EUS-guided ethanol ablation in patients with solid pseudopapillary neoplasm: a four-case series.

Solid pseudopapillary tumor (SPT) of the pancreas is a rare tumor, sometimes asymptomatic, mainly affecting young females. It has a low malignant potential, and its complete surgical resection with free margins is the treatment of choice, offering an excellent prognosis. We present the case of a 43-year-old healthy female who was referred to the surgery department for a large abdominal mass found in the abdominal ultrasound. In the course of the study of this mass, an abdominal CT with fine needle aspiration (FNA) was performed, revealing a solid pseudopapillary tumor of the tail of the pancreas. A laparotomic distal pancreatectomy with total splenectomy was performed. A grade B pancreatic fistula occurred on the third postoperative day, and she was released on postoperative day 11. Histopathology study revealed a 10 cm solid pseudopapillary tumor of the pancreas, with cavities filled with hematic content and limited by a partially calcified capsule. SPT is a rare and indolent tumor. Prognosis is highly favorable after an adequate surgical resection, hence the importance of a precise preoperative diagnosis. Therefore, it is important to choose the most appropriate surgical strategy for each patient.

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

Solid pseudopapillary neoplasm of the pancreas (SPNP) is a rare primary neoplasm with distinct clinicopathological features. The tumor most commonly occurs inyounger (premenopausal) women and is typified by low malignant potential and an excellent overall prognosis. A retrospective search over 20 years at two referral tertiary care institutions (King Faisal Hospital and Research Center andKing Abdulaziz University Hospital, Jeddah, Kingdom of Saudi Arabia)revealed 12 female patients diagnosed with SPNPs. The reslts of ancillary studies performed at the time of diagnosis were also reviewed and placed in the context of current recommendations. The clinical and pathological findings were reviewed. All patients were females, aged 18 to 30 years. Eight patients presented with abdominal pain, of which two experienced significant weight loss, and four presented with abdominal mass/discomfort. The tumor size ranged from 1.5 and 15 cm. Two cases were initially diagnosed as neuroendocrine tumors (NETs). One of the cases presented as a multifocal disease. All patients were treated surgically with a follow-up period between one and 11 years. Only one patient presented with peritoneal metastasis after seven years of follow-up, but generally, all are doing well. We have analyzed 12 SPNP cases in our population over 20 years (2001-2021) in this study. In brief, SPNP is a low-grade malignant potential tumor. Even though SPNP is a recognized entity, diagnostic challenges can ariseparticularly in the setting of limited sampling.Pathologists must be aware of the classic morphological features of SPNP and the characteristic profile of immunohistochemistryand be able to differentiate SPNP from other mimickers, especially well-differentiated NETs of the pancreas, andultimately to avoid misdiagnosis and unnecessary oncologic treatment. Adequate surgical resection with negative margins is associated with an excellent outcome.

INTRODUCTION: Solid pseudopapillary tumor (SPT) of the pancreas is a rare neoplasm. It accounts for 1% of all tumors of the pancreas. It is a benign tumor with a low malignant potential and a favorable prognosis. It typically presents in young women, and on imaging it can be seen as a large encapsulated mass with solid and cystic components. The female to male predominance is 10:1. The following case shows a typical presentation of SPT in a male patient. CASE DESCRIPTION/METHODS: A 25-year-old man from Guatemala with no medical history presented with epigastric pain, nausea, vomiting, weight loss and poor oral intake. The abdominal pain was stabbing, intermittent, and radiating to the back. Family, social and surgical history were negative. Vitals were normal. On physical examination he appeared dehydrated with epigastric tenderness and a palpable left upper quadrant mass. Labs showed normal chemistry, liver function tests, lipase, CA 19-9, CEA and CA 125. Complete blood count showed an elevated white blood cell count of 23,000. Computerized tomography revealed a 7.6 × 10 × 8 cm heterogenous mass in the distal body and tail of the pancreas, with solid, and cystic components and calcifications. The patient underwent esophagogastroduodenoscopy (EGD) and endoscopic ultrasound with fine needle aspiration (FNA) of the mass. EGD showed a small non-bleeding duodenal ulcer. Pathology on the FNA revealed SPT. The patient underwent a distal pancreatectomy and splenectomy with negative margins, and a normal spleen. Lymph nodes were negative for malignancy. The patient did well post operatively with resolution of his symptoms. DISCUSSION: SPT is a rare tumor predominately seen in females. In men it tends to occur at an older age with an aggressive behavior. The origin of SPT is not clear. 60% of SPT are found in the body and tail of the pancreas. SPT is primarily benign but about 15% exhibit malignant features such as metastasis or invasion of adjacent structures. The most common sites of metastasis are the liver and peritoneum. Typical presentation is a palpable abdominal mass with symptoms of abdominal pain, nausea, vomiting and early satiety. The histopathology of SPT reveals solid nests of poorly cohesive cells resulting in a pseudopapillary architecture with positive nuclear staining for beta catenin, positive immunostaining for CD10, CD56, vimentin; they are negative for chromogranin. Cystic fluid is often bloody with low CEA and low amylase. Surgical resection is curative. The five-year survival rate is 95%.

