Aortic regurgitation caused by fibrous strand rupture in a fenestrated aortic valve.

Fibrous strand rupture in a fenestrated aortic valve can cause acute severe aortic regurgitation. We report the case of a 56-year-old woman with severe aortic regurgitation. Transesophageal echocardiography showed an abnormal fibrous strand echo on the prolapsed left coronary cusp (LCC). The operative finding revealed one ruptured fibrous strand attached to the LCC at the commissure between the left and noncoronary cusps. Pathologic examination of the aortic valve revealed myxomatous degeneration.

[Small renal cell carcinoma presented with pleural metastasis and multiple bone metastasis ; a case report].

The patient is a 69-year-old male. His chief complaint was chest pain. Because imaging studies suggested pleural mesothelioma associated with multiple bone metastases, right pleural tumor resection was performed. Pathological diagnosis was metastatic pleural tumor, and renal cell carcinoma (RCC) was suspected as its origin. Dynamic computed tomography showed a small tumor in the right kidney. The tumor was 15 mm in diameter and consistent with RCC. Laparoscopic radical nephrectomy was performed for the right kidney. Pathological diagnosis was RCC, clear cell carcinoma with sarcomatoid component, T1aN0M1, stage IV. Sorafenib therapy was started 46 days after the operation as a systemic therapy, and stable disease has been maintained. Generally, small RCC is assumed to have a good prognosis. However, a small percentage of patients with small RCC have distant metastasis at the time of diagnosis, and the prognosis is reported to be poor. We report this case, and a review of the literature.

Ectopic thymoma presenting as a giant intrathoracic tumor: a case report.

Ectopic thymoma rarely presents as an intrathoracic tumor. We report a case of ectopic thymoma presenting as a giant right intrathoracic tumor that was treated with resection. The patient was a 50-year-old Japanese woman who presented with the chief complaint of chest pain. Detailed examination revealed a solid tumor measuring 15 × 10 × 8 cm in diameter, with a clear border. The Imaging findings suggested a solitary fibrous tumor, and surgery was performed. At surgery, the tumor was found to be adherent to the diaphragm, mediastinal pleura, and lower lobe of the lung, although it could be dissected with relative ease and was removed. Pathological diagnosis indicated a type B1 tumor with no capsular invasion according to the World Health Organization classification, and a diagnosis of Masaoka stage I thymoma was made. No continuity with the normal thymus tissue was seen, and the thymoma was considered to be derived from ectopic thymic tissue in the pleura.

Alpha-fetoprotein-producing primary lung carcinoma: a case report.

Alpha-fetoprotein (AFP)-producing lung adenocarcinoma is a rare type of lung cancer, with its characteristics not yet fully clarified. We recently encountered a case of this type of lung cancer. The patient was a 69-year-old man who consulted an internist with the chief complaint of epigastric pain. Chest X-ray and CT revealed a lobulated mass measuring 70 mm in diameter in the right lower lung field and a metastasis in the right hilar lymph nodes. Of the tumor markers, the serum AFP was elevated (4620 ng/ml), and the serum carcinoembryonic antigen and carbohydrate antigen 19-9 were also slightly elevated. Transbronchial lung biopsy revealed the diagnosis of lung cancer. Under thoracoscopic assistance, right lower lobectomy + mediastinal lymph node dissection was carried out. Immunostaining showed the tumor cells to be AFP-positive. The tumor was thus diagnosed as an AFP-producing lung adenocarcinoma. The patient followed an uneventful clinical course after the surgery, with serum AFP decreasing to the normal range by about 2 weeks after the surgery. As of this writing, no sign of tumor recurrence has been noted. This case is presented here with a review of the literature.

Localization of the most severely dysplastic/invasive lesions and mucin phenotypes in intraductal papillary mucinous neoplasm of the pancreas.

OBJECTIVE: The aim of this study was to define the relevance of mural nodules (MNs) as a "direct" indicator of malignancy of intraductal papillary mucinous neoplasm (IPMN) of the pancreas. METHODS: Thirty-nine surgically resected IPMNs excluding obviously invasive carcinomas were examined. The distribution of the most severely dysplastic lesions was mapped on specimens. Immunohistochemical analysis for MUC1 and MUC2 was performed on sections containing the histologically predominant lesions and the most severely dysplastic areas. RESULTS: The presence of MNs correlated well with the histological grade of IPMN (P < 0.01); however, the most severely dysplastic lesions were associated with a flat/nonelevated area rather than MNs (78.9%). In the MUC1-positive subgroup, minimally invasive carcinoma was colocalized to MNs, whereas most severely dysplastic foci including minimally invasive carcinoma with components of mucinous and tubular adenocarcinoma were observed in the areas apart from MNs in the MUC2-positive and MUC1/2-negative subgroups, respectively. CONCLUSIONS: Although our data support the concept that MNs represent areas of higher-grade dysplasia within IPMN, development of invasive lesions from MNs may be limited to cases that are MUC1-positive. Careful attention should be paid to the emergence of invasive IPMN from flat foci in MUC2-positive and MUC1/2-negative cases.

