Clear Cell Papillary Cystadenoma of the Ovary Masquerading as Metastatic Clear Cell Renal Cell Carcinoma: A Case Report and Review of the Literature.

Clear cell papillary cystadenoma of the epididymis is an uncommon benign neoplasm, usually seen in patients with von Hippel-Lindau disease. Morphologic and immunohistochemical examination aid in distinguishing clear cell papillary cystadenoma from malignant histologic mimics including low-grade mesothelial proliferations and metastatic clear cell renal cell carcinomas. Analogous lesions have been described in the female genital tract, often posing diagnostic challenges due to their low incidence. Here, we present the difficult diagnostic aspects of the first case of clear cell papillary cystadenoma involving the ovary, including the salient immunohistochemical, ultrastructural, and molecular characteristics.

Imaging diagnostics: congenital malformations and acquired lesions of the inner ear.

INTRODUCTION: Congenital malformations and acquired lesions of the inner ear are characterised by small structural changes in this region. In recent decades, treatment options have improved considerably. At the same time, there has been a great advancement in diagnostic methods, obtaining high-resolution labyrinth images. Currently, we use a 64-multislice computed tomography scanner in spiral mode (Brilliance 64 Phillips, Eindhoven, the Netherlands), with an overlap of 0.66 mm and an interval of 0.33 mm, 120 KV and 300 mA. The magnetic resonance images were taken with Signa HDxt 1.5 and 3.0 T units (GE Healthcare, Waukesha, WI, USA). We reviewed the radiological features of the lesions affecting the inner ear. They are classified as congenital (labyrinth malformation and statoacoustic nerve deficiencies) or acquired (otospongiosis, labyrinthitis, Ménière's disease, inner ear haemorrhage, intralabyrinthine schwannoma and endolymphatic sac tumour). CONCLUSION: Magnetic resonance imaging and computed tomography play an essential role in diagnosing patients with inner ear pathology. The technique selected should be chosen depending on the clinical setting. In a generic way, tomography is the method of choice for the study of traumatic pathology or otospongiosis. When tumour or inflammatory pathology is suspected, magnetic resonance is superior. In cases of congenital malformation, both techniques are complementary.

Papillary cystadenoma of anterior one-third of the tongue.

Papillary cystadenoma is an unusual benign cystic neoplasm with cystic spaces of diverse sizes and intraluminal papillary projections histologically, which is commonly seen in the lips, cheek and palate. We report a case of papillary cystadenoma in a 40-year-old man on the anterior one-third of the tongue with classical histological features. The paper highlights the rarity of the site of occurrence with emphasis on differential diagnosis and the need for considering papillary cystadenoma when benign cystic lesions are encountered in the anterior one-third of the tongue.

[A case of papillary cystadenoma of the epididymis mimicking a testicular tumor].

A 68-year-old male presented with painless left scrotal enlargement of one year duration. Ultrasound, computed tomography (CT) and magnetic resonance imaging showed a multilocular cystic mass, 10×7.5× 8.5 cm in size, in the left scrotum. The intracystic fluid was partially hemorrhagic. A solid part of the tumor, seen at the base of the scrotum, was partially calcified and was enhanced by contrast medium. The left testis could not be identified by imaging studies. Although CT imaging showed a simple cyst in the right kidney, no other lesions in the kidneys, adrenal glands, pancreas or the central nervous system were detected. Serum tumor marker values for testicular cancer were within the normal range. Under the pre-operative diagnosis of a left testicular tumor, left high orchiectomy was performed. Grossly the specimen consisted of a multilocular cystic tumor, 12.5×8.5×8.5 cm in size, with a 2.7 cm tan-colored solid component within the wall of the cyst. The left testis was atrophic, 1.3 cm in size, and demonstated no continuity with the solid part of the tumor. Histologically, the solid component of the tumor showed tubular and papillary growth of cuboidal and columnar tumor cells with clear cytoplasm. Histopathological diagnosis of papillary cystadenoma of the epididymis (PCE) was made. Von Hippel-Lidau disease was ruled out by subsequent genetic analysis. After follow up for 18 months, there was no sign of recurrence. To our knowledge, this is the 33rd and the largest case of PCE reported in Japan.

