Retroperitoneal congenital epidermoid cyst misdiagnosed as a solid pseudopapillary tumor of the pancreas: A case report.

BACKGROUND: Retroperitoneal cysts are rare and usually asymptomatic abdominal lesions. Epidermoid cysts are frequent benign cutaneous tumors, but retroperitoneal localization of these cysts does not occur very often. CASE SUMMARY: We report a case report of a 25-year-old woman with a giant mass in the abdominal cavity. Because imaging examination indicated that the mass probably originated from the pancreas, the mass was considered a solid pseudopapillary tumor of the pancreas (SPTP). However, surgery revealed a retroperitoneal epidermoid cyst located behind the pancreas neck and the root of the superior mesenteric artery (SMA). We performed complete resection of the tumor. Postoperative pathology showed an epidermoid cyst. The patient fared well after two months of follow-up. CONCLUSION: Surgery is the gold standard for the diagnosis and treatment of retroperitoneal epidermoid cysts. Retroperitoneal epidermoid cysts around the pancreas are easily misdiagnosed as cystic SPTPs. Surgeons should pay particular attention to preoperative diagnosis to reduce severe surgical complications and improve the quality of life of patients.

Solid Pseudopapillary Tumor of Pancreas: A Clinicopathologic Report from a Single Institution in Southern Iran.

BACKGROUND: Solid pseudopapillary tumor of pancreas (SPTP) is a rare tumor of the pancreas which mostly occurs in young women. Since its first description in 1969, more than 500 cases have been reported. There have been just 2 case reports and 1 review (7 cases) from Iran. In this study, we reported our experience with 22 patients with SPT from the largest pancreaticobiliary center in Southern Iran. METHODS: During 6 years (2012-2017), 22 cases of SPTP were operated on in our center. All of these cases were recruited and after confirmation of the pathological diagnosis, clinical charts were evaluated and all the clinicopathologic findings as well as outcome of the surgery were evaluated. RESULTS: Among the 22 patients, 20 were female and 2 were males. The age range was 15-52 years and the tumor sizes were 3.5 to 17 cm. All of the tumors had preoperative diagnosis by imaging modalities and were operated on with no complication or recurrences. Just one case showed liver metastasis. All of the patients with SPTP in this study were alive and in good condition. CONCLUSION: SPTP is not very rare in our center. In young patients presenting with pancreatic mass, especially in female patients, one of the most important diagnoses is SPTP. Conservative surgery and tumor excision is satisfactory and patients show excellent prognosis even after liver metastasis.

Solid Pseudopapillary Neoplasm of the Pancreas Clinicopathological Characteristics and Long-Term Outcome: a Single-Center Experience.

Solid pseudopapillary neoplasm is a rare tumor of the pancreas. These tumors are considered to have low malignant potential with good prognosis. Due to its rarity, details about clinical presentation and management of the disease are not very clear. This study aims to share our experience and to describe management of the disease. We retrospectively evaluated patients 13 patients diagnosed with SPT on histopathological examination of resected specimen. Data on their clinicopathological, management-related factors, and follow-up was collected. All the patients were females, with a median age of 20 years. Abdominal pain was the most common presentation. The mean tumor size was 6.5 cm and majority of tumors were located in the head region. R0 resection was obtained in all the patients. All patients were disease-free with a median follow-up of 68 months. Excellent prognosis can be achieved with a margin-negative resection in these tumors. Adjacent organ or vascular involvement is not a contraindication for surgical resection.

Solid Pseudopapillary Tumor of the Pancreas - Rare Neoplastic Disease in 20-Year-Old Woman.

