Frantz tumor with asymptomatic microscopic hematuria: Case report.

Purpose:Solid pseudopapillary neoplasms (SPN) are rare pancreatic cystic neoplasms with low malignant potential that tend to occur in young women. Due to the rarity of this disease, there are few large case series in the literature, and the exact pathophysiology remains unknown. In this article, we aim to share our institutional experience.Methods:Retrospective clinical data collection and analysis was performed on all patients with a diagnosis of SPN at the University of Colorado Hospital and Children's Hospital of Colorado (n = 28).Results:Twenty-eight patients were diagnosed with SPN during the study period. The median age was 21.5 years, and the majority of patients were female (89.3%) and Caucasian (60.7%). Six patients were diagnosed incidentally (21.4%). The majority of tumors were in the pancreatic tail (46.4%), and most underwent distal pancreatectomy (64.3%). The mean tumor size was 5.4 cm, and R0 resection was achieved in 25 patients (89.3%). Ten patients underwent laparoscopic resection (35.7%). The median hospital length of stay was 8.5 days, and postoperative complication rate was 39.3%. Median follow-up was 41 months, with 78.6% of patients alive without evidence of disease, while 2 patients were lost to follow-up. Two patients developed recurrence/metastases, which were resected; both are alive without evidence of disease.Conclusion:SPN are rare pancreatic tumors diagnosed most frequently in young women. Surgical resection is the mainstay of treatment, and outcomes are excellent if complete resection is achieved. Predictors of malignant disease are inconsistent in current literature. Considerations should be made for a minimally invasive approach in patients with SPN. Multidisciplinary clinics may be helpful in the diagnosis, management, and surveillance of pancreatic cystic lesions, with major potential for the advanced practitioner role.

IntroductionSolid pseudopapillary neoplasm of the pancreas is a rare neoplasm that has been reported to account for between 0.17% and 2.7% of all non-endocrine tumors of the pancreas. It is usually seen in young women. Because solid pseudopapillary neoplasms are rarely aggressive and have low-grade malignant potential and an excellent prognosis after complete resection, it is an ideal pancreatic tumor for treatment by minimally invasive surgery. Therefore, making an accurate pre-operative diagnosis is very important.Case presentationA 24-year-old Japanese man who had been found to have mild transaminase elevations at a medical check-up visited our hospital for further examination. Abdominal computed tomography showed a 40mm-diameter tumor in the pancreatic tail and mild fatty liver. He was admitted to our hospital for additional examination. The abdominal contrast-enhanced computed tomography scan taken at our institution showed an increasingly enhanced mass of 40mm diameter in the pancreatic tail. Ultrasonography showed a low-level echoic mass of 35mm diameter in the pancreatic tail. T1-weighted magnetic resonance imaging showed low signal intensity in the tail of the pancreas. T2-weighted magnetic resonance imaging showed high signal intensity there. Diffusion magnetic resonance imaging showed high signal intensity. An endoscopic ultrasound yielded the same results as the abdominal ultrasonogram. In addition, [18F]-fluorodeoxyglucose positron emission tomography/computed tomography showed abnormal accumulation (maximum standardized uptake value, 6.53). This finding raised our suspicion of a pancreatic malignant tumor. However, the patient could not be confidently diagnosed solely on the basis of imaging. Endoscopic ultrasound-guided fine-needle aspiration was performed, which led us to a diagnosis of solid pseudopapillary neoplasm. On that basis, we performed minimally invasive surgery (spleen-preserving laparoscopic distal pancreatectomy).ConclusionAtypical solid pseudopapillary neoplasm without cysts should be considered when diagnosing pancreatic tumors. A definitive pre-operative diagnosis of solid pseudopapillary neoplasm made on the basis of endoscopic ultrasound-guided fine-needle aspiration can guide the surgical approach used.

Les tumeurs pseudopapillaires et solides du pancréas (TPPS) sont des tumeurs épithéliales rares. Dans la plupart des cas, il s’agit de tumeurs survenant chez la femme jeune dans la deuxième ou la troisième décennie de la vie. La survie après résection primaire approche 90% à 5 ans. Nous rapportons le cas d’une jeune patiente de la vingtaine qui présente une tumeur pseudopapillaire et solide du pancréas découverte devant des douleurs abdominales sans perturbations des bilans biologiques. La tomodensitométrie (TDM), l'imagerie par résonance magnétique (IRM) et l'échographie endoscopique ont révélé une masse bien limitée se développant au dépend de l'isthme pancréatique. L'exérèse complète de la tumeur a été réalisée. L’examen anatomopathologique confirmait le diagnostic de tumeur pseudopapillaire et solide du pancréas. En conclusion, les tumeurs pseudopapillaires et solides du pancréas doivent être évoquées comme un des diagnostics différentiels de toute masse pancréatique en particulier chez les jeunes femmes. L'exérèse chirurgicale procure un bon pronostic.

