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Intraductal papillary mucinous neoplasms (IPMNs) of the pancreas are intraductally located, mucin-producing cystic neoplasms of the pancreas with a malignant potential. We report about a 54-year-old female who underwent segmental resection of the pancreas for non-invasive IPMN. The surgical margins were tumour-negative. Three years later a highly suspicious tumour of the pancreatic tail was detected during routine follow-up. Resection of the pancreatic tail was performed. The histological analysis revealed an adenocarcinoma. This case suggests the development of a pancreatic carcinoma from a non-invasive IPMN and raises the question about the extent of surgery of non-invasive IPMNs of the pancreas.

Cystic and neuroendocrine pancreatic neoplasms are quite rare tumors which diagnosis is often difficult due to their non specific symptomatology and limited diagnostic accuracy of conventional diagnostic instruments. Their treatment is still controversial.A young woman is admitted with abdominal pain and dyspepsia. Instrumental diagnosis reveals a cystic pancreatic lesion which seems to be malignant as CEA of pancreatic liquid is increased. The patient undergoes distal spleno-pancreatectomy and postoperative histological examination found IPMN associated with MCN and furthermore there was occasional diagnosis of a small neuroendocrine tumor in the pancreatic tail.A correct diagnostic approach is indispensable for a correct therapeutic approach. Radical surgical treatment is indicated in all cases of main- and branch duct IPMN and in case of MCN with signs of malignant transformation. Surgical treatment is also the gold standard for pancreatic neuroendocrine tumors if they are singular and in M0 stage.In case of cystic and neuroendocrine neoplasms of the pancreas the preoperative diagnostic approach is often not enough to obtain a definite diagnosis. Review of literature and this case report show that a definite diagnosis can only be done after postoperative histological examination. Recent prospective studies suggest the possibility for a follow up instead of surgical treatment for some neuroendocrine tumors.

Solid pseudopapillary tumor (SPT) of the pancreas is a rare pancreatic tumor mostly seen in young women. We here report a twelve-year-old girl presenting with recurrent attacks of pancreatitis. No history of a systemic disease, trauma, drug usage or infection was present. All other etiologic factors like familial, hypertriglyceridemia, hypercalcemia, cystic fibrosis, medications were excluded. On abdominal ultrasound a heterogeneous mass was noticed at the tail of pancreas. Computerized tomography and magnetic resonance imaging proved that the mass was cystic. The mass was surgically removed. The diagnosis was pancreatic solid cystic papillary epithelial neoplasm. Although acute pancreatitis due to SPT was exceptionally reported, this is the first description of SPT leading to recurrent pancreatitis especially in children.

Cystadenomas are a rare, painless, and slow-growing benign epithelial tumor of the salivary gland. This article describes the case of a papillary cystadenoma in the lower lip of a 46-year-old man. The lesion was removed using a carbon dioxide laser, and there were no signs of recurrence 1 year postoperatively.

We present a case of a 13-year-old boy with a left-sided abdominal mass which proved to be a papillary cystic neoplasm of the pancreas. This low-grade malignant lesion of young patients is very rare, and exceedingly rare in males. The prognosis following resection of this tumor is good. We present the ultrasound and computed tomographic picture of this lesion, as well as the gross and microscopic pathology.

A case of papilloferous serous cystadenoma of boundary malignancy in a 29-year old female patient, with VCS syndrome--status post phlebothrombosis ileofemoralis dextra. Thrombosis vena cava superior is presented.

We discuss a case of 25 year old male who was evaluated for primary infertility following marriage. He had no previous history of urogenital complaints or abnormalities. In fact, his past medical history was unremarkable. On examination epididymal masses were found. Semen analysis showed azoospermia. Right epididymal mass was surgically excised. Histopathology showed an epididymal neoplasm composed of simple and complex papillary processes lining and filling the ducts. The tumor cells were clear and positive for cytoplasmic glycogen. Immunohistochemistry was also supportive. Diagnosis of clear cell papillary cystedenoma was made based on histopathological and immunohistochemical features.

