-grade malignant cystic exocrine neoplasm of the pancreas of papillary architecture that typically afflicts young women with excellent postsurgical curative rates and rare metastasis. It may be locally aggressive. Pathological evaluation remains the gold standard in reaching a definitive diagnosis. We report a case of solid pseudopapillary neoplasm of the pancreas that was suspected on radiology and diagnosed preoperatively on biopsy guiding definitive surgery.

A Systematic Review of Solid Pseudopapillary Neoplasms: An Update Since 2012

Introduction: Solid-pseudopapillary neoplasms (SPNs) were first described by Frantz in 1959 and defined by the World Health Organization in 1996. SPNs are rare cystic tumors of the pancreas which usually present as large, solitary, well-circumscribed lesions. While the majority of patients have localized disease, there has been a reported incidence of malignant transformation of 9-15%. An initial literature review identified 718 cases of SPN that had been reported in the English language up to 2003. A follow up systematic review was then performed which identified 2,744 cases of SPN published in the English literature up to 2012. The aim of this study was to determine if there had been any change in the number of SPN cases detected and their evaluation or management over time since 2012. Methods: A systematic review of SPNs was performed of all articles published in English in PubMed and SCOPUS. Predetermined search strings that included the terms “pancreas” and “pseudopapillary” were used. A review of the bibliography of all studies was performed as part of the systematic review. Results: 1,170 patients with SPN were identifiedin 49 studies published between September 19, 2012 (end date of the previous systematic review) to May 1, 2017. 1,103 (94.3%) patients were female and the mean age was 33 years (S.D. ± 10.7). The most common symptom was abdominal pain in 52.7% with incidental detection in 28.1%. There were 1,114 patients who underwent pancreatic resection. The mean tumor size was 8 cm (S.D. ± 3.7). Follow up was reported for 870 patients, with mean follow-up of 44.7 months (S.D. ± 48.9). Disease-free survival was documented in 1,069 (91.4%) patients with recurrence in 101 (8.6%). The mean time to recurrence was 35.1 months (S.D. ± 30.7). Chemotherapy for treatment of recurrence was administered in seven cases, while transarterial chemoembolization (TACE) was used to treat liver metastases in two cases. Conclusion: The number of SPNs reported in the literature has increased by just over 40% in nearly five years. These tumors continue to be found primarily in young women and are frequently found with nonspecific symptoms, such as abdominal pain, or are found incidentally. Surgery remains the mainstay of treatment with an excellent long term prognosis.

Insulinoma-associated protein 1 (INSM1) is a useful marker for pancreatic neuroendocrine tumor.

Insulinoma-associated protein 1 (INSM1) is an important biomarker of Achaete-scute homolog-like 1-driven pathways. For diagnosis of pancreatic neuroendocrine tumors (PanNET), chromogranin A (CGA), synaptophysin (SYP), and neural cell adhesion molecule (NCAM) were also considered as potential biomarkers. However, it is often difficult to diagnose it immunohistochemically. Hence, we examined the expression pattern of INSM1 in pancreatic solid tumors. We detected INSM1, CGA, SYP, and NCAM immunohistochemically, in 27 cases of NET [pure type: 25 cases, mixed adenoneuroendocrine carcinoma (MANEC): 2 cases]. We included 5 cases of solid-pseudopapillary neoplasm (SPN), 7 cases of acinar cell carcinoma (ACC), and 15 cases of pancreatic ductal adenocarcinoma (PDAC) as the control group. Nuclear expression of INSM1 was found in all PanNET pure type cases. However, expression of INSM1 was negative in PDAC, ACC, and SPN in all cases, whereas faint expression was seen in the cytoplasm from SPN. MANEC comprises of two components: neuroendocrine carcinoma and adenocarcinoma components. The NET component was positive for INSM1 expression, whereas the PDAC component does not express INSM1, which aids in distinguishing these components. Our results suggest that INSM1 is a useful immunohistochemical marker for diagnosing pancreatic neuroendocrine tumor.

Редкие клинические наблюдения солидной псевдопапиллярной неоплазмы поджелудочной железы у беременных

The article presents the world data on the incidence, clinical picture and diagnosis of solid pseudopapillary neoplasm both in general and in pregnant women. Two clinical cases of solid pseudopapillary neoplasm in pregnant women were described in detail, with highly specialized surgical and perinatal care in the hospitals of the Center for Surgery of Liver, Biliary and Pancreatic Diseases n.V.S.Zemskov (surgical department) and in the department of surgical gynecology of the SI «Institute of pediatrics, obstetrics and gynecology National Academy of Medical Sciences of Ukraine». Demonstrate the features of diagnosis, surgical treatment of patients with solid pseudopapillary neoplasms during pregnancy. Key words: solid pseudopapillary neoplasm, surgical treatment, pregnancy, management of pregnancy and childbirth.

