Solid pseudopapillary tumor of the pancreas: a single-institution 20-year series of pediatric patients.

PURPOSE: Solid pseudopapillary tumor (SPT) of the pancreas is a rare neoplasm. The objective of this study was to review our institution's experience and provide an update on current management in the pediatric population. METHODS: Our pathology database identified all patients with SPT for a 20-year period (1991-2011). Demographics, clinical characteristics, operative details, pathology, and outcomes data were retrospectively reviewed. RESULTS: Eleven patients with SPT were identified. Most were female and Hispanic. Median age at resection was 14 years (9-17 years). Most patients presented with abdominal pain. Diagnostic imaging was most commonly an ultrasound or computed tomography. All tumors were resected en bloc. Median greatest tumor diameter was 5 cm (3.5-12 cm). Median length of stay was 8 days (5-19 days). Complications included pancreatic leak, chyle leak, delayed gastric emptying, fat malabsorption, and incisional keloid. Recurrence developed after 2.5 years in 1 patient with positive surgical margins. There were no metastases or deaths. Median follow-up was 1.4 years (0.6-5.9 years). CONCLUSION: This pediatric series of SPT from a single institution corroborates previous reports in the literature. In our experience, SPT behaves like a low-grade malignancy and has an excellent prognosis. Surgical resection is dictated by tumor location and remains the treatment of choice.

[Solid pseudopapillary pancreatic neoplasm: report of five cases]. / Neoplasia sólida seudopapilar del páncreas: comunicación de 5 casos.

Solid pseudopapillary pancreatic neoplasms (SPPN) are rare entities mainly diagnosed in young women. Prognosis is generally relatively favorable. Histological evaluation usually reveals papillary as well as solid areas of low malignant potential, as the name implies. A retrospective review of five cases, diagnosed and treated in our institution, is presented with the corresponding clinical and pathological data and follow-up. All SPPN occurred in women, with an average age of 19 years (13 to 27 years), who consulted for abdominal pain. In all patients, the tumors were > 4cm, distributed in several pancreatic segments. The histopathologic report confirmed papillary as well solid areas and low mitotic count. In two patients, neural and angioinvasion were found.

[Intraductal papillary mucinous tumor: diagnostic and therapeutic approach]. / Tumor papilar, mucinoso e intraductal: abordaje diagnóstico y terapéutico.

Primary cystic pancreatic neoplasms are rare tumors, with an approximate prevalence of 10% of cystic pancreatic lesions. Most of these lesions correspond to mucinous cystic neoplasm, serous cystoadenoma and intraductal papillary mucinous tumor (IPMT). IPMT is characterized by diffuse dilatation of the main pancreatic duct and/or side branches with inner defects related to mucin or tumor, or mucin extrusion from a patent ampulla. IPMT has a low potential for malignancy, with a low growth rate, a low rate of metastatic spread and postsurgical recurrence. Over the last few years, major advances have been made in the diagnostic and therapeutic management of this tumor.

Pancreatic solid-cystic-papillary tumor: clinicopathologic features in eight patients from Hong Kong and review of the literature.

Solid-cystic-papillary tumors (SCPTs) of the pancreas are rare. The clinicopathologic features and pathogenesis of these tumors have attracted a number of investigations, but the results remain unclear. We investigated the clinicopathologic data, immunohistochemical expression of the pan-endocrine markers, hormones, steroid receptors, and p53 overexpression in pancreatic SCPTs from eight Chinese patients (seven women, one man) collected over a 24-year period. They accounted for 2.5% of the primary pancreatic tumors. The tumors were seen in young women (mean age 27 years). They were large (mean size of resected tumor was 8.4 cm), benign, had solid and cystic areas, and were evenly distributed in the pancreas. The main differential diagnosis was pancreatic endocrine tumor. The tumors were negative for pan-endocrine markers, hormones, estrogen receptor, progesterone receptor, and p53. To date, 452 pancreatic SCPTs have been documented in the English literature. They occurred in patients of different ethnic groups, particularly in non-Caucasians. The tumors were frequently noted in young females. Uncommon cases of malignant pancreatic SCPTs were often found in older men and had indolent behavior. It was concluded that pancreatic SCPTs have distinct clinicopathologic characteristics. The present observations, together with a review of the literature suggests that overexpression of p53 or estrogen receptor is not important in the pathogenesis of pancreatic SCPTs.

Solid cystic tumor of the head of the pancreas in a young woman.

This a case report of a solid papillary tumor of the pancreas in a young woman of 18 years, who was referred to after having suffered for a period of 8 months with a rather vague symptomatology, characterized by dyspepsia, fatigue and, towards the end of the 8 month period, weight loss (approximately 2 kg). In the last week, as a consequence of a modest abdominal trauma, the patient was submitted to abdominal CT that showed a burden at the head of the pancreas, demonstrating a round neoformation about 6 cm in diameter with solid echogenicity slightly hypodense. Subsequently, she underwent an operation with the diagnosis of pseudocystis of the pancreas. During surgery, a big cystic formation of the head of the pancreas, into which a drain was introduced, was revealed. The histological postoperative examination was compatible with pancreatic tumor with a low grade of malignancy, cystic papillary or solid papillary type. Therefore, the patient came under our observation and underwent an operation of pancreatoduodenectomy. Two years after the operation, the patient had completely recovered. In this case, we discussed the problem of performing certain preoperative diagnoses despite the aid of modern diagnostic imaging, this being a very rare illness that almost exclusively plagues young women (median age 19 years). This diagnosis has an uncertain histological origin and is generally accompanied by a modest and vague symptomatology. The surgical procedure, given the low grade of malignancy of the neoplasm and the excellent long-term prognosis, must be, with respect to the oncological radicality, as conservative as possible.

