Case Of Solid Pseudopapillary Tumor Research Articles

Synchronous solid pseudopapillary neoplasm and intraductal papillary mucinous neoplasm of the pancreas: Report of a case

Solid pseudopapillary neoplasm (SPN) of the pancreas, most commonly found in young female subjects, is a rare neoplasm with low potential for malignancy. We report an unusual case of a 66-year-old male patient who had a simultaneous malignant SPN and an intraductal papillary mucinous adenoma (IPMA) of the pancreas. The patient was admitted to our department for the evaluation of the main solid tumor with calcification and small multilocular cystic lesions apart from the main tumor in the pancreatic head. We performed pylorus-preserving pancreaticoduodenectomy to treat the calcified tumor and multilocular cystic lesions. The diagnosis of malignant SPN was confirmed on the basis of histological invasion to the adjacent structures. The separate cystic lesions were diagnosed as a branch-type IPMA. The synchronous occurrence of IPMA and SPN in the present case did not demonstrate that there were tumors maintained through the common abnormal Wnt signaling pathway by immunohistochemical study. To our knowledge, this is the first known case of synchronous SPN and IPMA of the pancreas.

A case of pancreatic solid-pseudopapillary neoplasm with marked ossification.

A cystic lesion of the pancreas was detected in a 25-year-old asymptomatic woman during a company medical checkup. Abdominal computed tomography (CT) revealed an ossified lesion in the pancreatic body, and the patient was referred to us for further management. Abdominal ultrasound and endoscopic ultrasound showed a hypoechoic mass with a large ossific focus, measuring 25mm in the pancreatic body. CT and magnetic resonance imaging (MRI) showed a nonenhancing tumor lesion with a large calcific focus, measuring 20mm in the pancreatic body. Endoscopic retrograde pancreatography revealed mild stenosis of the main pancreatic duct near the ossific focus in the pancreatic body. From the above, a pancreatic solid-pseudopapillary neoplasm (SPN) was suspected, and resection of the pancreatic body was performed. Intraoperatively, a whitish tumor measuring about 20mm was recognized. Histopathologically, moderately large-sized tumor cells containing eosinophilic cytoplasm were arranged in a pseudopapillary formation, and infiltrated the surrounding normal tissue with ossification. Immunostaining was positive for α1-antitrypsin, not inconsistent with the diagnosis of SPN. We report this case of pancreatic SPN with ossification and a review of the literature.

Solid pseudopapillary tumor of the pancreas (SPPT): Still an unsolved enigma

Solid pseudo-papillary tumor (SPPT) is a rare cystic tumor of the pancreas (1-3% of exocrine tumors of the pancreas) which shows an "enigmatic" behavior on the clinical and molecular pattern. A retrospective analysis of the cytological studies and resected specimens of pancreatic cystic tumors from May 1996 to February 2010 was carried out. Three cases of SPPT were found, which are the objective of this study. The diagnosis was established upon occasional finding in the abdominal CT, in spite of sizing between 3 and 6 cm of diameter. In the three cases the preoperative diagnosis was confirmed by cytology and specific immunohistochemical staining. Cases 2 and 3 showed strong immunoreactivity for Beta-Catenin and E-Cadherin staining. Radical resection (R0) was carried out in the three cases. A young male -21 years of age (case 1)- who had duodenal infiltration and two lymph nodes metastases died of hepatic and peritoneal recurrence 20 months following surgery. The other two cases are free of disease. The current review of the literature reports roughly 800 cases since the first report in 1959, and shows the enigmatic character of this tumor regarding the cellular origin, molecular pathways, prognostic factors and clinical behavior.

A Young Male Case of Solid-Pseudopapillary Tumor That Has High Uptake in PET Preoperatively

35歳男性．検診超音波検査で膵嚢胞性病変が疑われた．CTでは境界明瞭な造影効果を有する充実性腫瘍で，MRIではT1WIでlow，T2WIでややhigh intensityで拡散能低下を示した．positron emission computed tomography（PET）では，腫瘍に一致して集積を認めた．膵癌の術前診断で膵体尾部切除術を行った．病理診断はsolid-pseudopapillary tumor（SPT）であった．SPTは若年女性に多く，被膜を有し，出血や壊死に伴う嚢胞性変化を特徴とする．本例は非典型例で術前診断が非常に困難であった．PETは強く悪性を示唆した．膵腫瘍の確定診断には近年US，CT，MRIに加えPETも重要視されるが，SPTのPET診断に関する検討は少ない．今回診断にPETを用いたSPTを経験したので，PET診断について考察し報告する．

A Case of Solid Pseudopapillary Neoplasm of the Pancreas Presenting with Left-sided Extrahepatic Portal Hypertension

A 53-year-old woman was referred to our hospital for management of gastric varices that ran transversely across the greater curvature of the gastric body, detected during routine upper gastrointestinal endoscopy. CT identified a low-density calcified mass near the tail of the pancreas and the splenic hilum. Based on the results of radiographic and pathological investigations, the tumor was diagnosed as solid pseudopapillary neoplasm (SPN), and the gastric varices were considered to have developed secondary to occlusion of the splenic vein by the tumor mass. This is a rare case of SPN associated with splenic vein occlusion and left-sided extrahepatic portal hypertension.