Background: Solid pseudopapillary tumour (SPT) of the pancreas is a relatively rare entity which most commonly occurs in young women. In this paper we report our clinical experience together with the current knowledge on the diagnostics, treatment and prognosis of this rare tumour. Methods: We reviewed hospital records of patients diagnosed with a solid pseudopapillary tumour of the pancreas between January 2002 and December 2011 at the Department of Surgery, University Hospital Hradec Kralove, Czech Republic. Clinical, operative, pathological data were obtained on all the patients. Results: Over the 10-year period of the study we performed 181 planned pancreatic resections in our department. Overall, the 30-day postoperative mortality rate in this series of patients was 2.2%. SPT was diagnosed in 4 cases. All the patients were women and the average age was 34 years. Preoperative endosonography with biopsy sample was performed in all the patients and the diagnosis of SPT was known in all the patients before the surgical procedure. Conclusions: The current knowledge of SPT is based only on case reports and small series. It typically occurs in young women and therefore the presence of a large pancreatic mass in a young woman may suggest a diagnosis of SPT. SPT has a low malignant potential and the prognosis is excellent following complete surgical resection in the majority of the cases.

Our aim was to summarize our experience with the diagnosis and surgical treatment of solid pseudopapillary neoplasm (SPN) of the pancreas to provide a reference for the management of this rare condition. We collected and analyzed retrospective data on the clinical presentation, laboratory investigations, radiologic imaging, pathology and operative details of patients with SPN of the pancreas diagnosed between February 2001 and December 2009. In all, 23 of 24 patients were women, and the mean age of all patients was 31 years. The most common clinical presentation was vague abdominal pain. Abdominal imaging showed solid or solid cystic masses in the pancreas, mostly in the tail or head of the gland. All patients were treated surgically. There were no postoperative deaths. After follow-up ranging from 4 to 109 months (median 68 mo), 20 of 22 patients who underwent curative resection were alive with no evidence of disease recurrence. Of the 2 patients with R1 resections, 1 died 42 months after surgery, whereas the other underwent a second operation and was alive after 36 months' follow-up. Solid pseudopapillary neoplasm of the pancreas is a relatively indolent tumour. The initial diagnosis of SPN of the pancreas is suggested by radiologic imaging findings but should be considered in the context of clinical and histopathologic characteristics. We advocate for complete surgical resection once SPN is diagnosed.

PurposeTo investigate the diagnosis, surgical treatment, and pathology of solid pseudopapillary tumors of the pancreas in our institution.Patients and MethodsWe retrospectively analyzed the demographic details, clinical features, imaging findings, and pathological findings of 87 patients with a confirmed diagnosis of solid pseudopapillary tumors of the pancreas (SPTP) and underwent surgery in Tongji Hospital of Tongji Medical College, over a period of 8 years from 2011 to 2018.ResultsOur study involved a total of 87 patients (16 males and 71 females) with a mean age of 31.3±13.1 years (range: 10–61 years). The main compliant was abdominal pain or discomfort (n=49) and the median tumor size was 58.6±31.7 mm (range: 16–156mm). Tumors were located in the head (27 patients, 31%), the neck (13 patients, 15%), and the body and tail (47 patients, 54%). There were no significant differences between the patients in terms of sex, age, or tumor location. Partial pancreatectomy was performed in 79 patients, enucleation in six patients, and total pancreatectomy in two patients. R0 resection was achieved in 86 patients. The postoperative morbidity was 36.8%, and the main complication was pancreatic fistula. Pathological examination and immunohistochemical markers were used to provide a final diagnosis. The main follow-up period was 46 months (range: 13–97 months). At the end of the follow-up period, 86 patients were alive and had not experienced recurrence; one patient was lost to follow-up.ConclusionThe accurate diagnosis of SPTP is vital. Our data showed that surgical resection is safe and associated with low morbidity and mortality rates. Pathological findings can play an important role in diagnosis and long-term survival.

Solid pseudopapillary tumors of the pancreas are rare, low-grade, epithelial neoplasms that are usually discovered incidentally in young women. Distinguishing solid pseudopapillary tumors from other pancreatic tumors, especially pancreatic endocrine tumors, can be challenging. The role of endoscopic ultrasound-guided fine-needle aspiration (EUS-FNA) in this context remains unclear. The purpose of this study was to describe the endoscopic ultrasound features of solid pseudopapillary tumors and the role of EUS-FNA in the preoperative diagnosis of these tumors. Patients from five tertiary referral centers with surgically confirmed solid pseudopapillary tumors who had undergone preoperative EUS-FNA were included. The endoscopic ultrasound findings, cytologic descriptions, immunostaining results, operative records, surgical pathology, and results of the most recent clinical follow-up were reviewed. A total of 28 patients were identified (four men [14 %], 24 women [86 %], mean age +/- standard deviation [SD] 35 +/- 10 years). Solid pseudopapillary tumors had been found as incidental findings on cross-sectional imaging in 50 % of cases. The mean tumor size +/- SD was 42 +/- 19.5 mm and the majority were located in the pancreatic body and tail. The endoscopic ultrasound report described a well-defined, echo-poor mass in 86 %; the tumors were solid in 14 patients (50 %), mixed solid and cystic in 11 patients (39 %), and cystic in three patients (11 %). A preoperative diagnosis of solid pseudopapillary tumor was made in 21 patients (75 %) on the basis of EUS-FNA cytology. Surgical resection was performed in all cases. Laparoscopic resection was performed in eight of these patients (29 %). A solid pseudopapillary tumor should be included in the differential diagnosis of any well-demarcated, echo-poor, solid or mixed solid/cystic pancreatic lesion seen during endoscopic ultrasound, particularly in young women. The diagnostic accuracy of EUS-FNA for solid pseudopapillary tumors was 75 % in this study. A definitive preoperative diagnosis can guide the surgical approach in selected cases.