Aggressive papillary tumors of the middle ear are rare, and their true origin is not clear. We describe the clinical, radiologic, genetic, and histopathologic features of a papillary epithelial tumor filling the middle ear of a 68-year-old woman. Imaging revealed no evidence of petrous temporal bone apex involvement, nor did genetic studies demonstrate the von Hippel-Lindau mutation. A literature search revealed 24 previously reported cases of such a papillary epithelial tumor of the middle ear. All except 2 cases demonstrated apical petrous temporal bone invasion on imaging, and it has been suggested that they arose from a primary endolymphatic sac tumor, which has a similar papillary epithelial histology. Substantial numbers of cases of papillary epithelial tumors involving the middle ear are reported to be associated with von Hippel-Lindau disease, as are known cases of endolymphatic sac tumor. This is the third reported case of papillary epithelial tumor of the middle ear that does not show apical petrous temporal bone invasion on imaging, suggesting that such neoplasms do not always arise from a primary in the endolymphatic sac.

Solid pseudopapillary tumor (SPT) of the pancreas is a rare exocrine pancreatic tumor behaving in a low-grade fashion, with limited local invasion risk and a rare metastatic evolution. We report SPT in two young females, revealed by abdominal pain and an epigastric mass. The diagnosis of a cystic tumor was based on abdominal ultrasound and CT data in the first case and on MRI in the second. A distal pancreatectomy and splenectomy were successfully performed in the first case and a central pancreatectomy in the second. Histological study confirmed the diagnosis of SPT of the pancreas.

Cystadenoma and cystadenocarcinoma are rare salivary gland tumours histologically characterized by prominent cystic and frequently papillary growth. We present two cases of cystadenoma of a minor salivary gland (upper lip) and parotid cystadenocarcinoma respectively, captured between 834 salivary gland tumors studied in our hospital from 1980 to 2004. The authors review the clinical, histological, and biological features of these two unusual tumours, and differential diagnosis with other salivary glands neoplasms. Both entities usually reveal papillary proliferation of the epithelial lining and are composed of cells that possess bland cytomorphologic features. Differentiation of tumour types depends largely on the identification of actual infiltration of salivary gland parenchyma or surrounding connective tissue by either cystic or solid epithelium in cystadenocarcinomas. Step sections of a borderline tumour may yield unequivocal evidence of invasion. The authors discuss the problematic diagnosis between these rare neoplasms and with other tumours and compare histological findings of these two entities.

Preoperative diagnosis of pancreatic cystic neoplasms is problematic. We evaluated our experience with endoscopic ultrasound (EUS) to determine the utility of fine-needle aspiration cytology (FNAC) in surgical decision-making.Patients evaluated for pancreatic cysts with EUS fine-needle aspiration (FNA) from 3/1996-10/2003 were included. Patients undergoing both preoperative EUS-FNA and pancreatic resection were identified. FNAC read as a mucinous cystic neoplasm (MCN), suspicious for neoplasia, or mucinous epithelial/atypical cells were classified as "concerning." Cytology with no malignant cells was negative. FNAC read as indeterminate, atypical cells of undetermined significance, or possible contamination was nondiagnostic.Of 95 patients evaluated with EUS FNAC, 29 underwent resection. On final pathology, 7/29 lesions (24%) were malignant [two neuroendocrine tumors, three adenocarcinomas, one invasive intraductal papillary mucinous neoplasm (IPMN), and one metastatic uterine tumor], 4/29 (14%) were benign (three serous cystadenomas and one chronic pancreatitis), and 18/29 (62%) were premalignant (ten MCNs and eight IPMNs). Seven patients had concerning FNAC. All seven harbored malignant or premalignant lesions. Nine patients had negative FNAC: three (33%) with benign lesions and six (67%) with premalignant lesions. Thirteen of the 29 patients (45%) had nondiagnostic FNAC with 12/13 (92%) harboring a malignant or premalignant lesion. Sensitivity, specificity, positive predictive value, and negative predictive value were 28%, 100%, 100%, and 18%, respectively.The decision to proceed with nonoperative management should not be based on a negative or nondiagnostic FNAC alone, as 67% of negative and 92% of nondiagnostic specimens were associated with malignant or premalignant pathology.

