Solid Pseudopapillary Tumor Of Pancreas Research Articles

Childhood Solid Pseudopapillary Neoplasm of the Pancreas: Acute Presentation of an Uncommon Tumor

AbstractSolid pseudopapillary tumor is a mixed solid and cystic neoplasm of the pancreas which occurs most commonly in females in second or third decade of life with few reported pediatric cases. It has a low malignancy potential with good prognosis after surgical resection.We present a case of an 11-year-old male child with solid pseudopapillary tumor of pancreas who presented with clinical features of acute pancreatitis. He was initially evaluated with ultrasound, followed by computed tomography (CT) and magnetic resonance imaging (MRI).Solid pseudopapillary tumor of pancreas presenting as acute pancreatitis is rarely reported in literature. This case is a unique presentation of a pancreatic tumor and highlights the value of multiphase CT imaging and MRI in the diagnosis.

Solid Pseudopapillary Neoplasm of the Pancreas: Unraveling Insights from a Single Institutional Study Emphasizing Preoperative Diagnosis of a Rare Tumor.

Solid pseudopapillary neoplasm (SPN), a slow-growing pancreatic tumor with a vague clinical presentation and non-specific radiological features, is rather uncommon. We share our experience emphasizing on preoperative diagnosis and the correlation with final histopathological examination. This is a retrospective analysis of the 468 patients who underwent pancreas-related surgery at our institution between January 2013 and July 2022. Demographic characteristics, symptoms at presentation, preoperative serum calcium carbohydrate antigen (CA 19-9), lesion characteristics on cross-sectional diagnostic imaging, surgical technique, complications in postoperative period, length of stay, histopathological features, and 3-year follow-up findings of the patients with SPN of pancreas were evaluated. The male-to-female ratio was 1:11 and the mean age at presentation was 33.3 ± 9.5 years. Upper abdomen discomfort was the most common presenting complaint (91%). And five patients had findings suggestive of SPN on preoperative CECT abdomen, and the remaining six individuals were diagnosed solely based on final histological examination. The tumor's median diameter was 5.6 cm (range, 4.1-7.9). The distal body and tail of pancreas was the most common location (63%), followed by the head (36%), and was managed with distal pancreatectomy with or without spleen preservation and Whipple's procedure, respectively. One patient developed grade III Clavien-Dindo complication. The average length of in-hospital stay was 8.27±2.72 days. None of the patients had recurrence on follow-up. Solid pseudopapillary neoplasm of the pancreas is often misdiagnosed preoperatively. Endoscopic ultrasound-guided FNA with IHC will be beneficial to diagnose it preoperatively especially in small-sized tumors with atypical features. Complete surgical resection with adequate margins without routine lymphadenectomy is curative in resectable tumors. Jayapal L, Kumar SR, Jebakumar GS, et al. Solid Pseudopapillary Neoplasm of the Pancreas: Unraveling Insights from a Single Institutional Study Emphasizing Preoperative Diagnosis of a Rare Tumor. Euroasian J Hepato-Gastroenterol 2023;13(2):50-54.

Organ-preserving enucleation in a case of solid pseudopapillary neoplasm of pancreas

Solid pseudopapillary neoplasm (SPN) is a rare pancreatic tumor affecting mostly females in the second or third decade of life. These tumors have a low malignant potential, and hence, surgical resection remains the main treatment. Large tumors may present with compressive symptoms such as abdominal pain or vomiting. Once resected completely, SPNs usually have an excellent long-term prognosis. We report a 16-year-old girl who presented with vomiting and abdominal pain localized to the right upper quadrant. Imaging showed an encapsulated tumor arising from the pancreatic head which prompted us to perform organ-preserving enucleation. Histopathology confirmed the tumor as an SPN.