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.

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 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.

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 neoplasms of the pancreas (SPNP) are rare pancreatic tumors that occur predominantly in young women, with very few cases reported in men. While the origin of the tumor may be unclear, it is characterized by a distinct histological appearance and a clinical course highlighting its low malignant potential. SPNP have an excellent prognosis and are potentially curable provided they are managed appropriately by complete surgical resection. In the rare instances where metastatic disease is encountered, surgical debulking has been shown to prolong survival. The role of chemotherapy and radiation therapy in the management of SPNP is still controversial. We report here on an unusual occurrence of SPNP in the area of the head of the pancreas in a 12-year-old female treated by pancreatico-duodenectomy, together with a review of the literature.

Solid pseudopapillary tumor (SPT) is a rare exocrine pancreatic neoplasm that accounts for 2-3% of pancreatic neoplasms. We present a unique case of a 20-year-old female that presented with pyelonephritis plus weight loss and was found to have SPT of the pancreas. A 20-year-old Jordanian female with history of type I diabetes mellitus presented to the ED with nausea, vomiting, dysuria, left sided flank pain, and a subjective 20-pound weight loss over the past 3 months. On physical exam, left sided CVA tenderness was elicited, but no palpable abdominal masses were found. Urinalysis and laboratory findings were consistent with pyelonephritis. Patient underwent CT abdomen/pelvis to evaluate for possible nephrolithiasis. Imaging was negative for nephrolithiasis but revealed a 7.5 x 7 cm mass in the pancreatic head with mass effect; suggestive of SPT of the pancreas or GIST tumor. GI recommended outpatient follow-up after resolution of the infection. Upon follow-up, EUS-FNA was performed and revealed an epithelial neoplasm, consistent with SPT of the pancreas. The tumor was compressing the gastric antrum and duodenal bulb causing partial gastric outlet obstruction. Surgery then performed a pylorus preserving Whipple resection. Patient recovered well post-op and was discharged home. SPT of the pancreas is a rare tumor that accounts for less than 3% of all pancreatic tumors. There has been a 7-fold increase in the incidence of SPTs since the year 2000, likely attributed to improved imaging and access to EUS. SPT predominantly affects younger females and is typically found in the pancreatic head or tail. The most common presenting signs are upper abdominal pain, palpable abdominal mass, or no symptoms altogether. In our case, the only presenting sign associated with SPT was weight loss, which is extremely rare. Diagnosis of SPT is made through a combination of imaging and pathologic findings. CT scan is the most common initial imaging modality followed by confirmation with EUS guided FNA. Overall, patients have a favorable outcome with a 5-year survival of 94-97% following surgical resection and have a recurrence rate less than 5%.1417_A.tif Figure 1: Non-contrast abdominal CT scan identifying SPT and duodenal compression1417_B.tif Figure 2: EUS identifying portal vein confluence1417_C.tif Figure 3: EUS identifying SPT and pancreatic bodyThis case is a unique presentation of SPT involving a patient with pyelonephritis and weight loss secondary to gastric outlet obstruction. Physicians should consider this neoplasm as a differential among patients, specifically females 20-30 years old, that present with unexplained weight loss and abdominal pain.

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 of the pancreas is a rare entity with low malignant potential and excellent overall prognosis. It has nonspecific clinical presentations such as abdominal pain and nausea, with vague radiologic features. Histologic features of this neoplasm are usually specific. The tumor shows minimally cohesive, uniform, monotonous cells lining delicate capillary-sized blood vessels, described as pseudopapillary architecture. Other features including hyaline globules, cytoplasmic vacuoles, and nuclear grooving are frequently present. Use of a select panel of immunostains always helps pathologists to differentiate this tumor from other circumscribed tumors of the pancreas. Recently, β-catenin, CD10, and E-cadherin have been shown to be very important in the diagnosis of solid pseudopapillary neoplasm. Nuclear staining of tumor cells by β-catenin and membranous presentation of CD10 is seen in almost 100% of cases. Tumor cells can be partially positive for synaptophysin and chromogranin. This tumor has a low malignant potential, and definite treatment is surgical resection.