The most common pancreatic tumour is the ductal adenocarcinoma. Many other benign and malignant pancreatic neoplasms have to be recognised and now account for more than 50 % of the pancreatic lesions seen in our daily routine. An improved differential diagnosis is, therefore, mandatory and will be discussed in this review.

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.

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.

Laparoscopic hepatectomy has been widely performed for patients with benign liver tumors such as hepatic hemangioma, focal nodular hyperplasia, and hepatic adenoma. We here present a case of a 78-year-old female patient who was initially admitted to our department due to fever and jaundice for 2 days. Abdominal enhanced computed tomography scan showed intrahepatic and extrahepatic bile duct dilatation with liver atrophy of left lobe. Unenhanced nodules were seen within the left intrahepatic bile duct. Ultrasonography revealed intrahepatic and extrahepatic bile duct dilatation with viscous fluid, tubular adenoma? Tumor markers including alpha fetoprotein, carcinoembryonic antigen, and CA19-9 were normal. Preoperative total bilirubin was 64.4 mmol/L. Laparoscopic anatomical left hepatectomy and common bile duct exploration were performed. In this procedure, a lot of mucus was seen within the common bile duct and left intrahepatic bile duct. No bile duct stones were found after the exploration. During parenchymal transection, intraoperative hemorrhage from middle hepatic vein was met, and we repaired middle hepatic vein by laparoscopic suture (5-0 Prolene). No air embolism and hypotension were met. This operation took 232 minutes and estimated blood loss was 300 mL. Postoperative ultrasonography indicated a normal outflow of middle hepatic vein and there was no stricture. The patient's postoperative course was uneventful and was discharged on the 6th day after surgery. Postoperative pathological diagnosis was intrahepatic bile duct papillary mucinous cystadenoma.

We identified the platelet derived growth factor receptor (PDGFR) as a potential target in epithelial ovarian carcinoma (EOC). This led us to test whether inhibition of the PDGFR affects ovarian cancer cell proliferation and survival and regulates other processes critical to tumor growth and metastasis. We postulated that there is a correlation between the PDGF-PDGFR axis and the secretion of VEGF in EOC. VEGF secretion in ovarian tumors, cancer cells, serum and ascites fluid was measured by IHC, Western Blot and ELISA. We found increased VEGF expression and secretion in most ovarian tumors (by IHC), in EOC malignant ascites and in the conditioned media of primary ovarian cancer cells (quantified by ELISA). In malignant ascites, the levels of secreted PDGF BB and VEGF were strongly correlated (Pearson coefficient of correlation R = 0.728), suggesting that the two pathways interconnect. In PDGFR expressing immortalized ovarian cancer cells, PDGF potently induced VEGF secretion, while imatinib mesylate (Gleevec), a partially selective PDGFR inhibitor, reduced PDGF stimulated VEGF production to basal state. In ovarian cancer cells overexpressing constitutively active Akt, imatinib inhibited partially VEGF secretion, suggesting that the PI3K/Akt pathway is implicated in PDGF-stimulated VEGF secretion. In summary, these results suggest a correlation between the PDGF and VEGF networks in ovarian cancer cells and tumors. The effects of imatinib on VEGF secretion in tumor cells may affect the tumor microenvironment in a manner detrimental to tumor progression.

Syringocystadenoma papilliferum, a hamartoma with mostly sweat gland, but also follicular infundibular elements, is described on the flank and head of two young cats. Clinically, lesions were cutaneous plaques characterized by irregular but sharply demarcated borders and a roughened, hyperpigmented surface. Complete surgical excision in one case was curative. Histologically, the lesion was limited to the superficial dermis and consisted of coalescing units of proliferating sweat glands. The proliferation was tubular or papillary, and may have been epitrichial, opening within dilated hypertrophied follicular infundibuli, or atrichial. Three types of epithelium were observed, recapitulating the formation of the follicular-sweat gland unit with infundibular, ductal and secretory epithelia. The glands reacted positively for alpha cytokeratin 8 and were supported by fibrous tissue with a plasmacytic, lymphocytic and neutrophilic infiltrate. As in humans, this lesion may be classified within the hamartoma-nevus-type category.