Pathological differential diagnosis of solid-pseudopapillary neoplasm and endocrine tumors of the pancreas

To investigate differential points of solid-pseudopapillary neoplasm (SPN) of the pancreas and pancreatic endocrine tumor (PET). Ten cases of SPN and fourteen cases of PET were studied in this retrospective study. Clinical and pathologic features, immunostaining reactions and beta-catenin gene mutations were analyzed. The mean age of SPN patients was 25.6 years and these patients had no specific symptoms. The mean diameter of the tumors was 11.0 cm, 9/10 cases were cystic or a mixture of solid and cystic structures, and there was hemorrhage and necrosis on the cut surface in 8/10 (80%) cases. Characteristic pseudopapillary structure and discohesive appearance of the neoplastic cells were observed in all 10 (100%) cases. The results of immunostaining showed that nuclear expression of beta-catenin and loss of E-cadherin in all the cases, was only seen in SPN. Molecular studies discovered that 9/10 (90%) cases harbored a point mutation of exon 3 in beta-catenin gene. On the other hand, the mean age of PET patients was 43.1 years. Eight of 14 cases presented with symptoms caused by hypoglycemia, and the other 6 cases presented with symptoms similar to those of SPN. The mean size of the tumors was 2.9 cm, most of the tumors were solid, only 3/14 (21%) were a mixture of solid and cystic structures, and macroscopic hemorrhage and necrosis were much less common (3/14, 21%). Histologically, tumor cells were arranged in trabecular, acinar or solid patterns and demonstrated no pseudopapillary structure and discohesive appearance in all 14 (100%) cases. The results of immunostaining and mutation detection were completely different with SPN that membrane and cytoplastic expression of beta-catenin without loss of E-cadherin, as well as no mutation in beta-catenin gene in all the cases. Both macroscopic and microscopic features of SPN are quite characteristic. It is not difficult to distinguish it from PET. If necessary, immunostaining of beta-catenin and E-cadherin is quite helpful to make the differential diagnosis.

Tumor sólido pseudopapilar de pâncreas: relato de um caso com apresentação incomum

Atypical clinical case of solid pseudopapillary tumor (SPPT) of the pancreas in a 37 year-old man with acute abdominal pain. After surgery, anatomopathologic investigation with immunohistochemistry showed pattern in keeping with SPPT and strong positivity for β-catenin. Two years post-surgery the patient does not present recurrence. The SPPT normally occurs in young women with a history of mass or abdominal discomfort, although in some cases it can be asymptomatic. The tumor has slow growth, low potential for malignancy and surgical resection yields high cure rates.

Solid-pseudopapillary neoplasm of the pancreas with massive central calcification in an old man

Solid-pseudopapillary neoplasm (SPN) is a rare pancreatic tumor primarily affecting women in their twenties. It is characterized by a well-demarcated or encapsulated mass, indolent behavior and favorable prognosis. Capsular or punctate calcification is occasionally observed. Reported herein is a case of SPN of the pancreas with massive calcification in a 76-year-old Japanese man. Macroscopically, the pancreatic tumor appeared to be a simple calcified nodule, but histological examination revealed that it was an epithelioid tumor with massive calcification. The tumor cells, forming nests and cords, had eosinophilic cytoplasm and small eccentric nuclei. They were immunohistochemically positive for vimentin, CD56 and neuron-specific enolase. Nuclear accumulation of β-catenin protein and a point mutation of the β-catenin gene by genomic DNA sequencing confirmed that the tumor was SPN. This is a very rare case of pancreatic SPN with massive calcification in an old man.