Operative management of papillary cystic neoplasms of the pancreas.

BACKGROUND: Papillary cystic neoplasm (PCN) is a rare malignant tumor of the pancreas that typically occurs in young females and has an excellent prognosis. STUDY DESIGN: We report a retrospective review of 12 patients treated during a 16-year period. Pre-, intra-, and postoperative data were evaluated in all patients to determine optimal management with specific reference to surgical strategy. RESULTS: All 12 tumors occurred in young women (mean age 22 years, range 14-36 years). Six patients presented with an epigastric mass, and three with severe abdominal pain. The correct diagnosis was made preoperatively in only five patients. Incorrect diagnoses included hepatoma, pancreatic pseudocyst, and hydatid cyst. The PCNs had a mean diameter of 12.5 cm (range 8-20 cm), and occurred in the head (four), neck (three), body (three), and tail (two) of the pancreas. All were resected. Operations performed were pylorus-preserving pancreaticoduodenectomy (three), central pancreatectomy with pancreaticogastrostomy (three), distal pancreatectomy (three), and local resection (three). In one patient two liver metastases were resected in addition to the pancreatic primary. One patient presented with tumor rupture and a major bleed into the lesser sac and died of multiple organ failure after resection. Postoperative complications included a stricture at the hepaticojejunostomy after pancreaticoduodenectomy, which resolved after temporary stenting, and a pancreatic duct fistula after local tumor resection, which required a distal pancreatectomy. Eleven patients are well at followup (mean 6.6 years; range 6 months to 15 years). CONCLUSIONS: PCN should be considered in the differential diagnosis of large pancreatic masses, especially in young females. Conservative resection, where technically feasible, is safe and effective and represents the therapy of choice.

Experience with papillary and solid epithelial neoplasms of the pancreas in children.

BACKGROUND: Papillary cystic tumour of the pancreas in children is a rare tumour. METHODS: Ten patients admitted to The Hospital for Sick Children, Toronto, have been reviewed, and presentation and management are documented. RESULTS: One patient who had disseminated disease at presentation died. CONCLUSION: Excision of the tumour irrespective of size is recommended.

Serial serum CA 125 measurements for evaluation of recurrence in patients with endometrial carcinoma.

OBJECTIVE: To evaluate the usefulness of serum assays for CA 125 to detect recurrent endometrial carcinoma. METHODS: Two hundred sixty-six patients were studied with 1101 post-treatment assays. Patients were categorized as low, medium, or high risk based on surgical-pathologic findings. CA 125 values were analyzed with respect to each patient's disease status. RESULTS: Serial CA 125 levels were elevated (greater than 35 U/mL) in 19 of 33 patients (58%) with recurrent disease. Among 236 surgically treated patients, 97 (41.1%), 42 (17.8%), and 97 (41.1%) were considered low, medium, and high risk, respectively. None of the low-risk and only two (4.7%) of the medium-risk patients developed recurrent disease. One of the latter patients was detected based on an elevated CA 125 level alone. Twenty-seven (27.8%) of the high-risk patients developed recurrent disease, 23 of whom had elevated pre-treatment CA 125. Fifteen of 16 (94%) with recurrent disease had an elevated CA 125 level. Nine of 12 patients with papillary serous carcinoma experienced recurrence; eight of these nine had elevated CA 125 levels at diagnosis and recurrence, in contrast to only one patient with a normal pre-treatment level (P = .018). False elevations were noted in 13 patients, 12 of whom had received radiation therapy. CONCLUSIONS: CA 125, if elevated at diagnosis of endometrial carcinoma, is an important marker for recurrent disease. The use of serial CA 125 assays is most beneficial in diagnosing recurrence in a high-risk population, including patients with papillary serous carcinomas. False elevations may occur following radiation therapy.

[Endometrial carcinoma. The experience of the Hospital San Juan de Dios]. / Carcinoma endometrial. Experiencia del Hospital San Juan de Dios.

Since april 1957 to april 1986, the Gynecologic Oncologic Unit of San Juan de Dios Hospital, Santiago, registered 255 cases of endometrial carcinoma. 48 cases where clinically etapified because of no previous pelviabdominal surgical exploration in: Stage I: 27 cases (56.3%); II: 6 (12.5%); III: 7 (14.6%); IV: 1 (2.1%). The remainder 207 cases were surgically etapified: I: 155 cases (74.9%); II: 10 (4.83%); III: 31 (14.92%) and IV: 11 cases (5.32%). Fourteen patients of the first group and 2 of the second one received palliative therapy. Primary therapy was performed with curative intention in 237 cases (34 clinically etapified and 203 surgically etapified). Stage I: 181 cases (76.37%), (26 clinical, 155 surgical); Stage II: 16 (4.92%). (6.10); Stage III: 31 (14.33%), (2.29) and Stage IV: 9 cases (4.4%), (0 + 9). Four different kinds of therapies were performed: A: only surgery in 153 cases (65%); B: surgery plus radiation in 49 (21%); C: radiation plus surgery in 20 cases (8.44%) and D: only radiation in: 15 cases (6.33%). Five years survival with no evidence of disease (SNFD) in 116 cases (70.04% of the 237 patients), and 58 cases (24.47%) died by endometrial cancer; 11 (4.64%) by intercurrent disease and 2 (0.84%) were lost from follow up. Survivor's distribution was: Stage I: 140 (77.34%); II: 12 (75%); III: 14 (45.16%). Five years survival according to therapy: A: 114 (74.51%); B: 36 (73.5%; C: 15 (75%); D: 1 (8.66%). In Stage I distribution of survivor was: A: 83.1%; B: 74.2%; C: 81.25%; and D: 10%.(ABSTRACT TRUNCATED AT 250 WORDS)