Solid-cystic papillary tumors (SCPTs) of the pancreas are unusual primary neoplasms with low potential for malignancy and unknown cell origin. These tumors are seen mostly in young women with distinct clinicopathologic characteristics. The tumor is encapsulated and has a favorable prognosis after complete surgical resection.1 There have been some reports that contrast-enhanced ultrasonography (CEUS) is useful in the diagnosis of neoplasms occurring in the pancreas, 2 but to our knowledge, there have been no reports of CEUS evaluation of SCPTs. We report a case of the CEUS features of an SCPT in a female patient.

To estimate the clinical and pathological features of pancreatic solid cystic papillary tumor (SCPT) in children.From 2000 to 2005, 8 cases with SCPT of the pancreas were analyzed retrospectively. All cases but one were females. Average age was 12.8 years. By case review, we discussed the clinical and pathological features of SCPT in children.The chief complains were abdominal pain and palpable mass. There were 3 cases in the head, 1 case in the body, and 4 cases in the tail of pancreas. The procedures employed included local resection (1 case), distal pancreatectomy (5 cases), pancreaticoduodenectomy (1 case), and biopsy (1 case). Histological examination showed solid with cystic areas and papillary protrusions in the 8 cases; as for immunohistochemical examinations, the positive rate was 100% for alpha-antitrypsin (AACT), 87.5% for vinmentin, and 62.5% for neuron-specific enolase (NSE). The patients were followed up for 2 months to 4 years but one was lost by follow-up and all were alive postoperatively. SCPT in 2 cases relapsed.Occurring predominantly in young females, SCPT is usually curable by surgical resection with a favorable prognosis.

A 74-year-old man who consulted our hospital complaining of painless left scrotal swelling. Computed tomographic demonstrated a left epididymal tumor in the hydrocele. We performed left high orchiectomy. Microscopically, several microcysts were found and tumor cells showing a papillary growth pattern were found in these microcysts. We finally diagnosed the patient as having papillary cystadenoma of the epididymis. Papillary cystadenoma of the epididymis is rare epididymal benign tumor. To our knowledge, 19 cases have been reported in Japan between 1976 and 2000. It is clinically important to determine whether or not the tumor is associated with von Hippel-Lindau disease. In our case, there was no evidence of von Hippel-Lindau disease and the patient has remained well with no complication to date.

Solid pseudopapillary tumor of pancreas (SPT) is a rare neoplasm that occurs most often in young females with the two distinct features, the 'solid-cystic' gross appearance, and the 'solid-pseudopapillary' microscopic pattern. It has been reported that almost all SPT tumors contain a mutation in the beta-catenin gene; however, the histogenetic origin of this tumor remains largely a mystery. E-cadherin is a cell adhesion molecule that links to catenins to form cell adhesion junctions, which is associated with the cytoskeleton formation. In this study, we examined the expression of E-cadherin and beta-catenin from SPT in an attempt to determine the molecular basis for the unusual morphology of this tumor. Nine cases of SPT were retrieved from Surgical Pathologic Archives of three institutions, including one male and eight females. H&E slides of each case were reviewed to confirm the diagnosis. The beta-catenin gene was sequenced in one case. E-cadherin and beta-catenin immunostains, were performed on all nine cases. Sequencing analysis on one case showed a point mutation of the beta-catenin gene, confirming previous findings that almost all SPT tumors contain mutation in the beta-catenin gene. Immunostains showed that, in both solid and pseudopapillary areas, all the tumor cells lost expression of E-cadherin, and beta-catenin nuclear expression was observed in all cases. Our findings suggest that loss of cytoplasmic beta-catenin protein in the cell adhesion complex due to beta-catenin gene mutation, results in instability of the complex, loss of E-cadherin in cell membrane, and eventually dissociation of the tumor cells to form the pseudopapillary pattern.