S151 Characteristics and Outcomes of Solid Pseudopapillary Tumors of Pancreas: A US Multicenter Retrospective Study

S151 Characteristics and Outcomes of Solid Pseudopapillary Tumors of Pancreas: A US Multicenter Retrospective Study

Solid Pseudopapillary Tumor of Pancreas in a 14-year-old Adolescent Presenting With Melena: A Case Report

Background: The incidence of pancreatic neoplasms in infants and children is 1.8 cases per 1000000. Three of children’s most common primary pancreatic neoplasms are pancreatoblastoma, solid pseudopapillary neoplasm of the pancreas, and pancreatic endocrine neoplasms. Solid pseudopapillary neoplasm of the pancreas is a low-grade malignant tumor. Solid pseudopapillary neoplasm in children is presented with a palpable mass (60%), followed by abdominal pain (33.3%). Although duodenal invasion frequently occurs in patients with pancreatic cancer, massive gastrointestinal bleeding is seldom encountered. The most helpful imaging technique is the CT scan. Surgical resection is the treatment of choice for solid pseudopapillary neoplasms. Case Presentations: A 14 years old male adolescent was presented to our pediatric emergency department with fatigue, dizziness, fever, vomiting, and tachycardia. He had melena 5 days before admission. Crystalloids, pantoprazole, and packed red blood cells were administered to stabilize the patient. As the initial resuscitation measures stabilized the patient, endoscopic gastroduodenoscopy was performed, and a vascular lesion measuring 60×70 mm was noted in the second part of the duodenum. CT scan of the abdomen with intravenous and oral contrast showed a mass with solid and cystic components measuring 75×52 mm between the head of the pancreas and gallbladder origination from the head of the pancreas. The patient underwent Whipple surgery. The diagnosis of the pathologic evaluation was a solid pseudopapillary tumor of the pancreas. Conclusions: Most pediatric pseudopapillary tumors of the pancreas present with a palpable mass and abdominal pain, and gastrointestinal bleeding is a rare presentation not mentioned in previous case reports.

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

Cytological evaluation of spectrum of Hepatobiliary and Pancreatic mass lesions

Hepatobiliary and Pancreatic mass lesions are commonly occurring intra-abdominal space occupying lesions. Image guided FNAC enables exact localization of the lesion, assessment of nature and invasion and is a useful tool in the diagnosis of such lesions. The present study aims to evaluate the spectrum of hepatobiliary mass lesions diagnosed by Image guided FNAC. We have conducted a retrospective study in Department of Pathology, Gauhati Medical College & Hospital, Guwahati, for a period of 5 years, including patients of all ages and both sexes presenting with clinical and radiological suspicion of hepatic, biliary or pancreatic mass lesion. Under aseptic conditions USG guidance FNAC was done from the appropriate site and smears were examined. Out of the total 135 patients, there were 70 cases of Hepatic lesions (51.8%), followed by 54 cases taken from Gall bladder (GB) (40%), 5 cases from Common Bile Duct (CBD) (3.7%) and 6 cases (4.44%) from Pancreatic lesions. There were 51 cases of hepatic malignancies, 36 GB malignancies, 4 cases each of CBD and pancreatic malignancies. The most common Hepatic malignancies were Metastases followed by Hepatocellular carcinoma. The most common GB malignancy was Adenocarcinoma, followed by Cholangiocarcinoma. There was 1 case each of Solid Pseudopapillary Neoplasm of Pancreas, Acinar Cell carcinoma, Mucinous Adenocarcinoma and HCC. These findings were in concordance with our reference studies. Image guided FNAC has proved to be a versatile tool in provide an early and accurate diagnosis of hepatobiliary and pancreatic mass lesions.

Central Pancreatectomy for Solid Pseudopapillary Tumour of Pancreas in A 13-Year Girl.

Solid Pseudopapillary Tumour (SPT) is a very rare tumour of the pancreas. A13-year girl presented to us with the complaint of upper abdominal pain and non-bilious vomiting for 15 days. Preoperative diagnosis of SPT involving the body of the pancreas was made by CT scan and ultrasound- guided Trucut biopsy. A sparingly rare procedure of central pancreatectomy with distal pancreatico-jejunostomy was performed. This procedure offers excellent results in benign and low-grade malignant pancreatic neck and body tumours. In addition, it preserves functional elements of the pancreas and also eliminates the infective and haematological effects of splenectomy. Key Words: Solid pseudopapillary tumour, Central pancreatectomy, Pancreatic tumours.

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

BACKGROUNDSolid 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.AIMTo investigate the malignant behavior and tumor-associated macrophage (TAM) infiltration between different histopathologic features of SPT patients.METHODSTwenty-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.RESULTSAmong 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).CONCLUSIONSPT 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.

Diagnosis and treatment of solid pseudopapillary neoplasm of the pancreas in children: A report of 18 cases.