INTRODUCTION: Benign cystic tumors represent only 2% of all pancreatic tumors (pancreatic cancer - PC). In contrast to malignant cystic tumors, these tumors occur typically in young women. A solid pseudopapillary tumor is a relatively rare affliction representing less than 4% of cystic PC. Although the tumor is considered benign, metastasis, especially to the spleen, has been reported in approximately 0.5-4% patients. Despite R0 resection, vascular and perineural invasion is monitored in 20% of cases. Invasion is the cause of tumor relapse in up to one third of affected patients. Characteristic features of the disease are latent clinical indicators such as signs of pain and malfunction of intestinal passage. The diagnostics is based on MR, sometimes in combination with positron emission tomography. Medical treatment is specifically surgical. CASE HISTORY: Authors present a case of a 20-year-old female patient who was examined due to pain in the epigastrium, further exasperated by a voluminous expansion of the abdominal cavity. An initial ultra-sonographic examination was conducted to examine for possible nodular focal nodular hyperplasia of the liver; however, an MRI scan revealed the likelihood of a malignant tumor in the subhepatic region. During laparotomy, a tumor protruding from the head of the pancreas was discovered and removed. Histological examination showed it was a solid pseudopapillary pancreatic tumor. After a month of good post-operative progress, the patient was re-operated because of the presence of pancreatic fistula. Complete healing of the fistula was achieved after total parenteral nutrition and administration of sandostatin. At her last examination, the patient was without any problems. CONCLUSION: Solid pseudopapillary pancreatic tumors are rare, mainly benign lesions. It is essential to consider them in the differential diagnostics of afflictions of the subhepatic region, especially in young women. The only generally accepted cure nowadays is surgical resection. It is necessary to monitor patients consistently considering the rather high frequency of relapse of disease despite R0 resections. In the case of surgical removal, the 5-year survival rate is near 97%. Key words: solid pseudopapillary tumor of pancreas - diagnostics - therapy The authors declare they have no potential conflicts of interest concerning drugs, products, or services used in the study. The Editorial Board declares that the manuscript met the ICMJE recommendation for biomedical papers. Submitted: 17. 4. 2018 Accepted: 13. 8. 2018.

Diagnosis and prognosis of solid pseudopapillary tumor of pancreas: an analysis of 68 patients / 中华肝胆外科杂志

Objective To study the diagnosis,treatment and prognosis of patients with solid-pseudopapillary tumor of the pancreas (SPTP).Methods The clinical and follow-up data of 68 patients with SPTP treated in the Affiliated Tumor Hospital of Zhengzhou University from January 2008 to March 2017 were retrospectively analyzed.Results There were 6 males and 62 females,with an average age +/-S.D.of (32.0 ± 12.0) years.The mean tumor size was (4.4 ±2.3) cm.The primary symptom was abdominal pain,and a pancreatic mass was subsequently detected.All the patients underwent surgical resection which included pancreaticoduodenectomy,duodenal preserving pancreatic head resection,distal pancreatectomy,middle pancreatectomy,local excision,resection of pancreatic tail plus splenectomy and distal pancreaticosplenectomy.Postoperative histopathological examination confirmed solid pseudopapillary tumor of the pancreas in all these patients.Twenty-one patients (30.9％) developed postoperative complications,which included pancreatic fistula in 12 patients (17.7％),hemorrhage in 2 patients (2.9％),pleural effusion in 2 patients (2.9％),incision infection in 3 patients (4.4％),and gastric emptying disorder in 2 patients (2.9％).There was no in-hospital mortality.The average length of hospital stay was (26.0 ± 10.0) days.Of the 57 patients (83.8％) who were followed-up (mean 38 months,range 3 to 114 months),11 patients developed postoperative indigestion and 2 patients diabetes.No patient developed tumor recurrence,metastasis and death.Conclusions SPTP is a low grade malignant tumor,which is found primarily in young women.The clinical characteristics are non-specific,and preoperative diagnosis is difficult.Excellent prognosis can be achieved with surgical resection which is the preferred treatment for SPTP.

Value of MSCT with dual-phase enhanced in the diagnosis of solid pseudopapillary tumor of pancreas / 中国医师进修杂志

Objective To explore the CT imaging features of solid pseudo- papillary tumor of pancreas (SPTP) and the diagnostic value of dual-enhanced scanning in SPTP. Methods CT imaging data of 38 cases with SPTP confirmed by surgical pathology were retrospectively analyzed, among whom there were 20 cases who had underwent dual-phases enhanced scanning and 18 cases who had underwent three-phases enhanced, the CT imaging features of the tumors,enhancement degree and the enhancement peak phase of tumors′solid composition were observed. Results All the tumors were single.Tumor located in the head of pancreas in 14 cases, in the neck in 4 cases,in the body in 8 cases, in the tail in 7 cases, in the body-tail of border in 4 cases,and 1 case in the head-neck of border.The size of tumors was 2.4+7.8 cm;tumor of 12 cases was solid mainly,of 24 cases was solid and cystic,and of 2 cases was cystic mainly. Enhancement was uneven. The solid composition showed mild-to-moderate reinforcement, and the enhanced peak stage was in portal vein phase or delay phase.Conclusions There are some characteristics in CT imaging findings of SPTP, CT imaging combined with clinical characteristics can make an accurate diagnosis before operation. The diagnosis accuracy of SPTP with MSCT through dual- phases enhancement scaning and the three- phases enhancement shows no difference,and the former scaning technology can reduce the received effective radiation dose of patients and improve the efficiency of examining.It is worth of promoting.