Vascular endothelial growth factor (VEGF) is a hypoxia inducible angiogenic and vascular permeability factor. Although VEGF expression in glioblastoma is induced by hypoxia, its expression in renal cell carcinoma and hemangioblastoma is thought to be related to mutation of the von Hippel-Lindau (VHL) gene. It is not certain whether other lesions in VHL syndrome are associated with an elevated VEGF level. We report a VHL syndrome patient with multiple hemangioblastomas and bilateral epididymal clear cell papillary cystadenomas. In situ hybridization revealed high levels of VEGF mRNA in the clear cells of the epididymal tumor and the stromal cells of the hemangioblastoma. This lends support to the notion that upregulation of VEGF is caused by loss of the wild-type VHL protein. We postulate that the elevated VEGF levels may account for the cyst formation and vascularized stroma present in these VHL-associated tumors.

Solid-pseudopapillary tumor (SPT) is an unusual pancreatic neoplasm that is characterized by a mixture of solid and cystic components and a fibrous capsule. Recently, the tumorigenesis of SPT has been reported to be associated with gene mutations of beta-catenin, which is a molecule participating in the Wnt signaling pathway. Reported herein is the case of a 53-year-old woman with SPT. The tumor, approximately 3 cm in diameter in the pancreas body, had a clear margin and central calcification but had neither a cystic component nor fibrous capsule. Several lines of pathological findings in the surgically resected specimen indicated SPT: (i) pseudopapillary proliferation of eosinophilic polygonal cells with oval nuclei; (ii) positive expression of several marker molecules indicating differentiation into acinar and endocrine cells; and (iii) zymogen granule-like structures in the cytoplasm on electron microscopy. Further, the tumor cells had intense nuclear accumulation of beta-catenin and an activating mutation, (34)Gly(GGA) to Arg(AGA), in exon 3 of the beta-catenin gene, as previously reported in most SPT. These findings suggest that association of the beta-catenin phenotype with development of the rare phenotype of SPT, a non-cystic and unencapsulated tumor, is unlikely.

Laparoscopic resection of benign tumors of the pancreas has been reported in adults, but only four cases of partial laparoscopic pancreatectomy in children have been described in the English-language literature.We describe the case of an 11-year-old girl with a solid pseudopapillary tumor who was treated with a laparoscopic, spleen-preserving, distal pancreatectomy. The specimen was extracted in an endoscopic bag retrieval system through a Pfannenstiel incision. Operative time was 120 minutes, and minimal blood loss occurred. The literature is reviewed.The postoperative course was uneventful. Twenty-two months after the operation, clinical follow-up (including assessment of exocrine and endocrine pancreatic function) revealed nothing abnormal. The functional and aesthetic results were satisfactory.The technique used for our case is simple and reproducible, was completed safely within a reasonable operative time, and yielded a good result.

Solid and pseudopapillary tumor of the pancreas is a benign and low malignant potential tumor. Prognosis is good after surgical resection but its malignant potential is usually defined after metastasis. We compared benign and malignant cases with clinicopathological, immunohistochemical and DNA flow cytometric studies.From January 1991 to July 2004, seven patients were found to have solid and pseudopapillary tumor of the pancreas at Taipei Veterans General Hospital. The paraffin sections were reevaluated with hematoxylineosin stain, immunohistochemical stains, and DNA flow cytometric studies.It included 6 benign and one malignant case. The progesterone receptor, vimentin, neuron-specific enolase, and chromogranin A showed diffused positive stain in all cases. Estrogen receptor and P53 stain were negative in all 7 patients. Synaptophysin stain was negative in 6 no recurrence patients, but was positive only in the patient who suffered from recurrence. DNA flow cytometry showed diploid results in six non-malignant tumors. In the malignant patient, the tumor in the first operation showed diploid result, but tumors in second and third operations showed aneuploidy.Solid and pseudopapillary tumor of the pancreas should be considered as a potentially malignant disease in all patients and regular follow-up is mandatory.

A case of papilloferous serous cystadenoma of boundary malignancy in a 29-year old female patient, with VCS syndrome--status post phlebothrombosis ileofemoralis dextra. Thrombosis vena cava superior is presented.