PurposeTo explore the incidence, imaging and treatment of solid pseudopapillary tumor of pancreas in children, and Summarize the experience of treatment.MethodsThe clinical data of 18 children with a solid pseudopapillary tumor of the pancreas treated in our hospital from January 2012 to June 2021 were analyzed retrospectively. The age range was 8–16 years old, the average age was 11.67 years old, and the median age was 11.5 years old, namely, three boys with an average age of 10 years old and 15 girls with an average age of 12 years old. In total, two cases were admitted to the hospital because of trauma, seven cases were found in physical examination, and nine cases were admitted with the abdominal pain as the main complaint. Enhanced CT examination was performed before operation, nuclear magnetic resonance examination and abdominal ultrasound examination were performed in some children, and plain CT scan was performed after operation.ResultsSolid pseudopapillary tumor of the pancreas can occur in all parts of the pancreas, especially in the body and tail of the pancreas. Among the 18 cases, SPN occurred in the head of pancreas in 5 cases (27.78%), the neck of pancreas in 2 cases (11.11%), and the body and tail of pancreas in 11 cases (61.11%). All the 18 children were treated by operation. among them, 4 cases underwent choledochal pancreatico duodenectomy (1 case), 4 cases underwent pancreaticoduodenectomy combined with splenectomy (3 cases), 6 cases underwent spleen-preserving pancreatectomy / tail pancreatectomy (1 case), 3 cases underwent enucleation of pancreatic tumor due to exogenous growth, 1 case underwent laparoscopic partial pancreatectomy and pancreaticoenterostomy. Laparotomy was performed in 12 cases and endoscopic surgery in 6 cases. Postoperative pathology confirmed solid pseudopapillary tumor of the pancreas. None of the patients received radiotherapy and chemotherapy after operation and were followed up for 6 months to 2 years. There was no recurrence, metastasis or pancreatic dysfunction.ConclusionSolid pseudopapillary tumor of pancreas in children is a rare, low-grade malignant solid tumor with no specific clinical manifestations and laboratory examinations. Preoperative diagnosis mainly depends on enhanced CT. Surgical resection of tumor is a reliable treatment, and the specific operation is mainly based on the experience of the chief surgeon, the location of the tumor and the invasion of surrounding tissue. At present, there is no evidence of the effectiveness of other treatment options, and surgical resection of the tumor has a good prognosis.

Nonhypovascular pancreatic ductal adenocarcinomas: CEUS imaging findings and differentiation from other types of solid pancreatic lesions.

To observe and assess the diagnostic value of contrast-enhanced ultrasound (CEUS) in patients with iso-/hypervascular solid pancreatic lesions. 70 pancreatic lesions (all confirmed by surgery or biopsy pathology) that manifested iso-/hyperenhancement on CEUS were retrospectively studied from January 2018 to January 2022, including 24 pancreatic ductal adenocarcinomas (PDAC), 15 mass-forming pancreatitis (MFP), 24 pancreatic neuroendocrine tumors (PNET) (14 hyper-PNETs, 10 iso-PNETs), and 7 solid pseudopapillary tumors of pancreas (SPTP). 65 pancreatic ductal adenocarcinomas (PDAC) that manifested hypoenhancement on CEUS were retrospectively studied from January 2020 to January 2022. CEUS patterns and the clinical and pathologic features were analyzed, and the diagnostic ability of CEUS for iso/hyperenhanced solid pancreatic lesions was assessed. Centripetal enhancement, heterogeneous enhancement, early washout, and hypoenhancement in the late phase mostly appeared in iso-/hyper-PDACs (p < 0.05). Heterogeneous enhancement in small lesions (< 3 cm) as the diagnostic criterion for iso-/hyper-PDACs had an accuracy of 74.3% and a specificity of 91.3%. Iso-PNETs more commonly had larger tumor sizes and more often showed heterogeneous enhancement than hyper-PNETs (p = 0.007, p = 0.035, respectively). The characteristics of the combination of isoenhancement, homogeneous enhancement, and synchronous wash-in/out for MFP had a high accuracy of 90%. Capsular enhancement with heterogeneous enhancement inside for SPTP had an accuracy of 97.1%. CEUS enhancement patterns have potentially great value in the differentiation of iso-/hyperenhanced pancreatic lesions.

Solid pseudopapillary neoplasm of pancreas presenting after bicycle handlebar injury: diagnostic pitfalls in a young female-A case report.

