Solid-Pseudopapillary Tumor: Case Report and Literature Review

Solid-pseudopapillary tumor of the pancreas (SPT) or Frantz’s tumor is a rare epithelial neoplasm that represents 0.3% to 2.7% of exocrine pancreatic tumors. These tumors occur mainly in young women and have a good prognosis. We present a case of a 19-year-old female patient who presented to the emergency for abdominal pain. Physical examination reveals a left hypochondrium mass. Ultrasound imaging showed an encapsulated caudal pancreatic mass with cystic components. The patient underwent tumor excision and lymph node removal. Macroscopically, the tumor was encapsulated and measuring 12 × 8 × 7 cm. It has a solid-cystic and hemorrhagic appearance inside. Histologically, the tumor had two components: solid and papillary. Tumor tissue showed monomorphic tumor cells radiating around blood vessels. Perineural invasion and vascular emboli were not seen. Three lymph nodes without metastases were observed. The diagnosis of solid-pseudopapillary tumor of the pancreas (SPT) or Frantz’s tumor was retained. Solid-pseudo-papillary tumor of the pancreas is rare and not always suspected by the physician. The clinical and imaging patterns are not specific. Anatomopathological examination confirmed the diagnosis. The patient underwent curative surgery.

Aggressive behaviour of solid-pseudopapillary tumor of the pancreas in adults:A case report and review of the literature

Solid-pseudopapillary tumor (SPT) is a rare neoplasm of the pancreas that usually occurs in young females. It is generally considered a low-grade malignant tumor that can remain asymptomatic for several years. The occurrence of infiltrating varieties of SPT is around 10%-15%. Between 1986 and 2006, 282 cystic tumors of the pancreas were observed. Among them a SPT was diagnosed in 8 patients (2.8%) with only one infiltrating variety. This was diagnosed in a 49-year-old female 13 years after the sonographic evidence of a small pancreatic cystic lesion interpreted as a pseudocyst. The tumor invaded a long segment of the portal- mesenteric vein confluence, and was removed with a total pancreatectomy, resection of the portal vein and reconstruction with the internal jugular vein. Histological examination confirmed the R-0 resection of the primary SPT, although a vascular invasion was demonstrated. The postoperative course was uneventful, but 32 mo after surgery the patient experienced diffuse liver metastases. Chemotherapy with different drugs was started. The patient is alive and symptom-free, with stable disease, 75 mo after surgery. Twenty-five patients with invasion of the portal vein and/or of mesenteric vessels were retrieved from the literature, 16 recent patients with tumor relapse after potentially curative resection were also retrieved. The best treatment remains a radical resection whenever possible, even in locally advanced or metastatic disease. The role of chemotherapy, and/or radiotherapy, is still to be defined.

Solid-pseudopapillary tumor of the pancreas: Clinical experience and literature review

AIM: To evaluate the clinical presentations of solidpseudopapillary tumor of the pancreas (SPT) and examine the diagnosis, treatment, low grade malignant potential of this rare disease.METHODS: We retrospectively reviewed a series of seven patients with SPT managed in our hospital between July 1990 and October 2003. Six females and one male with mean age of 31 years (range 13 to 50 years) were diagnosed with SPT at our institution.RESULTS: Clinical presentation included a palpable abdominal mass in two patients and vague abdominal discomfort in another two. Two patients were asymptomatic;their tumors were found incidentally on abdominal sonographic examination for other reasons. The final patient was admitted with hemoperitoneum secondary to tumor rupture. The mean diameter of the tumors in the seven patients was 10.5 cm (range 5 to 20 cm). The lesions were located in the body and tail in five cases and in the head of the pancreas in two. Surgical procedures included distal pancreatectomy (3), distal pancreatectomy with splenectomy (2), pancreaticoduodenectomy (1) and a pylorus-preserving Whipple procedure (1). There were gross adhesions or histological evidence of infiltration to the adjacent pancreas and/or splenic capsule in four cases. None of the patients received adjuvant therapy.The mean follow up was 7 years (range 0.5 to 14 years).One patient developed multiple liver metastases after 14 years of follow up.CONCLUSION: SPT is a rare tumor that behaves less aggressively than other pancreatic tumor. However, in cases with local invasion, long-term follow up is advisable.