Solid cystic papillary tumor (SCPT) of the pancreas in children is rare with limited references of its clinical features and treatment. Therefore, to estimate the clinicopathologic features of SCPT of the pancreas in children is necessary.Clinical data of 8 children with pathologically confirmed SCPT of the pancreas, treated from 2000 to 2005 with surgery, were analyzed retrospectively. The clinicopathologic features were analyzed with literature review.All patients were girls but one, with the average age of 12.8 years. The chief complaints were burbulence, abdominal pain, and palpable mass. The tumors were mainly located in the head or tail of the pancreas. Of the 8 patients, 1 received local resection, 5 received distal pancreatectomy, 1 received pancreatico-duodenectomy, and 1 received only biopsy. Seven patients were followed-up for 2 months to 4 years and were still alive; 2 patients suffered recurrence. All cases showed solid with cystic areas and papillary protrusions. Immunohistochemically, the positive rates were 100% for alpha-antitrypsin, 87.5% for vinmentin, and 62.5% for neuron-specific enolase.SCPT of the pancreas occurs predominantly in young girls, and its pathogenesis is still unknown. Surgical resection is usually curative, and the prognosis is good.

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.

Solid pseudo-papillary tumor of the pancreas is a rare tumor that occurs predominantly in young women. It often presents as a big abdominal mass whose preoperative diagnosis appears to be difficult. It is a low- grade malignancy tumor and surgery is the mainstay of treatment. The Authors report the case of a 40 year-old woman with an abdominal mass supposed to origin from the mesenterium, diagnosed as a solid pseudo-papillary tumor of the pancreas after histological examination and treated by enucleation. Moreover literature data about epidemiology, histogenesis, diagnosis and treatment are analysed.

Fine needle aspiration cytological features of solid-cystic tumor (SCT) of pancreas occurring adjacent to pancreas is reported for its rare occurrence. On cytology, diagnosis of paraganglioma was suggested. After histologic diagnosis of SCT, cytology was reviewed that showed typical features of SCT. On cytology, diagnostic problem in SCT occurring outside pancreas is illustrated.

Solid and cystic papillary tumor of pancreas (SCPT) are rare tumors. Of the well described cases 95% have occurred in women of reproductive age, suggesting a role of hormonal factors in the pathogenesis of this tumor. Only few studies evaluating estrogen and progesterone receptor status have found it to be positive. Cytohistocorrelation and immunohistochemistry for estrogen (ER) and progesterone (PR) receptors of three such cases are reported here. All three were diagnosed by fine needle aspiration cytology (FNAC) and showed cytoplasmic positivity for ER and PR receptors. May-Grunwald Giemsa, Papanicolaou and hematoxylin and eosin stained slides were evaluated for cytology and histopathology. Unstained methanol fixed slides were used for immunohistochemistry. The cytoplasmic staining of ER and PR receptors cannot be underestimated, as shown by Carbone A et al which represents ERII receptors. This study confirms the presence of ER and PR receptors in these tumors and highlights the already established cytological features.

A retrospective study of anal sac tumours without pulmonary metastases, from the author's clinical records for the period July 1989 to July 2002, was conducted to establish the response to treatment with surgery and melphalan chemotherapy. Of 21 dogs with tumours of the anal sacs 19 had apocrine gland adenocarcinomas of anal sac origin, one had a benign papillary cystadenoma and another had a malignant melanoma. Two of the 19 dogs had bilateral anal sac adenocarcinomas. Ten of the 19 dogs with apocrine gland adenocarcinomas of anal sac origin had sublumbar lymphadenopathy. Five dogs were excluded by their owners from recommended treatment. Fourteen dogs with apocrine gland adenocarcinomas of anal sac origin were treated by surgical cytoreduction and chemotherapy with melphalan. Seven of the 14 dogs had regional lymph node metastases. Cytoreduction was by local excision of the anal sac in all 14 dogs and concurrent removal of the sublumbar retroperitoneal lymph nodes in the seven dogs with regional lymph node metastases. The median survival time of dogs with sublumbar nodal metastasis was 20 months and for dogs with tumour localised to the anal sac the median survival time was 29.3 months. There was no difference in median survival of those dogs with sublumbar metastases compared to those without. This study suggests there is a role for melphalan in the treatment of dogs with anal sac adenocarcinoma when combined with cytoreductive surgery, with treatment survival times and the local recurrence rate of the primary tumour comparing favourably with previously published treatment regimes.