Introduction and importanceSolid pseudopapillary neoplasm (SPN) of pancreas is a rare neoplasm with very low malignant potential. It mainly affects females in third decade of life and has favourable prognosis.Case presentationWe report a case where a 17-years old girl presented with history of blunt abdominal trauma with handlebar of bicycle 6 months back, following which she developed abdominal pain and fullness in epigastrium. Keeping pancreatic pseudocyst as provisional diagnosis, she was investigated. The final diagnosis of SPN of pancreas came as a surprise.The mass, about 6x5cm, was excised and Roux-en-Y distal pancreatico-jejunostomy was done.Clinical discussionPatient showed no recurrence or metastasis till one year of follow-up. This is the first case report of SPN presenting after handlebar injury.ConclusionTimely and adequate resection provides good long-term survival.

Laparoscopic Parenchymal Sparing Multiple Metastatectomy Combined with Left Lateral Segmentectomy for Long Term Hepatic Recurrence from Resected Solid Pseudopapillary Tumor of Pancreas

Introduction: Solid pseudopapillary neoplasm(SPN) of the pancreas are rare tumors that occurs in young women. SPN can metastasizes in 5–15% of all cases and the common site is the liver. The management of metastatic tumors of SPN is unclear. We report a case of Laparoscopic Parenchymal Sparing Multiple Metastatectomy Combined with Left Lateral Segmentectomy for long term Hepatic Recurrence from Resected Solid Pseudopapillary tumor of Pancreas. Case/methods: A 26-year-old lady underwent distal pancreatectomy with splenectomy on 30/3/2015 for solid pseudopapillary tumor of pancreas. Patient was on routine follow up and a PET CT done on 14/10/2020 showed Multiple Bilobar Liver Metastasis in Segments 2/3, 6, 7, 8. Patient underwent of Laparoscopic Parenchymal Sparing Multiple Metastatectomy Combined with Left Lateral Segmentectomy on 22/10/2020. Intra operatively, metastasis was noted at segment 6,7,8 sub capsular. Intraoperative USG showed deep seated segment 2 metastasis for which Laparoscopic left lateral Segmentectomy was done. Results: Postoperative period was uneventful. Histopathology confirmed the 4 metastasis with adequate margins. She was discharged on post-operative day 2. Post 10 months of surgery patient is having a disease-free survival. Conclusion: Long term hepatic recurrence in SPN is rare. Aggressive management of Liver Metastasis ensures a long term disease free survival. In our case we were able to manage the multiple liver metastasis using a combination of Laparoscopic Parenchymal sparing hepatic metastatectomy and Left Lateral Segmentectomy to achieve a R0 resection and this reinforces the role of laparoscopic liver surgery even in such rare tumors.

Solid pseudopapillary neoplasm of pancreas in a 14-year-old girl

Solid pseudopapillary neoplasm (SPN) of the pancreas is an infrequent neoplasm that is known to have a low malignant potential and represents less than 3% of all exocrine tumors. Herein, we report a case of SPN of the pancreas in a 14-year-old girl who presented with a long history of dyspepsia and epigastric pain. CT revealed a sizeable well-defined cystic mass at the porta hepatis/pancreatic head mesenteric region. On exploration, showed a tumor in the head of the pancreas, which was completely resected. The final diagnosis was Solid pseudopapillary neoplasm of the pancreas. We highlight its pathological and surgical background as well.

English

BACKGROUND Solid pseudopapillary neoplasm (SPN) of pancreas is a rare epithelial tumour of low malignant potential. SPN accounts for less than 1 to 2 % of exocrine pancreatic tumours. It mainly affects women between the second and third decade of life, and its management is not well defined. The aim of this study was to report clinicopathological characteristics of SPN and its outcome. METHODS A retrospective study was conducted in a tertiary care centre from January 2015 to December 2019. All patients who were diagnosed and treated as SPN of pancreas in our institute were retrospectively reviewed. A data of the characteristics of these patients was developed, including age, gender, size, location of tumour, treatment, histopathological and immunohistochemical features. RESULTS Six patients were diagnosed as having SPN of pancreas, during the 5-year period. All 6 patients were female. Youngest age of occurrence was 15 years. Maximum age was 41 years. Average age was 25 years. All patients were symptomatic and the most common symptom was dull aching upper abdominal pain. Contrast enhanced computed tomography (CECT) was done for all patients. 3 patients had typical features of SPN. Endoscopic ultrasound (EUS) was done for 4 patients and EUS fine needle aspiration cytology (FNAC) was done for 3 patients. Patients were provided with procedure details and informed consent was taken. All patients were subjected to surgical treatment. Out of six patients, two underwent laparoscopic spleen preserving distal pancreatectomy, two patients underwent classical Whipple’s procedure and two patients had undergone median pancreatectomy. CONCLUSIONS SPN are rare neoplasms, typically affecting young females without clear histogenesis and with a malignant potential. Appearance from imaging studies can be adequate to guide surgical resection without pre-operative pathological assessment. But in unclear cases, EUS-FNAC with immunohistochemistry helps in establishing a pre-operative diagnosis. Surgical resection should be offered when feasible. Prognosis of SPN of the pancreas is good due to its favourable biological features, even in the presence of distal metastasis. KEYWORDS Solid Pseudopapillary Neoplasm (SPN)