Atypical tumour-like involvement of the colon in secondary systemic amyloidosis which vanished after 1 month of observation.

Amyloidosis occurs as a result of the extracellular deposition of protein fibrils in organs and tissues, thus causing mild to severe pathophysiological changes. The gastrointestinal tract is a common site of amyloid deposition. While intestinal amyloidosis frequently results in polypoid lesions, ulcerations, nodules and petechial mucosal haemorrhage, tumour-like lesions are rarely developed and infrequently diagnosed before the resection because of the difficulty in differentiating them from colon cancer. The authors herein reported a case of intestinal amyloid A amyloidosis with a complication of a tumour-like lesion endoscopically resembling a malignant lesion, which was completely diminished after 1 month of observation with bowel rest. Such conservative treatment is a feasible option to cure intestinal tumour-like lesions in patients with intestinal amyloidosis when no neoplastic change is histologically detected, possibly decreasing the need for surgery of the fragile mucosa.

Adenoid cystic carcinoma of the peripheral lung: a case report.

Adenoid cystic carcinoma of the peripheral lung is a rare entity. We recently encountered a patient with adenoid cystic carcinoma. A 75-year-old woman showed a nodular lesion with 10 mm in diameter in the right upper lung field on chest radiography. The diagnosis was unclear, but lung cancer could not be ruled out. Thoracoscopic biopsy was performed, and intraoperative pathological diagnosis revealed the carcinoma of the lung. We enforced upper lobectomy and mediastinal lymph node dissection to the patient. Histopathological examination revealed adenoid cystic carcinoma with a characteristic cribriform structure. Immunohistochemical examination revealed that the tumor cells were positive for thyroid transcription factor 1 (TTF-1), this tumor was diagnosed primary ACC of the lung.

High incidence of chromosomal abnormalities at 1p36 and 9p21 in early-stage central type squamous cell carcinoma and squamous dysplasia of bronchus detected by autofluorescence bronchoscopy.

Heavy smokers with central type squamous cell carcinoma (SCC) frequently have multiple cancerous lesions in the bronchus. Autofluorescence bronchoscopy (AFB) is useful in the detection of early bronchogenic cancer and dysplastic lesions. We investigated the loss of heterozygosity (LOH) and microsatellite instability (MSI) and expression of four proteins in 13 early stage SCC (early SCC) and 9 squamous dysplasia detected by AFB and 19 cases of surgically resected invasive SCC (invasive SCC). In early SCC and squamous dysplasia, LOH/MSI of chromosome 1p36 was found in 62 and 33%, respectively, and of 9p21 in 54 and 63%, respectively. TAp73 expression of early SCC and squamous dysplasia was lower than that of normal bronchial epithelium, and p16 expression was not detectable in these lesions. These results suggested that the genetic abnormalities had already developed in the early stage of carcinogenesis of SCC, including squamous dysplasia. The AFB system was able to reveal abnormal autofluorescence in these precancerous lesions, including squamous dysplasia.

Multifocal pancreatic intraepithelial neoplasia (PanIN) lesions of the branch ducts associated with lobular parenchymal atrophy in a Japanese patient diagnosed to have familial pancreatic cancer.

This report presents a case of Japanese familial pancreatic cancer (FPC) with multifocal pancreatic intraepithelial neoplasia (PanIN) lesions of the branch ducts probably associated with lobular parenchymal atrophy. The risk of pancreatic cancer is significantly increased in those associated with FPC, and this risk increases with increasing numbers of affected first-degree relatives, but there have been four Japanese cases reported. A 63-year-old Japanese male was referred to the hospital for evaluation and treatment of a pancreatic head tumor. His family history included pancreatic cancer in two-first-degree relatives and three-second-degree relatives. A pylorus-preserving pancreatoduodenectomy with a regional lymphadenectomy and intraoperative radiotherapy were performed. The histological findings of the main tumor showed a moderately differentiated tubular adenocarcinoma in the head of the pancreas without metastasis of the resected lymph nodes. Interestingly, multifocal PanIN lesions in the branch ducts were individually developed and some of these lesions were probably associated with small lesions of lobular parenchymal atrophy. He remained in good condition for 37 months after the operation. Although the concept of FPC has not been clearly established in Japan, nationwide registries of FPC are probably useful for management of FPC patients.

[A case report of ball valve syndrome caused by the gastrointestinal stromal tumor arising from the muscularis mucosae in gastric fornix].