Solid pseudopapillary tumor of the pancreas: a case series of 26 consecutive patients

Solid pseudopapillary neoplasms(SPNs) of the pancreas are rare tumors with low malignant potential that usually occur in young girls and women. This study investigated the treatment experiences and outcomes after surgery for pediatric SPNs in our institutions as pediatric case series ofsolid SPNs are few, and long-term follow-up is also limited. We retrospectively reviewed the outcomes of nine patients diagnosed with SPNs who underwent surgery in our three hospitals (The Jikei University Hospital, The Jikei University Kashiwa Hospital, and Kawaguchi Municipal Medical Center)between 2001 and 2023. All nine patients who underwent surgery were girls. Their ages ranged from 8 to 15 years (median: 10years). The location of the tumor was at the pancreatic head, body, and tail in five, one, and three patients, respectively. Enucleation, pancreaticoduodenectomy, and laparoscopic distal pancreatectomy (LDP) were performed in four, two, and three patients, respectively. Regarding postoperative complications, a pancreatic fistula was detected in six patients, with three and three patients having grades A and B fistulas, respectively; two patients required percutaneous drainage, and one patient required endoscopic ultrasonography (EUS)-guided transgastric drainage. The follow-up period rangedfrom six to 261 months (median: 97 months). At the final follow-up, all nine patients were alive without recurrence. SPNs of the pancreas are incidentally diagnosed by diagnostic workups following trauma in children more frequently compared to adults. Additionally, the tumor resection by minimally invasive approaches, such as enucleation, or laparoscopic procedures results in a good prognosis in some cases.

PurposeSolid-pseudopapillary neoplasm (SPN) of the pancreas, a rare tumor, has low malignant potential. However, some patients develop metastasis and recurrence after resection, with aggressive biological behaviors. This study aimed to explore the features and risk factors associated with the aggressive biological behaviors of SPNs.Patients and MethodsWe retrospectively analyzed the clinicopathological and long-term follow-up data of 63 patients diagnosed with SPN at the First Affiliated Hospital of Bengbu Medical College between January 2007 and February 2019.ResultsSixty-three patients presented atypical clinical symptoms. The median tumor size was 7.0 cm (range, 2.4–17 cm), and imaging features were solid and cystic or solid tumors with uneven density. Frequent and diffuse nuclear LEF1 protein expression (94.2%) was observed with LEF1 having a higher sensitivity and specificity. Overall survival significantly correlated with tumor size, Ki-67 index, and lymph node metastasis (P < 0.05).ConclusionSPN is a rare low-grade malignancy with a specific pseudopapillary structure. LEF1 is an effective biomarker of SPNs. Although SPNs generally display indolent biological behavior, a large tumor size, high proliferation index, and lymph node metastasis may be risk factors for the aggressive behavior and poor prognosis of SPN.