Diagnostic Utility of LEF1 Immunostain in Cytology Specimens of Solid Pseudopapillary Neoplasm of Pancreas

Solid pseudopapillary neoplasm (SPN) of the pancreas is a rare neoplasm. Diagnosis of SPN requires an integrated approach with aid of radiology, biopsy, cytology, and immunohistochemical stains. Although morphological features in combination with nuclear positivity of β-catenin IHC have been the gold standard of SPN diagnosis, but overlapping morphology and immunohistochemical findings with other entities in differential diagnoses such as pancreatic neuroendocrine tumors and pancreatic ductal adenocarcinoma make the diagnosis of SPN difficult particularly in limited cytology specimens. Lymphoid enhancer-binding factor 1 (LEF1), a key player in the Wnt signaling pathway, has shown promising diagnostic utility in SPN in recent literatures. Methods: In this retrospective study, we evaluated the diagnostic utility of LEF1 IHC in SPN in cytology specimens. LEF1 IHC was performed and compared with β-catenin, synaptophysin, and chromogranin immunostains in 13 SPN and 23 pancreatic neuroendocrine tumors (PanNETs) cytology cases with retrievable cell blocks. Results: LEF1 was positive in 13 of 13 (100%) SPNs and was negative in all PanNETs (0%). Conclusion: LEF1 shows 100% sensitivity and specificity in cytology specimens for SPN and can be valuable immuno­stain in the diagnosis of SPN in cytology cell blocks.

Solid pseudopapillary tumour of pancreas: A rare tumour with good prognosis: A case report

Solid pseudopapillary neoplasm (SPN) of pancreas is a rare exocrine tumour of pancreas. It has low malignant potential with excellent prognosis and usually effects females in adolescent age group. We report a case of 40 year old female who presented with pain abdomen. Radiological investigations revealed a mass lesion in the pancreas. The diagnosis was confirmed on histopathological examination. Due to its low incidence, the clinical and pathological features of this tumour have not been extensively studied. It is important to be aware of its specific microscopic features, immunohistochemistry and differential diagnosis as timely surgical resection provides long- term survival.

Solid Pseudopapillary Neoplasm of the Pancreas - A rare entity with emphasis on the differential diagnosis.

Solid Pseudopapillary Neoplasm of Pancreas (SPNP) is a rare, low-grade malignant solid-cysticneoplasm with papillary architecture. It accounts for 2% to 3% of pancreatic neoplasms and 0.9% to2.7% of exocrine pancreatic neoplasms. It occurs almost exclusively in young women and has anexcellent postsurgical curative rate. Metastasis is rare although it may be locally aggressive. Thesolid pseudopapillary neoplasm of the pancreas pose a diagnostic challenge both clinically andradiologically as it has a nonspecific clinical presentation with vague radiologic features.Histopathological evaluation and immunohistochemistry remains the gold standard in reaching adefinitive diagnosis. Due to its low incidence, the clinical and pathologic features of SPNP have notbeen extensively studied. We report a case of a 32-year-old lady with solid pseudopapillaryneoplasm of the pancreas that was suspected on abdominal CECT as a well-defined mass in theampullary-periampullary region abutting head of the pancreas and confirmed on histopathologicalevaluation with immunohistochemistry.