An 83-year-old woman who was admitted to other hospital, was consulted us because of continuous tarry stool and abdominal fullness. On upper endoscopy, the submucosal tumor from greater curvature of gastric fornix invaginated into the duodenal bulbus, showing so-called "ball valve syndrome (BVS)". As the tumor incarcerted again on the following endoscoopy, she underwent laparoscopic partial gastrectomy. The submucosal tumor was diagnosed histologically as gastrointestinal stromal tumor (GIST) arising from the muscularis mucosae. This is the first report that GIST arising from the muscularis mucosae in gastric fornix showed a BVS.

[Two cases of pancreatic tumor with von Hippel-Lindau disease].

Von Hippel-Lindau disease (VHL disease) is an inherited neoplasia syndrome. VHL disease which frequently complicates pancreatic lesions is rarely diagnosed by existence of pancreatic involvements. We report two cases of VHL disease with pancreatic lesions. The first patient was a 40-year-old woman. Adrenal pheochromocytoma, spinal hemangioblastoma and pancreatic endocrine tumor were resected. The second case was a 68-year-old woman with past surgical histories included cerebellar and spinal hemangioblastoma. Subtotal pancreatectomy was performed for multiple serous cystadenoma. IPMN which has been never reported as pancreatic involvement of VHL disease were documented by imaging diagnosis in the first case, and by histological examination in the second case. We considered VHL disease from coexistent multiple tumors include pancreatic involvements and finally diagnosed by genetic examination in both cases. Care should be taken regarding the patient's right for treatment against for the genetic disease. We hold a genetic conference composed of multidisciplinary team. Consequently we detected another VHL disease patient from patient's family.

Mucinous carcinoma of Vater's ampulla with a unique extension along the main pancreatic duct.

We report a case of mucinous carcinoma of Vater's ampulla with a unique extension along only the main pancreatic duct (MPD) and microinvasion to the pancreas. A 52-year-old man was referred to our hospital for the evaluation and treatment of acute pancreatitis. Abdominal computed tomography (CT) demonstrated swelling in the head of the pancreas with a mass in the duodenum. Hypotonic duodenography and endoscopic examination revealed a well-defined mass, measuring about 25 mm in size, in Vater's ampulla. A biopsy specimen of the tumor showed moderately differentiated adenocarcinoma. A pylorus-preserving pancreaticoduodenectomy with a regional lymphadenectomy was performed, under a preoperative diagnosis of adenocarcinoma of Vater's ampulla with direct invasion into the head of the pancreas. The resected specimen of the duodenum confirmed the presence of the mass, which measured 22 x 15 mm in size, in Vater's ampulla. Microscopically, the tumor consisted of two components: moderately differentiated adenocarcinoma in the peripheral region of the tumor Vater's papilla and mucinous carcinoma in the central region of the tumor. The mucinous carcinoma component uniquely extended along only the MPD with microinvasion to the pancreas. Immunohistochemically, both the moderately differentiated adenocarcinoma and the mucinous carcinoma were positive for cytokeratin 20 (CK20) and negative for cytokeratin 7 (CK7) which is the pattern of intestinal-type carcinoma of Vater's ampulla. We concluded that the original site of this tumor may have been the duodenal epithelium of Vater's ampulla originally moderately differentiated adenocarcinoma-which subsequently changed to mucinous carcinoma that extended along only the MPD with microinvasion to the pancreas.

Gallbladder carcinoma associated with occult pancreatobiliary reflux in the absence of pancreaticobiliary maljunction.

We herein report a case of gallbladder carcinoma associated with occult pancreatobiliary reflux (PR) in the absence of pancreatobiliary maljunction. A 67-year-old woman was referred to our hospital for the evaluation and treatment of a gallbladder tumor. Ultrasonography and computed tomography showed a nodular lesion in the fundus of the gallbladder, indicating the possibility of a gallbladder carcinoma. Endoscopic ultrasonography showed the nodular tumor and thickness of the surrounding epithelium. Endoscopic retrograde cholangiopancreatography revealed a normal pancreaticobiliary junction without the common channel and a slight dilatation of the common bile duct (15 mm in diameter). An open cholecystectomy and partial resection of the liver bed of the gallbladder with regional lymphadenectomy was performed. A C-tube was inserted from the cut end of the cystic duct into the common bile duct to prevent bile stasis. Biliary amylase and lipase levels sampled in the gallbladder were 2604 IU/l and 775 IU/l, respectively. Biliary amylase level in the bile collected from the C-tube in the common bile duct was 119 550 IU/l on postoperative day (POD) 6 and 22 265 IU/l on POD 12. These observations suggested that PR was present in this patient. The histopathological findings of the resected specimen showed a well-differentiated adenocarcinoma of the gallbladder with invasion to the muscle layer and no metastasis of the resected lymph nodes. A high index of nuclear staining for MIB-I in the cancer cells (about 10%) was exhibited, and a few cells in the normal epithelium also stained positive.