Multicentric reticulohistiocytosis (MRH) is a rare histocytic disease characterized by destructive arthritis in association with classic skin findings. Although MRH is not strictly a paraneoplastic disease, one quarter of cases are malignancy related. We report a case of MRH with an initial remission followed by an acute exacerbation several years later heralding the clinical presentation of endometrial carcinoma. During this flareup a skin biopsy specimen revealed a diffuse dermal infiltrate composed of histiocytes with ground-glass cytoplasm and multiple atypical mitoses. Approximately 40% of the cells stained with the proliferation marker Ki-67. Treatment of endometrial carcinoma resulted in improvement of skin and joint symptoms, and a repeat biopsy specimen no longer demonstrated mitotic figures. These findings support a reactive and proliferative cause of MRH.

Solid and cystic papillary epithelial neoplasms of the pancreas are uncommon tumors occurring predominantly in young women. These tumors have excellent prognosis and after complete surgical resection, more that 95% patients are cured. Occasionally, they invade the surrounding pancreatic parenchyma. These tumors can recur even many years after resection, so long-term follow up is essential. Very few cases metastasize. We present a case of a middle aged woman with metastases to liver and omentum.

Clear cell papillary cystadenoma is a rare epithelial tumor of the epididymis, which may present as an isolated lesion or as a component of von Hippel-Lindau disease (VHLD). Recently, tumors have also been described in the female genital tract with similar histology. Recognition of clear cell papillary cystadenoma is critical because of its association with VHLD and its potential diagnostic confusion with metastatic renal cell carcinoma because of a shared architecture and clear cells. In this study, we report on the immunohistochemical differentiation of 5 clear cell papillary cystadenomas, 3 of the epididymis and 2 of the mesosalpinx, from metastatic renal cell carcinoma. In 2 cases, there was a history of renal cell carcinoma in the setting of VHLD; and in 1 of these cases, an epididymal papillary cystadenoma was initially considered to be metastatic renal cell carcinoma. Immunohistochemically, tumor cells were moderately intensely positive for cytokeratin AE1/AE3 and epithelial membrane antigen, strongly positive for CK7 and negative for CK20 and RCC. Four of 5 cases were negative for CD10. This staining profile contrasts with that reported for clear cell renal cell carcinomas, which are typically negative for CK7 and immunoreactive for renal cell carcinoma (RCC) and CD10. Our findings indicate that, in cases where there is uncertainty about the histologic diagnosis of clear cell papillary cystadenoma, the above immunohistochemical panel helps to rule out metastatic renal cell carcinoma.

Papillary solid cystic neoplasm (PSCN) of the pancreas is a tumor of low malignant potential and has an excellent prognosis. Although the cytologic features are well documented, it can pose a diagnostic problem when it presents as an extrapancreatic mass.A young woman presented with a retroperitoneal mass. Sonography and computed tomography (CT) showed a partially cystic mass touching the spleen and pancreas but distinct from both organs. The CT-guided aspiration cytologic diagnosis was paraganglioma. At surgery the mass was attached to the tail of the pancreas by a pedicle. The histologic diagnosis was PSCN of the pancreas.The cytologic findings in paraganglioma and PSCN may be strikingly similar, with both showing a perivascular pattern, acinar formations, cells with a moderate amount of ill-defined cytoplasm with red granularity on May-Grünwald Giemsa stain and a uniform chromatin pattern. This may be a source of diagnostic error, particularly in a patient presenting with a retroperitoneal, extrapancreatic mass.

Solid cystic papillary tumors of the pancreas are rare. This tumors were seen in young women. They have distinct clinicopathologic characteristics. Their prognosis is excellent because of their low grad of malignancy. The tumor was encapsulated and have favorable prognosis after complete surgical resection. We report one case of solid cystic papillary tumor of the pancreas occuring in female patient who are 20 years old. This observation have been a pre operative morphologic and ultrastructural study (CT, Ctscan, EE, IRM and immunohistochemistry study) who permetted to elaborate diagnosis.