Diagnosis and treatment of solid-pseudopapillary tumor of the pancreas

BACKGROUND: Solid-pseudopapillary tumor (SPT) of the pancreas is a rare exocrine pancreatic tumor. Despite the increasing recognition of the tumor in recent years, its pathogenesis and apparent therapeutic algorithm remain unclear. This study was designed to define the clinical, imaging, and pathologic features and to improve the diagnosis and treatment of this rare disease. METHOD: The clinical, imaging, and pathologic findings of 9 SPT patients managed in our hospital between 2001 and 2005 were retrospectively analyzed, and related literatures were reviewed. RESULTS: In the 9 patients aged from 14 to 68 years, 8 were female and 1 male. The mean age of these patients at diagnosis was 30 years. Initially, 8 patients complained of vague abdominal pain and one patient had pancreatic mass detected incidentally by abdominal CT. The levels of blood and urine amylase and tumor markers were all within the normal range. B-US, CT and MRI demonstrated that tumors were well encapsulated and contained some degree of internal hemorrhage or cystic degeneration. The mean transverse diameter of these tumors was 5.4 cm (range, 2-10.5 cm). The tumors were located at the head (2 patients), body (2), body and tail junction (4), and tail (1) of the pancreas. Surgical procedures included pancreaticoduodenectomy, distal pancreatectomy, distal pancreatectomy with splenectomy, and enucleation. Histological examination showed solidified cystic areas and papillary protrusions. Two malignant tumors demonstrated retroperitoneal metastases and vascular invasion. Follow-up for 2.5 years on average showed that one patient died of tumor recurrence at 10 months and the rest were alive. CONCLUSIONS: SPT exhibits unique clinical and pathologic features and is readily diagnosed by its characteristic imaging and histological appearance. Surgical resection of the primary tumor and metastases is the treatment of choice.

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

AIM:To investigate differential points of solid-pseudo-papillary neoplasm (SPN) of the pancreas and pancre-atic endocrine tumor (PET).METHODS:Ten cases of SPN and fourteen cases of PET were studied in this retrospective study. Clinical and pathologic features,immunostaining reactions and β-catenin gene mutations were analyzed.RESULTS: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 pseudo-papillary structure and discohesive appearance of the neoplastic cells were observed in all 10 (100%) cases. The results of immunostaining showed that nuclear expression of β-catenin and loss of E-cadherin in all the cases,was only seen in SPN. Molecular studies discov-ered that 9/10 (90%) cases harbored a point mutation of exon 3 in β-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 hypoglyce-mia,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 β-catenin without loss of E-cadherin,as well as no mutation in β-catenin gene in all the cases. CONCLUSION:Both macroscopic and microscopic features of SPN are quite characteristic. It is not difficult to distinguish it from PET. If necessary,immunos-taining of β-catenin and E-cadherin is quite helpful to make the differential diagnosis.

Clinical analysis of solid-pseudopapillary tumor of the pancreas:report of 15 cases

BACKGROUND: Solid-pseudopapillary tumor of the pancreas (SPTP) is an uncommon and enigmatic pancreatic neoplasm that occurs mainly in young women. Although more and more cases have been reported in recent years, misdiagnosis and incorrect treatment still frequently take place. This study was designed to stimulate consideration of this tumor. METHODS: We retrospectively reviewed the experience of diagnosis and treatment of 15 patients with SPTP and compared them with 516 patients with pancreatic cancer from January 1997 to March 2007. RESULTS: Most of the SPTP cases were asymptomatic except for one palpable mass. Almost all SPTPs demonstrated a solid structure with hypo- or isoattenuation, cystic structure with hypo-attenuation on precontrast CT scan, and enhancement of solid portions on post-contrast CT scan. By contrast, most cases of pancreatic carcinoma had multiple symptoms and abnormal blood results. The tumors showed hypo-attenuation on both precontrast and post-contrast CT scan, and only a few showed iso-attenuation on post-contrast CT scan. All cases of SPTP in our group were cured by surgical resection, while only 16.86% of patients with pancreatic carcinoma could undergo a radical resection. CONCLUSIONS: Clinical features and CT scans were helpful to differentiate SPTP from pancreatic carcinoma. Radical surgical resection was the most effective and safe method for the treatment of SPTP.