Misdiagnosed gastrinoma: A case report.

Gastrinoma is most commonly located in the gastrinoma triangle (comprising of the duodenum, pancreas and bile ducts) or in the adjacent lymph nodes. Due to the low mortality rate, it is often misdiagnosed as other diseases with similar clinical characteristics, such as a solid pseudopapillary tumor of the pancreas (SPTP). Therefore, the current study reports a rare case of gastrinoma located in the tail of the pancreas of a female patient under medical examination, who exhibited no clinical symptoms. The tumor, which was located in the body and tail of the pancreas, was successfully resected and the spleen was preserved. The outcome of surgery combined with the postoperative pathological examination resulted in the patient being misdiagnosed with a SPTP. During the consequent six-year follow-up period, low-density liver lesions and an intractable peptic ulcer gradually appeared. Finally, the patient diagnosis was confirmed as a malignant pancreatic neuroendocrine carcinoma with liver metastases. On June 1, 2011, a liver transplant was successfully performed and the patient has maintained a good overall condition. The underlying clinical and pathological factors that may have resulted in misdiagnosis are investigated in the present study. Through providing our preliminary clinical experiences and lessons, the aim of the present study was to focus the attention of clinicians on this type of cancer in order to improve its diagnosis and treatment.

CD 99 immunocytochemistry in solid pseudopapillary tumor of pancreas: A study on fine-needle aspiration cytology smears.

BACKGROUND: Solid pseudopapillary tumor of pancreas (SPTP) is a rare pancreatic tumor of uncertain histogenesis usually affecting young women. Though these tumors have characteristic cytomorphology, it is sometimes difficult to differentiate them from neuroendocrine tumors of the pancreas. We reviewed cases of SPTP to delineate the diagnostic cytological features and also observed utility of CD 99 (MIC 2) immunostaining to aid in the diagnosis of this tumor. AIMS: This study was designed to demonstrate the utility of CD 99 immunostaining along with cytological features for making a pre-operative diagnosis and delineating it from the neuroendocrine tumor of pancreas which is a close mimic. MATERIALS AND METHODS: Cytomorphological features of 11 cases of solid pseudopapillary neoplasm diagnosed by pre-operative fine-needle aspiration cytology (FNAC) at our institute were reviewed. Immunocytochemistry for CD 99 was also performed on the smears. RESULTS: All the cases had cellular smears with monomorphic cells lying singly, as loosely cohesive clusters as well as forming delicate pseudopapillae. Presence of intra and extra-cellular basement membrane material, background foamy macrophages and nuclear grooves were the other salient features. Immunocytochemistry for CD 99 could be performed on eight cases and demonstrated typical paranuclear dot-like positivity. CONCLUSIONS: Pre-operative early diagnosis of SPTP can be made by FNAC which can further be aided by CD 99 immunocytochemistry.

Diagnosis and treatment of solid-pseudopapillary tumor of pancreas / 国际外科学杂志

Objective To study the clinic-pathological features,diagnosis,treatment,and prognosis of solid-pseudopapillary tumors of pancreas.Methods A retrospective clinical and pathological analysis was made on 10 patients(1 ∶ 4),admitted from January 2003 to December 2011.There were 2 male and 8 female patients,aging from 16 to 65 years with a mean of 32.6 years.Results The main symptom was mild abdominal pain (4/10).B-US,CT,MRI demonstrated that all tumors were located in pancreas (3 cases at the head,1 case at the neck,and the others at the body and tail),and well circumscribed,contained some degree of cystic degeneration.All the 10 patients had curative resections including,3 patients received standard duodenopancreatectomy,1 patients received tumor resection and pancreaticojejunostomy,2 patients received distal pancreatectomy,and 4 patients received distal pancreatectomy plus splenectomy.The specimens appeared as solid (3/10) or solid and cystic(7/10),and the biggest tumor diameter was between 3.3-14.0 cm with an average about 5.91 cm.Immunohistogically,the tumors were positive for Neuron-Specific Enolase (NSE,9/9)、Vimentin (9/9),CD56(7/7),Synaptophysin (Syn,5/8),Progesterone Receptor (PR,5/6),CD10 (4/6),Chromogranin A (1/4).Totally 10 cases were followed up with a average period of 36.6 months without tumor recurrence.Conclusions Solid-pseudopapillary tumor of pancreas is a rare tumor with low grade malignancy.Accurate diagnosis was difficult before operations.Surgical resection is recommended as the main treatment,and the prognosis is good.