Eight Cases of Solid Pseudopapillary Tumors (SPT) of the Pancreas

膵Solid Pseudopapillary Tumor（SPT）は若年女性に好発する低悪性度の稀な膵腫瘍とされる．治療方針は，手術での腫瘍完全摘除であり，術式としては良性では核出術，悪性では膵切除が基本とされ，予防的なリンパ節郭清は不要とされている．今回外科的治療方針を明らかにする目的で当科で切除した膵SPT症例8例の臨床所見，術前画像所見，術式，病理組織学的検査を検討した． 術前の画像所見で局所浸潤，リンパ節転移，遠隔転移などの悪性所見は認めず．術式は膵頭十二指腸切除術を4例，膵体尾部切除術を3例，膵中央区域切除術を1例に施行し，全例でリンパ節郭清を行った．病理組織検査では8例中4例に悪性を認め，リンパ節転移を1例に認めた．術後平均観察期間は70.9ヶ月で，全例無再発生存中である． 膵SPTは術前画像検査で非転移例の良悪性診断は困難であり，治療方針はリンパ節郭清を伴う膵切除が妥当である．

Solid Pseudopapillary Neoplasm of the Pancreas: Report of Two Cases and Review of the Literature

Solid pseudopapillary neoplasm (SPN) of the pancreas is a rare low-grade malignant-potential epithelial tumor that predominantly affects young women aged 20–35 years with a mean age of 22 years. It is currently categorized in the World Health Organization classification under exocrine pancreatic tumor. Here, we present two cases of SPN with initial presentation of large intra-abdominal masses. Both patients underwent successful en bloc distal pancreatectomy and splenectomy. Local recurrence and distant metastasis were not detected at the follow up at 21 months and 9 years respectively. In summary, a large, well-encapsulated cystic mass in the pancreas of a young woman should raise suspicion of SPN.

Nuclear E-cadherin Immunoexpression

E-cadherin is a well-recognized molecule that is important in cell adhesion. Its abrogation has been linked to increased invasiveness in several malignancies. The normal immunohistochemical localization of E-cadherin is the cell membrane, however, both cytoplasmic and nuclear immunostaining has been reported. Loss of membrane staining and/or nuclear staining for E-cadherin is seen in 100% of cases of solid pseudopapillary tumors (SPTs) of the pancreas. In the context of SPT, E-cadherin staining is of diagnostic use. Nuclear staining has been seen in cases of pancreatic neuroendocrine tumors, Merkel cell carcinomas, clear cell renal cell carcinoma, esophageal squamous carcinoma, colorectal and gastric cancer, and synovial sarcoma. The difference in the staining patterns seen (complete loss vs. nuclear staining) is due to the type of E-cadherin antibody used. Antibodies recognizing the extracellular domain show loss of E-cadherin staining in SPT, whereas the antibody to the cytoplasmic domain results in nuclear staining in all cases of SPT. Therefore, E-cadherin staining is of diagnostic use in the immunohistochemical work-up of SPT. Nuclear E-cadherin staining of pancreatic neuroendocrine tumors identified a subset of cases with more aggressive potential, whereas nuclear staining of clear cell renal cancers identified a subset of tumors with a better prognosis. The exact mechanism by which E-cadherin enters the nucleus is not known but it is likely that it is closely related to several partner molecules such as beta-catenin, p120, and presenilin-1.

Fine-Needle Aspiration

Fine-needle aspirates of the pancreas are increasingly being performed to obtain preoperative diagnoses of pancreatic lesions. This has also led to an increased recognition of nonductal pancreatic malignancies such as solid pseudopapillary neoplasm of the pancreas and pancreatic endocrine tumors. These tumors often show overlapping cytomorphologic features, which could lead to errors in interpretation by unsuspecting pathologists. This report describes the salient cytologic features of various nonductal pancreatic neoplasms that, if followed closely, should help avoid pitfalls in diagnoses. This discussion uses a case of solid pseudopapillary neoplasm of the pancreas diagnosed on endoscopic ultrasound-guided fine-needle aspirate as the model.

Histogenesis of solid pseudopapillary tumor of the pancreas: the case for the centroacinar cell of origin

Solid pseudopapillary tumor (SPT) is an unusual pancreatic neoplasm of low malignant potential that most frequently occurs in young women. The tumor is indolent, with long patient survival, even in the presence of extension into adjacent organs and metastases. Histologically, it is a solid and cystic tumor with a prominent vascular network and degenerative pseudopapillae formation. Despite its distinctive morphology and cytological features, its histogenesis is unclear. Herein, we report a case of solid pseudopapillary tumor in a 41-year-old female in which the tumor cells immunohistochemically and ultrastructurally suggest a centroacinar cell origin. The tumor cells and the normal centroacinar cells stained positive for alpha-antitrypsin (α-AT), CD10, cyclin D1 and NSE. Ultrastructural examination shows similarities in nuclear shape, nucleoli location and cytoplasmic contents between neoplastic cells and normal centroacinar cells of the pancreas. Based on both immunohistochemical and ultrastructural features, we propose that the centroacinar cell is the origin of SPT.