Low p38 MAPK and JNK activation in cultured hepatocytes of DRH rats; a strain highly resistant to hepatocarcinogenesis.

DRH rats are a hepatocarcinogenesis-resistant strain isolated from hepatocarcinogenesis-sensitive Donryu rats, and the liver of DRH shows less histological damage and fewer/smaller neoplastic hepatic lesions by the treatment with hepatocarcinogens. To investigate the mechanism of the resistance, the properties of hepatocytes of DRH and Donryu were compared. In primary culture, DRH hepatocytes exhibited higher proliferation and less apoptosis than Donryu hepatocytes in the presence of EGF and insulin. However, such difference was not correlated to the degree of DNA damage associated with cell culture or cell cycle checkpoint function. Although the mitogen-activated protein kinases [EGF receptor (EGFR) and extracellular signal regulating kinases (ERK1/2)] were activated to the same degree, the stress-activated protein kinases [p38 mitogen-activated protein kinase (p38) and c-jun N-terminal kinase (JNK)] were activated to a lesser degree in the DRH hepatocytes. Treatment with 2-acetylaminofluorene (2-AAF) in vivo also resulted in less JNK and p38 activation in the DRH livers. Furthermore, apoptosis signal-regulating kinase 1 (ASK1) was inhibited by the lysate from the DRH but not by the Donryu hepatocytes. The low activation of the stress-activated protein kinases may be linked to the resistance to cellular stress, which may underlie the hepatocarcinogenesis-resistance in DRH rats.

Pylorus-preserving total pancreatectomy for an intraductal papillary-mucinous neoplasm of the pancreas.

BACKGROUND/PURPOSE: Total pancreatectomy (TP) is rarely performed to treat invasive ductal carcinoma of the pancreas, due to the associated markedly impaired quality of life and poor prognosis after the resection. Intraductal papillary-mucinous neoplasm (IPMN) of the pancreas is characterized by extensive intraductal spread and a favorable outcome even when presenting at an invasive stage. We herein reappraise the role of pylorus-preserving total pancreatectomy (PPTP) as a viable alternative pancreatic resection modality for borderline and malignant IPMN. METHODS: A total of five patients with IPMN underwent PPTP and their clinical follow-up data were reviewed. RESULTS: TP was performed due to recurrent IPMN in the remnant pancreas after distal pancreatectomy in three patients and due to massive involvement of the entire pancreas in the others. All patients were treated by the pylorus-preserving method, while the spleen was also preserved in one patient. The surgical margins were negative and no metastasis to the resected lymph nodes was evident, based on histological examinations. One patient underwent a re-operation due to postoperative intraabdominal bleeding, while another patient required tubedrainage for left pleural effusion. Three of the four patients who underwent PPTP with a splenectomy experienced postoperative gastric ulcer, which were controlled by medication. One patient died due to suicide 16 months after the PPTP. All the others were doing well without recurrence at periods of 62 to 127 months after the PPTP. CONCLUSIONS: PPTP is therefore considered to be indicated as an effective treatment for borderline or malignant IPMN with extensive involvement, when the patient's condition permits, in order to achieve complete resection of the IPMN.

A malignant nonfunctioning pancreatic endocrine tumor with a unique pattern of intraductal growth.

The intraductal growth of nonfunctioning pancreatic endocrine tumors (NFPTs) is considered to be rare, and in our survey of the English-language literature, we found only three cases to have been described previously. We herein report the case of a 36-year-old man with a malignant NFPT that uniquely grew within the lumen of the main pancreatic duct (MPD) and completely obstructed the MPD, as shown by endoscopic retrograde pancreatography (ERP). Endoscopic ultrasonography clearly detected the tumor with intraductal growth. In addition, positron emission tomography (PET), using 18F-fluorodeoxyglucose (FDG) and computed tomography (CT) with the same scanner (FDG-PET/CT) showed enhanced uptake of FDG in the tumor. A pylorus-preserving pancreaticoduodenectomy and regional lymphadenectomy were performed under the preoperative diagnosis of an NFPT. Microscopically, positive immunoreactions for synaptophisin and vasoactive intestinal peptide indicated neuroendocrine differentiation of the tumor, while in addition, metastasis to a lymph node along the common hepatic artery was also observed. The patient has survived for 6 months after the surgery without any evidence of recurrence or metastasis. Both ERP and FDG-PET/CT were thus found to be useful for predicting the malignant potential of an NFPT in the preoperative diagnosis.