Diagnosis and treatment of solid-pseudopapillary tmnor of pancreas: 10 cases / 中华内分泌外科杂志

Objective To explore the diagnosis and treatment of solid-pseudopapillary tumor of pancreas (SPTP).Methods The clinical data of 10 cases of SPTP treated from Feb.2003 to Feb.2009 in China-Japan Union Hospital of Jilin University were retrospectively analyzed.Results All the 10 cases underwent abdominal ultrasound examination and the accurate rate was 30％,and 6 of them underwent abdominal CT examination as well and the diagnosis accurate rate was 16.7％.All patients underwent surgery and pancreatic fistula occurred in 1 case after operation.There was no recurrence during the postoperative follow-up of 3-70 months.Conclusions SPTP has a low clinical incidence and it is frequently seen in young females.SPTP has no specific clinical manifestation.Imaging examinations play important roles in its diagnosis.SPTP can be found in any part of the pancreas.Resection is the first choice of treatment.The surgery extent depends on the position of SPTP and its surrounding tissues.If the intraoperative pathological examination shows the possibility of malignancy,radical surgery resection should be considered.

The management of solid-pseudopapillary tumor of the pancreas:a report of 4 cases / 中华内分泌外科杂志

Objective To discuss the management of solid-pseudopapillary tumor of the pancreas. Methods Four patients of solid-pseudopapillary tumor of the pancreas (SPT) were diagnosed by pathology. One patient was underwent magnetic resonance and two enhanced computer tomography scan. The mass was located in tuberculum (1 case), neck (1 case), and body-tail (2 cases) of pancreases respectively. Four cases were underwent duodenopancreatectomy, local resection of mass, resection of body-tail of pancreases respectively. The maximal diameter of tumor ranged from 5 cm to 16cm, averaged 10.3cm. The blood regular test and biochemistry were normal, and the tumor markers including AFP,CA19-9,CA125,CEA also were normal. The specimens were examined by pathology and immunohistochemistry. Results Four cases all were diagnosed solid-pseudopapillary tumor of the pancreas by pathology. At histologic analysis, the tumor was composed of uniform polygonal cells with moderate to abundant amphophilic cytoplasm and arranged in solid nests with areas of degeneration characterized by separation of the cells into pseudopapillary aggregates with intervening accumulation of mucopolysaccharide rich ground substance. The expressions of Vimentin, α-ACT, α-AAT were positive, and CgA negative. Four cases were followed up for six months and norecurred. Conclusions SPT of pancreases was a kind of tumor of low potential malignancy, and the magnetic resonance and computer tomography were often to be used. The effective therapy was thoroughly resection, and the prognosis in most patients is excellent.

Pathological characteristics of the solid pseudopapillary tumor of pancreas / 中华胰腺病杂志

Objective To summarize and analyze the pathological characteristics of solid pseudopapillary tumor of pancreas (SPTs).Methods The clinical data of 51 cases of SPTs were retrospectively analyzed.The immunohistochemical localizations of different markers (HSE,SYN,CD_(56),CD_(10),Nestin,Vim,a1-ACT,EMA,AE1/AE3 and CK19) on 39 SPTs were studied.Results Pathological features included a combination of solid and cystic components with pseudopapillae formation and degenerative regions without glands.Among the 39 cases of SPTs,the expression rate of NSE was 97.4%,the expression rate of CD_(56),CD_(10) was 84.6%,the expression rate of Nestin and Vim was 64% and 87%,the expression rate of S100 was 79.5%,the expression rate of a1-ACT and a1-AT was 82.1% and 79.5%,while the expression rate of SYN was 12.8%;however there was low expression and weak positive reaction of EMA,AE1/AE3 and CK19.Conclusions The typical pathological characteristics of SPTs may result from gradual degenerative changes induced anoxemia in some SPT's areas.The heterogeneity of SPTs on different antibody markers showed that the SPTs may be originated from pancreatic embryonic stem cells,and result from immature differentiation of the pluripotential stem cells during pancreatic genesis.