Solid-pseudopapillary neoplasm of pancreas with long delayed liver metastasis

A solid pseudopapillary tumor (SPT) of the pancreas is a rare neoplasm that mainly occurs in young women. We herein report the case of a small SPT arising from the head of the pancreas in an asymptomatic 32-year-old man, plus a literature review of this tumor. A 32-year-old man was admitted to our department at Kumamoto University Hospital for the evaluation of a pancreatic mass. The tumor had central necrosis, which was poorly perfused on contrast-enhanced computed tomography (CT) and which had a high intensity on T2-weighted magnetic resonance imaging (MRI). Histology revealed the lesion to be a solid pseudopapillary tumor of the pancreas, with the characteristic pseudopapilla formation and central degeneration. However, no capsule formation was observed. The tumor was positive for CD56, CD10, alpha1-antitrypsin, alpha1-antichymotrypsin, beta-catenin, and progesterone receptor. However, the tumor was negative for pancreatic hormones, chromogranin-A, carcinoembryonic antigen, and carbohydrate antigen 19-9. We diagnosed the patient to have an SPT based on these histological findings. Small-sized solid pseudopapillary tumors of the pancreas are being increasingly recognized because of the recent advances in CT and MRI. We should also consider SPT even if it occurs in a male when the tumor contains necrosis-suspected areas which are poorly perfused on contrast-enhanced CT with a high intensity on T2-weighted MRI.

Solid-Pseudopapillary Tumor (SPT) of the pancreas is considered to be a rare pancreatic tumor that occurs in young females. Most SPTs are considered to be benign. However, the natural course of history has not yet been clarified. We reported a case of 25-year-old women with a patent history of splenectomy for hemolytic anemia, who presented intermittent abdominal pain and vomiting of 6 months ago. Physical examination revealed a hard mass of 8 × 5 cm in the left upper quadrant of the abdomen. Routine laboratory tests were within the normal range. CT-Scan showed an 8 × 6 cm lesion with irregular low density in the body and the tail of the pancreas. MRI showed a mass of 8 cm, surrounded by a capsule, with the irregular intensity both in T1 and T2 enhanced-images. Distal pancreatictomy was done as a definitive treatment via a Bi sub costal incision. Histopathological examination confirmed the diagnosis of SPT. There was no vascular invasion or other malignant features. The resection margins are free of lesions. The post operative course was uneventful. During post operative follow-up of 24 months, there is no sign of recurrence on CT-scan. SPT is a rare condition with a low potential for malignancy and favorable prognosis. Surgical resection is generally curative. Characteristic CT and MRI scans combined with age and sex profile should be sufficient for the diagnosis and the decision to operate.

Solid pseudopapillary neoplasm (SPN) of the pancreas is a rare pancreatic tumor with low malignant potential. It occurs characteristically more often in young women. Radiological and pathological studies have revealed that the tumor is quite different from other pancreatic tumors. Limited information is available in the literature reporting their accumulation of fluorine-18 fluorodeoxyglucose (18F-FDG) in positron emission tomography/computed tomography (PET/CT). Here, we report a case of pancreatic SPN imaged with contrast-enhanced FDG PET/CT. A percutaneous fine needle aspiration from the metabolically active lesion revealed SPN, and it was confirmed with histopathological results. Recurrence or metastasis was not found after 7 months of follow-up.