Papillary solid and cystic neoplasm (PSCN) is a rare neoplasm of the pancreas with low-grade malignant potential and favorable prognosis. We report an 18-year-old girl with PSCN presenting with advanced disease. The tumor regressed with six cycles of gemcitabine and cisplantin-based neoadjuvant chemotherapy; spleen-preserving distal pancreatectomy was then done. She is disease-free at 13 months' follow-up.

A case report of the papillary cystadenoma from minor salivary gland in lower lip of a 54-year-old man is described.

Background During pancreaticoduodenectomy, early neck division may be inadequate, particularly in cases of replaced or accessory right hepatic artery (RHA) or diffuse intraductal papillary and mucinous neoplasm (IPMN) or when invasion of superior mesenteric artery is suspected. Methods Modification of the technique of pancreaticoduodenectomy with dissection of the superior mesenteric vessels performed from behind the head of the pancreas before any pancreatic or digestive transection. The pancreas is divided later, after adequate mobilization of the specimen from the vessels, on either the neck or the body, according to underlying disease. Results We used this technique successfully in 20 patients with a replaced or accessory RHA, which was successfully preserved in 19; one patient had inadvertent division of a 2-mm diameter accessory RHA that was ligated with no postoperative complications. Additionally, we used this technique in 18 patients with IPMN—14 pancreaticoduodenectomies extended to the body and 4 total pancreatectomies—and in 3 patients with adenocarcinoma involving the porto-mesenteric confluence and needing en-bloc vascular resection. Conclusions Early division of retroperitoneal margin is a useful technical variant of pancreaticoduodenectomy, which can be recommended in selective indications to improve safety and radicality of the procedure.

The Japan Pancreas Society performed a multiinstitutional, retrospective study of 1379 cases of intraductal papillary mucinous tumor (IPMT) and 179 cases of mucinous cystic tumor (MCT) of the pancreas. Clinicopathologic features and postoperative long-term outcomes were investigated. IPMT were most frequently found in men and in the head of the pancreas. In contrast, all patients with MCT were women. Ovarian-type stroma were found in only 42.2% of the MCT cases. Prognostic indicators of malignant IPMT included advanced age, positive symptoms, abundant mucous secretion, presence of large nodules and/or large cysts, remarkable dilatation of the main pancreatic duct, and main duct- or combined-type IPMT. Advanced age, positive symptoms, and presence of large nodules and/or large cysts were predictive of malignant MCT. The 5-year survival rate of IPMT patients was 98%-100% in adenoma to noninvasive carcinoma cases, 89% in minimally invasive carcinoma cases, and 57.7% in invasive carcinoma cases. The 5-year survival rate of MCT patients was 100% in adenoma to minimally invasive carcinoma cases and 37.5% in invasive carcinoma cases. In conclusion, IPMT and MCT show distinct clinicopathologic and prognostic differences. The results from this study may contribute to the diagnosis and treatment of IPMT and MCT.

Palmar fasciitis and polyarthritis syndrome (PFPAS) is an uncommon paraneoplastic syndrome associated with several malignant neoplasms. We identified 4 patients with PFPAS and ovarian carcinoma. Palmar fasciitis, at times severe, and inflammatory polyarthritis dominated the clinical presentation in all 4 patients. In 3 of our 4 patients the presentation of palmar fasciitis and inflammatory polyarthritis preceded the diagnosis of ovarian carcinoma. Magnetic resonance scanning and biopsy examination of palmar nodules in one patient revealed findings of inflammation and fibrosis. A literature review found 10 other cases of PFPAS associated with ovarian carcinoma. Improvement in palmar fasciitis and inflammatory arthritis often occurs after successful treatment of the ovarian carcinoma. Digital contractures, however, can persist. We recommend a gynecologic examination in any woman presenting with the sudden onset of unexplained hand pain, palmar inflammatory fasciitis, palmar fibromatosis, and digital contractures.