Clinical and pathological characteristics of the solid pseudopapillary tumor of pancreas / 国际外科学杂志

The solid pseudopapillary tumor of pancreas (SPT) is a rare pancreatic tumor with low malignant potential. It primarily affects young women. The clinical characteristic is nonspecific, but the pathological characteristic is distinct. The tumor consists of both cystic and solid component. The typical histopathological appearance of SPT is the pseudopapillary structure with a fibrovascular surrounded by several layers of epithelial cells.Only a few SPTs present invasive behaviors. The tumor cells express a wide range of immunohistochemical markers. The pathogenesis is associated with the Wnt signaling pathway, which is changed by β-catenin, Pl20-catenin, adenomatous polyposis coli gene( APC), the cyclin-dependent kinase inhibitors p21 and p27.

The solid-pseudopapillary tumor of pancreas:the clinical characteristics and diagnosis / 中国医师进修杂志

Objective To study the clinical characteristics and diagnosis of the solid-psendopapillary tumor of pancreas (SPT).Methods The clinical data of 40 SPT from January 1996 to January 2008 were retrospectively analyzed. The average age was (32.9 + 13.6 )years. The average clinical course was (8.6±0.1) months.Clinical symptoms usually included distensible pains and secret anguish in abdomen (60.0%).No jaundice appeared in any case.Results The surgical resection was favorable for the treatment of SPT,which had excellent prognosis.No tumor recurrence were found in those following-up patients. Grossly,the cut surface showed areas of solid and papillary tissue,cystic degeneration,hemorrhage,and necrosis.Pathological features included a combination of solid and cystic components with pseudopapillae formation and degenerative regions without glands.Conclusions SPT has its uniquely clinical and pathological characteristics.Its main diagnosed points are helpful for clinical doctors to make timely diagnosis and reduce the rate of misdiagnosis and mistreatment.

Diagnosis and treatment of solid-pseudopapillary tumor of pancreas / 中华消化外科杂志

Objective To summarize the clinical experience of diagnosis and treatment of solid-pseudopapillary tumor of pancreas(SPTP),and to work out the diagnosis and treatment process which matches the conditions of China.Methods The clinical data of consecutive 50 patients with SPTP who had been admitted to our hospital from January 2001 to March 2007 were retrospectively analyzed.Of all patients,3 were male and 47 female.Their median age Was 24 years(13-60 years).Preoperative imaging examination revealed typical findings and tumor markers were negative.SPIP of 23 cases were located in the head of pancreas,3 in the neck of pancreas,2 in the uncinate process of pancreas.3 in the body of pancreas,18 in the tail of pancreas,and the location of SPTP of 1 case was uncertain.Forty-eight patients underwent the operation of SPTP resection,1 of exploratory laparotomy and 1 of CT-guided fine-needle aspiration biopsy of metastatic lesions.Results Forty-nine out of 50 patients were diagnosed"pancreatic mass"preoperatively.Eighteen of the 32 patients with intact tumor capsule and 11 of the 16 patients without intact tumor capsule underwent operation without pathological examination.Thirty-two patients were followed up for 3-55 months,and no metastasis or invasion of the SPTP was observed.Conclusions Yong females are in the high risk group of SPTP.CT is the most valuable preoperative imaging test.Complete surgical resection is the first choice treatment with good prognosis.Resection of the head of pancreas with the preservation of duodenum and resection of body and tail of pancreas+splenectomy are the commou modalities used for the treatment of SPTP.The intactness of the tumor capsule is an important factor in making surgical plan and deciding the necessity of intraoperative frozen section pathological examination.The most common postoperative complication of SPTP resection is pancreatic fistula.

CT Misdiagnosis of Solid-Pseudopapillary Tumor of Pancreas / 医疗卫生装备

Objective To analyze the reasons of CT misdiagnosis of solid-pseudopapillary tumor of pancreas (SPTP), including wrong orientation, mislocation. Methods The literatures on SPTP were discussed. Results Lesions of SPTP could appear at any position of the pancreas. Conclusion The result is very significant for guiding treatment and judging prognosis.