Solid pseudopapillary epithelial neoplasm (SPEN) of the pancreas is a rare tumor that accounts for less than 2% of exocrine pancreatic tumors affecting non-Caucasian females in the second and third decades of life. We describe a rare case of SPEN presenting in a Caucasian pediatric patient. A 12-year-old Caucasian female with no past medical history presented with three weeks of intermittent right upper quadrant and epigastric abdominal pain associated with nausea and vomiting. She was found to have a leukocytosis, elevated amylase, lipase and alkaline phosphatase initially concerning for pancreatitis. Non-contrast CT scan demonstrated a well-circumscribed lesion and a mildly dilated gallbladder with mass effect causing gastric outlet obstruction. Contrast MRI demonstrated a 7.8 x 7.5 cm encapsulated mass arising from the pancreatic head with prominent solid, cystic and hemorrhagic components with an enhancing capsule. Endoscopic ultrasound (EUS) was performed demonstrating an 8.1 x 6.8 cm mass in the peripancreatic-periduodenal region and EUS-guided fine needle biopsy (FNB) was performed using a 19-gauge core needle with a through-the-needle Moray biopsy forceps. Pancreatic FNB showed normal pancreatic acini among blood and degenerative cells. Malignancy was ruled out and biopsy findings were consistent with SPEN. She underwent pancreaticoduodenectomy and cholecystectomy. Her post-operative course was uneventful and she was discharged home. Pathology of the pancreatic mass showed a solid and pseudopapillary neoplasm of the pancreas with extensive necrosis (90%) and benign pancreatic tissue with focal chronic inflammation. SPEN of the pancreas may present with vague abdominal pain and/or a gradually enlarging abdominal mass. These neoplasms are typically large at the time of diagnosis with an average size of 8 cm. The tumor often presents in the pancreatic tail with varying degrees of hemorrhage, cystic changes, or necrosis. In our patient, the mass arose from the head of the pancreas. Generally, SPEN are rare tumors with low malignant potential and complete surgical resection is typically curative. EUS-guided fine needle biopsy helps in establishing tissue diagnosis and helps to facilitate a care plan for surgical resection.Figure: Contrast MRI with a 7.8 x 7.5 cm encapsulated mass with prominent solid, cystic and hemorrhagic components with an enhancing capsule.Figure: EUS with a 8.1 x 6.8 cm mass in the peripancreatic-periduodenal region.

Management of a solid pseudopapillary tumor of the pancreas with liver metastases

Solid pseudopapillary tumors (SPT) are rare, unique pancreatic tumors with benign entity and low malignant potential. Limited information is available in the literature reporting their accumulation of fluorine-18 fluoro deoxyglucose ((18)F-FDG) using positron emission tomography/computed tomography (PET/CT). The aim of this retrospective study was to define t he uptake-accumulation of (18)F-FDG PET/CT in a comparatively large cohort of SPT, and to compare their uptake with the uptake of (18)F-FDG in pancreatic ductal adenocarcinomas (PAC) and neuroendocrine tumors (PNET). Between June 2007 and January 2013, 18 pathologically proven SPT were identified from the total of patients studied by PET/CT in our Center, including 13 women and 5 men, aging from 23 to 56 years old (mean age, 38.5 years). Malignant SPT was histologically classified using the WHO criteria. Eighty-six PAC patients and 28 PNET patients were also identified and included in this study for comparison. Positron emission tomography results were considered as positive if focal accumulation of (18)F-FDG exceeded the surrounding normal pancreatic tissue. Regions of interest were drawn on the pancreatic lesions, and the maximal standardized uptake values (SUVmax values) were calculated. The mean values of SUVmax were compared with independent-samples t test or with the nonparametric Mann-Whitney U method. Correlation of SUVmax values and tumor size were analyzed in cases of SPT. Receiver operating characteristics (ROC curve) were used to study the efficiency of SUV values for the differential diagnosis between SPT versus (vs) PAC and SPT vs PNET. A value of P<0.05 was considered statistically significant. All SPT cases were (18)F-FDG-PET positive, with SUVmax values ranging from 3.5-18.3. The SUVmax values of SPT had poor correlation with tumor size, and no significant difference by gender and age. Areas under the curve ROC were 0.619 and 0.526, respectively for the differentiation of SPT from PNET and PAC tumors. Five SPT tumors were malignant, and exhibited relatively low (18)F-FDG uptake (SUVmax range, 3.0-4.5) except a tumor after recurrence (SUVmax 17.7). Images of CT were of low dose and thus were not evaluated. In conclusion, our results suggest that SPT benign or malignant are consistently hyperaccumulating (18)F-FDG above SUVmax 3. Differentiation from PAC and PNET if only based on the higher SUVmax values was not possible but if based on lower SUVmax, of ≤2.6 (in 14%) and ≤2.5 (in 21,4%) of PAC and PNET, respectively, these pancreatic tumors could be differentiated from SPT.

Early Diagnosis of a Pediatric Pancreatic Tumor by Abdominal Physical Examination: A Case Report.