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BACKGROUND/AIM: Intraductal papillary neoplasms of the bile duct (IPNB) are histologically and clinically classified as type 1 and 2. This study aimed to identify the differences between these two types. MATERIALS AND METHODS: Based on multiple gene expression analysis (MGEA) using type 1, type 2, and pancreatic intraductal papillary mucinous neoplasms (n=4, 6, and 5, respectively), immunohistochemistry of DNMT1 and methylation-specific PCR for p16, APC, BRCA1, hMLH1, TIMP3, and SOX17 were performed on type 1 and 2 IPNBs (n=14, each). RESULTS: The DNMT1 protein was highly expressed (p<0.001) in 28.6% of type 1 cases and all type 2 cases. The DNA methylation ratio for the six genes in total as well as for SOX17 was lower in type 1 than in type 2 (p<0.05 each). CONCLUSION: Type 2 IPNB showed increased DNMT1 protein expression and increased DNA methylation frequency of the examined tumor suppressor genes compared to type 1. DNMT1 IHC may be helpful in discriminating between these two types.

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Intraductal oncocytic papillary neoplasm (IOPN) is a rare intraductal tumor of the pancreatobiliary system. Currently, little is known about its distinct characteristics, unlike intraductal papillary mucinous neoplasms (IPMN) and intraductal papillary neoplasms of the bile duct (IPNB). The present study compared 22 IOPNs (18 pancreatic and 4 biliary) with those of 61 IPMNs/8 IPNBs. IOPNs were classified into pure and combined types, depending on the coexistence of IPMN/IPNB. Multiple gene expression analysis (nCounter system) was performed, and hierarchical clustering analysis separated IOPNs(n = 4) and IPMNs(n = 3)/ IPNBs(n = 3), and pathway score analysis supported the result. Volcano plot identified follistatin (FST) as the most upregulated mRNA in IOPN in comparison to the gastric subtype (log2 fold change of 5.34) and the intestinal subtype (that of 5.81) of IPMN/IPNB. The expression of FST in IOPN was also high in quantitative polymerase chain reaction and immunohistochemical analysis. We also found lower apoptotic activity in IOPN, particularly in pure type, compared to high-grade or invasive IPMN/IPNB using immunohistochemistry for cleaved caspase 3. But, combined type IOPN was more similar to IPMN/IPNB than pure IOPN. In conclusion, we proved that IOPN, particularly pure IOPN, is distinct from IPMN/IPNB in FST mRNA overexpression and exhibits lower apoptotic activity.

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We present 3 cases of extrapulmonary lymphangioleiomyomatosis (LAM) identified incidentally in the uterine corpus and pelvic nodes resected for other reasons. One patient, a 47-yr-old female with corpus cancer, underwent a total hysterectomy and nodal dissection; the other 2 patients, aged 44 and 49 yr, underwent simple hysterectomy for corpus leiomyomas. None of the patients had evidence of tuberous sclerosis complex or any significant lesions in other organs. An area of spindle cell proliferation, intimately associated with dilated and tortuous lymphatic vessels, was found in the myometrium of all 3 patients, and nodal involvement with spindle cell proliferation was observed in the patient with corpus cancer. The spindle cells had faintly eosinophilic cytoplasm and a bland appearance. They were immunoreactive for α-SMA, gp100 (HMB45), and Melan-A. Tumor cell clusters lined with a single layer of lymphatic endothelium were floating in the lymphatic vessel lumen. These lesions were diagnosed as lymphangioleiomyoma in the uterine corpus and associated lymph nodes. Two of the cases seemed to be the earliest manifestations of extrapulmonary LAM, and the other case represents early-phase metastasis of LAM from the uterus. The present cases support the speculation that the uterus is the primary source of LAM cells.