Solid-pseudopapillary tumor of the pancreatic tail

We report a case of the rare solid-pseudopapillary tumor of the pancreas. In contrast to other pancreatic tumors,the solid-pseudopapillary tumor has a favorable prognosis.The 60-year-old female patient we report on here was treated by left pancreatic resection combined with splenectomy for a non-metastasizing tumor of the pancreas. A solid-pseudopapillary tumor was found on histology. The patient had no signs of metastases at present.Since a microscopically invasive tumor growth is assumed,oncologically curative resection should be preferred vs the less radical enucleation. The rare solid-pseudopapillary tumor of the pancreas has a good prognosis after successful oncological resection.

The clinicopathological and immuohistochemical analysis of solid-pseudopapillary tumor of the pancreas:report of 9 cases

Objective：To investigate the clinical features, pathological characteristics and immunophenotype of solid-pseudopapillary tumor of the pancreas（SPTP）. Methods：Nine surgically treated cases of SPTP were retrospectively reviewed. Hematoxylin and Eosin（HE） staining and immunohistochemical staining were used to analyze all cases, and the general clinical data was collected. Results：Six patients were asymptomatic except for a palpable mass. Two patients complained of vague-epigastric pain. One patient appeared jaundice. The tumor was encapsulated and solid tissues alternately with cystic tissues. Histologically, the histological structure of solid portion was pseudopapillary with a fibrovascular core. Tumor cells were uniform and medium-sized which were arranged in sheets ets or nests or pseudopapillary patterns. Immunohistochemical studies demonstrated that SPTP proved positive in vimentin（9/9 cases）, AAT（9/9 cases）, NSE（9/9 cases）, ACT（7/9 cases）, CK20（2/9 cases）, CgA（1/9 cases）, S-100（3/gcases）, PR（4/gcases）, Syn（3/9 cases） and CD56（5/9cases）, negative in CEA and ER. Conclusion：SPTP is a tumor predominantly occurring in young women frequently without special symptoms. This tumor has various characteristical histological patterns with different immunophenotype.

Solid-Pseudopapillary Tumor of the Pancreas:One Case Report and Literatures Review

IntroductionSolid-pseudopapillary tumor(SPT)is a very rare primary neoplasmof the pancreas.Franz first described it in 1959.It is usually seen inyoung females.In spite of possible histological findings of malignan-cy,SPPT typically shows a benign clinical course and a low malig-nant potential.The pathogenesis of these tumors is still controversial.It has been suggested that it might originate from ductal and acinarpancreatic cells,endocrine cells or pluripotential stem cells.

Solid-Pseudopapillary Tumor of the Pancreas in Adults: A Case Report

Solid-pseudopapillary tumor of the pancreas (SPTP) is an uncommon low grade exocrine pancreatic malignancy. We represented a 22 years old female with an abdominal mass of 4 years history and symptoms of weakness, loss of appetite, 7 kilograms weight loss and swelling for almost 2 months. She was suspected of pancreatic malignancy due her abdominal ultrasound and CT reports and therefore underwent explorative surgery that revealed a huge pancreatic tumor .The tumor was resected totally and hystopathological examination reported significant components of pancreatic solid pseudopapiller tumor features which was also the final diagnosis.

Plummer-Vinson syndrome associated with solidpseudopapillary tumor of the pancreas

Plummer-Vinson syndrome （PVS）, also known as Paterson-Kelly syndrome or sideropenic dysphagia1 is characterized by dysphagia, iron deficiency anemia and esophageal webs. This syndrome is known to be associated with an increased risk of hypopharyngeal and/or cervical esophageal cancer. Three to 15 percent of the patients with PVS, mostly women between 15 and 50 years of age, have been reported to develop esophageal or pharyngeal cancer. There is a decreasing trend in the overall incidence of hypopharyngeal cancer in women, probably due to the diminished prevalence of PVS. There are few reports of gastric cancer in association with PVS.2