A solid pseudopapillary tumor arising from the greater omentum followed by multiple metastases with increasing malignant potential

Solid pseudopapillary tumor is a rare, indolent neoplasm almost exclusively seen in the pancreas. We describe an unusual case of solid pseudopapillary tumor arising in the greater omentum of a 45-year-old man with subsequent multiple liver metastases and peritoneal dissemination. The patient underwent a total of ten laparotomies and died of unresectable disease 8 years after the initial presentation. Microscopically, the primary tumor and the relapsed tumors consistently exhibited identical growth patterns, which were characterized by solid sheets intermingling with pseudopapillary arrangements of uniformly small cells. Immunohistochemical staining was diffusely positive for vimentin and focally positive for alpha-1-antitrypsin. These features were compatible with those of conventional pancreatic solid pseudopapillary tumors. We also performed quantitative evaluation of Ki-67 immunoreactivity and mitotic figures, which indicated malignant transformation of this extremely rare tumor. This is the first detailed report of solid pseudopapillary tumor arising outside the pancreas complicated by repetitive liver metastases and peritoneal carcinomatosis, suggesting the existence of a more lethal subgroup of tumors.

Solid pseudopapillary neoplasms of the pancreas are associated with FLI-1 expression, but not with EWS/FLI-1 translocation

Solid pseudopapillary neoplasms of the pancreas are rare pancreatic tumors with mostly benign behavior, affecting almost exclusively women. Their histogenetic origin is still unsolved, but a recently reported EWS/FLI-1 translocation t(11;22)(q24;q21) and the consistent expression of CD56 and the progesterone receptor, both genes located on the long arm of chromosome 11, point to chromosome 11q as a potential locus of gene aberration in solid pseudopapillary neoplasms. To further elucidate this issue, we studied 30 cases of solid pseudopapillary neoplasms by comparative genomic hybridization (CGH), fluorescent in situ hybridization (FISH) and immunohistochemistry. Immunohistochemically, 38% showed nuclear expression of FLI-1 and all cases revealed positivity for CD56 and the progesterone receptor, whereas no solid pseudopapillary neoplasm expressed CD34. No translocation of the EWS gene was found by FISH and no gross chromosomal gain or loss was detected by CGH. It is concluded that FLI-1 expression in solid pseudopapillary neoplasms is not associated with an EWS/FLI-1 translocation. In addition, there are no chromosomal gains or losses, especially on chromosome 11, where the FLI-1 gene is located adjacent to the gene for CD56 (NCAM). These data add another feature to the complex phenotypic appearance of solid pseudopapillary neoplasms.

急性膵炎を契機に発見された膵solid-pseudopapillarytumorの1例

急性膵炎を契機に発見された膵solid-pseudopapillarytumorの1例

12歳女児のsolid-pseudopapillary tumorの1例

12歳女児のsolid-pseudopapillary tumorの1例

Four cases of solid pseudopapillary tumors of pancreas: Imaging findings and pathological correlations

Objective Solid pseudopapillary tumor of the pancreas (SPTP tumor) is a rare pancreatic neoplasm with low malignant potential, which usually affects female patients in the second or third decades of life. It is a non-functional, slow-growing neoplasm that very often reaches considerable size before the first symptoms appear. Symptomatology is frequently related to tumor size. Surgical excision is usually curative in most cases. Infrequently the tumor can appear in male patients or in aged women, which can make the diagnosis more difficult. Some patients develop liver metastases in the follow-up that can be resected. Our purpose is to review the radiological and pathological findings of SPTP with emphasis on these infrequent cases. Subjects and methods The medical records and radiological findings of patients who underwent surgery for SPTP between 2000 and 2005 were retrospectively reviewed. Study eligibility required that patients had undergone surgical resection and that a SPTP had been pathologically proved. Results Four cases of solid pseudopapillary tumor of the pancreas were diagnosed and treated in our institution in the study period. Two of the patients, developed on follow-up liver metastases, and peritoneal, hepatic, and nodal metastases, respectively. Conclusion Solid pseudopapillary tumors are well-encapsulated neoplasms that usually have a good prognosis after surgical excision. A malignant behavior is uncommon and in this case lymph node involvement, hepatic metastases and occasionally peritoneal invasion may also occur. Resection of liver metastases can prolong the long-term survival of the patients.

Tumor sólido seudopapilar del páncreas

Solid-pseudopapillary tumor of pancreas is a very rare neoplasm of low malignant potential and unknown origin. It generally occurs in young women and the prognosis is usually good after complete surgical removal. Two cases of solid pseudopapillary tumor with distinct disease duration are reported.