A Case of Solid Pseudopapillary Neoplasm Spontaneously Ruptured Into the Duodenum: Case Report

There have been no reports of ruptured solid pseudopapillary neoplasm (SPN) into adjacent organs. A 22-year-old female was referred to our hospital for treatment of a pancreatic head tumor. Computed tomography (CT) examination at our hospital showed a 5-cm tumor containing air, although CT at a previous hospital revealed an 8-cm tumor without air. Thus, a spontaneous rupture of the tumor into the duodenum was suspected. Subtotal stomach preserving pancreaticoduodenectomy with combined resection of the portal vein was performed. Contrast radiography of resected specimen showed the medium injected into the tumor leaking out from the 2nd portion of the duodenum. Histologically, the patient was diagnosed as SPN. Microscopic invasion to the portal vein and duodenum were also confirmed. She did not experience any postoperative complications and has remained well without any signs of recurrence during 2 years of follow-up. Although there have been 14 studies reporting ruptured SPN, this is the first report of SPN that spontaneously ruptured into the duodenum. An extremely rare case of SPN of the pancreatic head that spontaneously ruptured into the duodenum was reported.

Image Findings of Solid Pseudopapillary Neoplasms of the Pancreas on Multiphasic Multidetector CT Scan-A Single Institute Experience from Southern India.

Solid Pseudopapillary Neoplasms (SPN) are uncommon pancreatic tumours and are slow growing with uncertain malignant potential, showing female preponderance. Postoperative prognosis is good and metastasis is rare. To summarise the imaging and pathological features of seven cases of SPN in three years period, from January 2013 to January 2016. In this retrospective study the imaging features of seven cases on triphasic multidetector Computed Tomogram (CT), a 16-slice scanner, were reviewed along with CT-guided Fine Needle Aspiration Cytology (FNAC) and histopathological examination. Statistics were expressed in terms of percentiles. All cases were female patients with an age range of 13-35 years (mean: 23.3 years). On CT assessment, the size of the tumours varied from 2.5-14 cm (mean: 5.3 cm). All these tumours were well capsulated and round to oval in shape. In four out of seven cases, the tumour was located in the tail of pancreas. All the solid enhancing portions showed moderate enhancement of at least 20-30 HU compared to unenhanced scan, on the other hand the cystic parts remained unenhanced with <5 HU variation in comparison to the plain scan. Histopathological examination exhibited characteristic poorly cohesive cuboidal cells arranged in papillaroid pattern having fine nuclear chromatin with nuclear grooves. Solid pseudopapillary neoplasm is a high diagnostic possibility in case of a young female having pancreatic mass and needs to be evaluated with triphasic contrast enhanced CT scan, followed by FNAC and or histopathological examination.

The Value of MRI in Diagnosis of Solid Pancreatic Tumors

Aim: To investigate the value of 3.0 T MRI in the diagnosis of solid pancreatic tumor. Materials and Methods: Aggregate of 36 patients were enlisted with suspected or proven as solid pancreatic tumors were collected from the first affiliated Hospital of Anhui Medical University Teaching Hospital during the period of January 2012 to August 2015, contained 20 cases of pancreatic adenocarcinoma, 7 cases of pancreatic neuroendocrine tumors (PNETs), 5 cases of pancreatic metastases, 4 cases of solid pseudopapillary tumors (SPTP). All the patients performed with plain scan and multiphasic contrast enhanced imaging on 3.0 T MR, 23 cases performed with MRCP. The data of clinic and imaging were retrospectively analyzed. Results: Pancreatic adenocarcinoma was irregular in shape, lesion was hypo intense in 10 cases, slightly hypo intense in 9 cases and isointense in one case on T1WI. 10 tumor showed slightly enhancement in arterial phase, and all of tumors showed moderate enhancement during portal and delayed phase; 19 cases showed double duct sign, and one case showed distal pancreatic duct dilation. Of the 7 cases with PNETs, the margin was well defined in six well differentiated lesions and ill-defined in one pancreatic neuroendocrine carcinoma; six tumors were marked enhancement in arterial phase and one tumor was moderate enhancement in pancreatic parenchyma in all of three phases. The mean diameter of SPTP was 5.2 cm. The solid part of tumors was hyperintense on T1WI and slightly hyper intense on T2WI, with progressively enhancement. 4 SPTPs had capsule which were hyperintense on T1WI and T2WI exhibited moderate enhancement. Of the 5 pancreatic metastases, one cases had only one lesion, two cases had multiple lesions and two cases showed diffuse pancreatic enlargement, one case showed heterogenous slightly enhancement, two case showed peripheral rim enhancement, two case showed marked enhancement in arterial, then wash-out in delayed and portal phases. Conclusion: MRI is valuable for diagnosis and distinguishing solid pancreatic tumors.