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The combined colonic adenoma/microcarcinoid tumor is a rare intestinal neoplasm featuring intermingled adenomatous and carcinoid components. A few case reports and small case series have suggested that this entity exhibits an indolent clinical course. Here, we report two cases with these tumors, and describe the morphological features and clinical follow-up. A 61-year-old male and 78-year-old male presented with heme-positive stools at their medical checkups. Colonoscopy revealed masses in the colons; we performed endoscopic mucosal resection. Both lesions featured low-grade adenomas and low-grade neuroendocrine tumors. We diagnosed combined colonic adenomas/microcarcinoids. The clinical courses of both patients were benign at follow-up at 2.5 and 6 years. Awareness of this rare colonic tumor should prevent potential diagnostic pitfalls and may help clarify the natural history of these tumors and their possible relationships with composite glandular/carcinoid tumors.

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BACKGROUND: Primary pulmonary meningiomas are very rare, and primary intraosseous meningiomas outside the head and neck region have not yet been reported. We report an extremely unusual case of concurrent meningiomas arising in the pulmonary parenchyma and vertebral bone. CASE PRESENTATION: A 40-year-old Asian woman presented with a destructive lesion of the lumbar vertebral bone and a small nodule in the right lung. Five years later, both lesions slightly increased in size. To evaluate both the pulmonary and vertebral lesions, video-assisted thoracic surgery and curettage of the lytic lesion were performed. Both lesions showed similar histopathological findings corresponding to an intracranial meningioma of World Health Organization grade 1. The patient made good postoperative progress and remained free from disease at 41 months after the operation. CONCLUSIONS: Our patient presented with almost synchronous pulmonary and lumbar vertebral intraosseous meningiomas. Regarding the relationship between the two lesions, there are two possibilities: Independent tumors occurred coincidentally or the primary pulmonary meningioma metastasized to the vertebral bone despite its bland morphology. It is important to keep in mind the exceptionally rare condition of extracranial meningioma.

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BACKGROUND: Mesonephric adenocarcinoma (MA) is a rare tumor believed to arise from mesonephric remnants occurring mostly in the uterine cervix and, to a lesser extent, the corpus. Since the first case report of MA in the corpus in 1995, only 16 cases have been reported in the English literature. A recent report suggested that MA originates in Müllerian tissue and exhibits the mesonephric differentiation phenotype. CASE PRESENTATION: An asymptomatic 61-year-old woman was referred to our hospital because of elevated levels of tumor markers. Imaging revealed an intramural lesion of the uterine corpus exhibiting fluorodeoxyglucose uptake. A total hysterectomy and bilateral salpingo-oophorectomy were performed. The tumor was completely confined to the corpus wall and was composed of an intracystic bulky component and an invasive component in the myometrial layer. The tumor exhibited a variety of growth patterns, including a characteristic tubular pattern with dense eosinophilic secretion reminiscent of the thyroid, as well as a variety of morphologies, such as acinar, papillary, and ductal structures. The structures were immunoreactive for CK7, vimentin, CD10, calretinin, PAX8, and GATA3 and almost completely negative for ER/PgR. CA125 and CA19-9 antigen expression was also detected. CONCLUSION: A case of MA with a unique growth pattern of an intracystic mass within the corpus wall is presented. The histogenesis and differential diagnoses are discussed. The histogenesis of MA is not yet clear. We hypothesize two different pathways involved: 1) direct development from the mesonephric remnants and/or 2) mesonephric transformation of Müllerian adenocarcinoma.

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A case of esophageal carcinoma exclusively composed of adenocarcinoma simulating an esophageal gland duct in a 61-year-old man is presented. The tumor arose as a slightly elevated lesion in the middle intrathoracic esophagus. It was almost completely overlaid with non-neoplastic stratified squamous epithelial cells. Beneath the overlying surface epithelium, an adenocarcinoma that was bilayered in structure diffusely invaded both the mucosal and submucosal layers. Although the tumor consisted exclusively of adenocarcinomatous cells, a keratinizing squamous cell carcinoma component was focally observed. The invasive carcinoma was focally continuous with the small area of the surface squamous epithelial layer, which was confirmed to be neoplastic by immunohistochemistry. Morphological and immunohistochemical examinations suggested that the adenocarcinomatous component arose from the esophageal surface epithelium and clearly differentiated into an esophageal gland duct. It is important to consider the possibility of this type of adenocarcinoma when diagnosing a ductal or glandular lesion of the esophagus in small biopsy specimens.