Volumetric and morphological analysis of intraductal papillary mucinous neoplasm of the pancreas using computed tomography and magnetic resonance imaging.

OBJECTIVE: The purpose of this study was to predict the malignancy of intraductal papillary mucinous neoplasm (IPMN) based on data obtained by computed tomography and magnetic resonance imaging. METHODS: Sixty-nine patients with IPMN underwent computed tomography, magnetic resonance imaging, and surgery. The tumors were classified pathologically as IPMN (adenoma), IPMN (in situ carcinoma), and invasive carcinoma derived from IPMN, and analyzed morphologically for the following characteristics: tumor size, main pancreatic duct (MPD) diameter, tumor area, MPD area, tumor volume, MPD volume, and intraductal volume (tumors volume + MPD volume). RESULTS: Main pancreatic duct diameter (P = 0.017) and intraductal volume (P = 0.0013) showed significant differences among IPMN (adenoma), IPMN (in situ carcinoma), and invasive carcinoma derived from IPMN. When IPMN (in situ carcinoma) and invasive carcinoma derived from IPMN were classified as malignant IPMN, an MPD diameter of 6 mm or more and an intraductal volume of 10 cm or more were set as cutoff levels predictive of malignancy using receiver operating characteristic curve analysis. On the basis of these criteria, the sensitivity and specificity for identifying malignancy in MPD were 83% and 59%, and those for intraductal volume were 70% and 73%, respectively. CONCLUSION: Intraductal volume (≥10 cm) determined by volumetric analysis is useful for diagnosis of malignant IPMN.

A large cystic tumor with bile duct communication originating around the hepatic hilum.

Biliary cystic tumors are rare neoplasms occurring in the liver and less frequently in the extrahepatic biliary system. Recently, biliary cystic tumors in the liver are thought to be divided into a biliary mucinous cystic neoplasm and intraductal papillary neoplasm of the bile duct. We report a case of a large cystic tumor originating around the hepatic hilum which had luminal communication with the bile duct. A 74 year-old-woman underwent abdominal ultrasonography for a routine checkup. It revealed a large cystic tumor in the liver. CT scan and MRI showed a multilocular cystic tumor about 12 cm in diameter with a mural nodule occupying the medial and anterior segment of the liver. Intraoperative cholangiography showed a communication between the cystic tumor and the bile duct. Central bisegmentectomy of the liver and extrahepatic bile duct resection was performed. A papillary tumor existed in the common hepatic duct and was connected with the cystic tumor in the liver. The tumor was mostly composed of noninvasive papillary adenocarcinoma with adenoma components, and was associated with focal microinvasion of adenocarcinoma. Ovarian-like stroma was not observed. This lesion was diagnosed as a cystic variant of intraductal papillary neoplasm of the bile duct. The patient is alive with no recurrence for 18 months since the surgery.

[Carcinoma of the pancreas at the site of an intraductal papillary mucinous neoplasia]. / Pankreaskarzinom auf dem Boden eines intraduktal papillär-muzinösen Tumors.

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.

[The solid pseudopapillary tumor of pancreas: two cases and literature review]. / Tumeurs pseudopapillaires et solides du pancréas: deux observations et revue de la littérature.

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.

[Differential diagnosis of pancreatic lesions using endoscopic ultrasound]. / Endosonografische Differenzialdiagnose von Pankreastumoren.

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.

Solid cystic papillary tumor of pancreas in eight children.

OBJECTIVE: To estimate the clinical and pathological features of pancreatic solid cystic papillary tumor (SCPT) in children. METHODS: 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. RESULTS: 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. CONCLUSION: Occurring predominantly in young females, SCPT is usually curable by surgical resection with a favorable prognosis.

Solid and cystic papillary epithelial neoplasm of the pancreas in an 11-year-old girl: imaging features with pathological correlation.

Solid and cystic papillary epithelial neoplasms (SCPEN) of the pancreas are rare tumours seen in young women. Pancreatic masses in children are extremely rare and SCPEN should be considered in the radiological differential diagnosis. Although there have been many series of SCPEN reported in the published literature in adults, only two series have focused on paediatric patients. The radiological and histopathological features of SCPEN in a paediatric patient are presented.

Natural history of intraductal papillary mucinous tumors of the pancreas: actuarial risk of malignancy.