Expression of SOX-11 and TFE3 in solid-pseudopapillary tumor of pancreas and its clinical significance

Objective: To investigate the expression status and diagnostic value of SRY related high mobility group box 11 (SOX-11) and transcription factor E-3 (TFE3) in solid pseudopapillary tumors of pancreas (SPTPs). Methods: Thirty-eight cases of SPTPs, 36 cases of well-differentiated pancreatic neuroendocrine tumors (PanNETs) and six cases of pancreatic acinar cell carcinomas (PACCs) were collected at the Affiliated Drum Tower Hospital of Nanjing University Medical School from 2012 to 2019. The expression of SOX-11, TFE3 and β-catenin was detected by immunohistochemistry, and the TFE3 gene status was detected by FISH in 18 cases of SPTPs. Results: Among the 38 SPTP patients, 29 were female and 9 were male, with a mean age of 50 years; among 36 PanNET patients, 32 were female and 4 were male, with a mean age of 39 years; for the six PACC patients, four were male and two were female, with a mean age of 60 years. β-catenin was positive in all 38 SPTPs, but was negative in all 36 PanNETs and 5/6 PACCs. SOX-11 was positive in 35/38 (92.1%) of SPTPs, but was negative in all 36 PanNETs and 6 PACCs. TFE3 was positive in 36/38 (94.7%) of SPTPs, but was negative in all 36 PanNETs and 6 PACCs. Among these three tumors, the specificity and sensitivity of β-catenin were 97.6% and 100.0%, the specificity and sensitivity of SOX-11 were 92.1% and 100.0%, the specificity and sensitivity of TFE3 were 94.7% and 100.0%, respectively. There was a significant difference of the expression status of all three markers in SPTPs compared with PanNETs and PACCs (P<0.01). The results of SOX-11 and TFE3 immunostaining showed high consistency (Kappa>0.6). No gene rearrangement (0/18) of TFE3 was found in SPTPs. Conclusion: SOX-11 and TFE3 are highly expressed in SPTPs, and their specificity in the differential diagnosis of SPTPs is better than that of β-catenin.

S1448 Solid Pseudopapillary Neoplasm of Pancreas: A Rare Pancreatic Malignancy

INTRODUCTION: Solid pseudopapillary neoplasm of pancreas (SPN) is one of the rarest types of pancreatic tumors, <2% of all pancreatic malignancies, also known as a Hamoudi or Frantz tumor. SPNs usually presents asymptomatically in females in the third decade of life and found incidentally on radiological imaging. CASE DESCRIPTION/METHODS: Here, we present three proven SPN cases by histology. Case 1: A 42-year-old Hispanic female with no past medical history who presented with 1-day history of cough, incidentally found to have a 3 cm hypodense lesion in the pancreatic body on CT scan. Endoscopic ultrasound (EUS) with biopsy was performed and immunohistochemical (IHC) studies found tumor cells to be positive for B-catenin, progesterone receptor and neuron specific enolase, consistent with a diagnosis of SPN. Case 2: A 48-year-old African American female with a past medical history of GERD presented with intermittent right upper quadrant abdominal pain. CT demonstrated a large solid, periportal hepatic mass (19 × 17 × 19 cm) as well as a multi-cystic mass (14 × 7 × 10 cm) arising from the distal pancreatic tail. Exploratory laparotomy was performed and pathology showed a uniform population of small cells with pseudopapillary structures with IHC profile supporting the diagnosis of metastatic SPN. Case 3: A 31-year-old Hispanic female with no significant past medical history presented with 2 months history of post-prandial epigastric abdominal pain found to have a hypodense mass (4 × 4 cm) in the distal pancreatic tail. Surgical resection was performed. Pathology findings of neoplastic tumor cells with occasional groves, collections of histiocytes, and hyaline globules with positive IHC staining for vimentin, B-catenin and progesterone receptor, consistent with diagnosis of SPN. DISCUSSION: On imaging, SPN tumors typically appear as a well-defined mass, with a thick, enhancing capsule, often with peripheral or central calcification. Although most often solid, they can demonstrate variable internal hemorrhagic or cystic components. EUS is the modality of choice for further imaging and definitive diagnosis, as biopsy can be performed. Here, we describe three cases including a unique and rare case of metastatic SPN. Clinicians should be aware of this relatively rare pancreatic lesion as its presentation may be insidious and early imaging is key. Once diagnosed, physicians should pursue multidisciplinary discussions with early surgical management even in metastatic disease, as outcomes are excellent with a favorable prognosis.Figure 1.: MR Imaging - SPN.Figure 2.: Endoscopic US - SPN.Figure 3.: Histopathology - SPN.