BACKGROUND & AIMS: Natural history of intraductal papillary mucinous tumors of the pancreas (IPMTs) is unknown. Cross-sectional studies suggest that exclusive branch duct (BD) involvement is associated with a lower risk of carcinoma than main pancreatic duct (MPD) involvement. The aim of our study was to calculate longitudinal risk of malignant transformation of IPMT since the first sign. METHODS: All the patients with a diagnosis of highly probable or histologically proven IPMT were included. Actuarial risks of occurrence of at least low-grade dysplasia (>or=LGD), high-grade dysplasia (>or=HGD), or invasive carcinoma (IC) were calculated by Kaplan-Meier method from the first sign attributable to IPMT. The risks according to sex, acute pancreatitis, tumor size, and involvement of MPD were compared by log-rank test. RESULTS: One hundred six patients were included with a proven (n = 76) or probable (n = 30) IPMT. The tumor was confined to BD in 53 cases. Median duration since the onset of the first sign to the end of follow-up was 21 months (range, 0-241). Ten-year actuarial risk that IPMT grade was >or=LGD, >or=HGD, or IC was 67%, 49%, and 29%, respectively. The only morphologic risk factor of malignant transformation was involvement of MPD, with a 5-year actuarial risk of >or=HGD of 63% in the MPD group compared with 15% in the BD group (P < .001). CONCLUSIONS: Longitudinal risk of at least HGD or IC is time-dependent. Patients with BD IPMT present a much lower risk, justifying a nonoperative surveillance.

Solid pseudopapillary tumors of the pancreas: diagnosis and curative treatment.

OBJECTIVES: To highlight an infrequent occurrence using a series of clinical cases with symptoms and signs, and specific radiological findings allowing its diagnosis and treatment, which is in most cases successful. PATIENTS AND METHODS: A descriptive and retrospective study of patients diagnosed by computed tomography scanning and then treated with surgery in the Pancreas and Biliary Unit of a University Hospital from March 1999 to September 2005. RESULTS: There were 6 female patients with a mean age of 33.5 years (range 11-72). Most common signs included pain and a palpable mass in the abdomen. Three patients were diagnosed by computed tomography scanning, and a differential diagnosis with a neuroendocrine tumor was performed for the remaining three subjects. Surgical treatment was adapted to each patient according to the findings and images seen in their computed tomography scans. Biopsy results confirmed the presumed diagnoses, and showed one case of solid pseudopapillary carcinoma of the pancreas. Average hospital stay was of 18.16 days (range 8-30). Mortality rate was 0%. No recurrences occurred during follow-up for 46.3 months on average (range 12-76). CONCLUSIONS: The presence of a huge mass in the pancreas of a young female should prompt suspicion for a solid pseudopapillary tumor. Given its low malignant potential, and the presence of specific radiographic patterns, its diagnosis should be accurate, as radical surgical treatment is effective.

Solid and cystic epithelial neoplasm of pancreas with metastasis: report of a highly unusual case.

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.

Cytology of papillary solid-cystic neoplasm of the pancreas presenting as an extrapancreatic mass: a case report.

BACKGROUND: 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. CASE: 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. CONCLUSION: 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.

[A case of a papilloferous cystadenoma of boundary malignancy in a 29-year-old female patient with high surgical risk].

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.

Intraductal papillary mucinous tumor of the native pancreas in a pancreas-kidney transplant recipient.

Recent data from the International Pancreas Transplant Registry confirm the benefits of pancreas transplantation, even in high-risk diabetic patients previously considered unqualified for such procedures. With patient survival currently extending into decades, de novo malignancies are now more frequently diagnosed in pancreas recipients. This is the first reported case of an intraductal papillary mucinous tumor of the native pancreas in a high-risk pancreas after kidney recipient. The patient underwent a partial pancreaticoduodenectomy and has remained tumor-free for 9 months. This case report demonstrates that (1) pancreas transplantation can be successfully done in patients with significant cardiocerebrovascular disease, (2) de novo pancreas malignancies can occur within the first year after pancreas transplantation, and (3) tumors of the native pancreas warrant the same surgical treatment in pancreas recipients as in the general population, despite the need for posttransplant immunosuppression and the increased operative risk.