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Perineuriomas are uncommon benign peripheral nerve sheath tumors that include soft tissue, sclerosing, reticular, and intraneural variants. Soft tissue perineuriomas arise in a wide anatomic distribution and mostly in patients older than 20 years of age. We report an atypical perineurioma in a 7-year-old girl. The tumor, located in the tongue, was uniformly hypercellular. The tumor cells were spindle-shaped with a slender, elongated, bipolar, wavy cytoplasmic process formation and wavy elongated nuclei, and the architecture was composed of predominantly short fascicles with areas exhibiting a vague storiform pattern. Although the tumor cells generally appeared bland, the tumor showed worrisome features including an infiltrative pattern and occasional mitotic figures. Psammoma bodies were observed in the periphery of the tumor. Immunohistochemically, the cells were positive for epithelial membrane antigen, vimentin, claudin-1, and GLUT-1, but negative for S-100 protein, CD34, and type IV collagen. The authors document a case of soft tissue perineurioma with atypical histological features that occurred in the tongue of a child.

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A 51-year-old man was hospitalized for evaluation of dysphagia and bloody stool. Gastrointestinal endoscopy showed esophageal cancer invading the gastric fundus. A metastatic lesion was demonstrated in the sigmoid colon. The patient agreed to have concurrent chemoradiotherapy for the primary lesion, followed by additional chemotherapy. The first course included 30 Gy of radiotherapy given over 3 weeks, together with daily oral administration of S-1 (80 mg/m2 per day) for 2 weeks, and a 24-h infusion of cisplatin (70 mg/m2) on day 8. After a second course of chemoradiotherapy, four additional courses of chemotherapy with S-1 and cisplatin were administered, at 4-week intervals. After the additional chemotherapy, gastroscopy and colonoscopy showed disappearance of both the primary and the metastatic lesions. One year after his initial hospitalization, no recurrence of either the primary or the metastatic tumor lesions is evident.

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A 52-year-old man was hospitalized for evaluation of dysphagia. Esophagography depicted an irregular narrowing extending 7 cm from the cervical esophagus to the upper thoracic esophagus. Esophagoscopy with biopsy showed cervical esophageal cancer narrowing the lumen. Surgery was contraindicated because of a previous cardiac infarction. The patient selected concurrent chemoradiotherapy with TS-1 and cisplatin. The first course included 30 Gy of radiotherapy given over 3 weeks, together with daily oral administration of TS-1 (120 mg/day) for 2 weeks, and a 24-h infusion of cisplatin (70 mg/m2) on day 8. After a second course of chemoradiotherapy, 4 courses of chemotherapy with TS-1 and cisplatin were administered at 4-week intervals. After additional chemotherapy, esophagoscopy and cervical CT showed that the primary lesion had disappeared. Two years after initial hospitalization, no recurrence has been detected.

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A 51-year-old male patient with esophageal cancer and cervical, thoracic and celiac artery lymph node metastases was treated by combination chemotherapy of TS-1 and cisplatin. TS-1 (80 mg/m2/day) was administered for 14 days followed by 14 days rest as 1 course. Cisplatin (70 mg/m2/day) was administered in 24-hour continuous intravenous infusion at day 8 after the start of TS-1. Before treatment, the tumor marker, CEA showed 27,060 ng/ml. After 5 courses of chemotherapy, endoscopy revealed that the primary tumor had disappeared and no cancer cells were detected by endoscopic biopsy. Chest and abdominal CT scan also showed almost total disappearance of the lymph nodes metastases. CEA decreased to 710 ng/ml. No high-grade toxicities (WHO grade 3 or 4) were seen during the chemotherapy. He is now very well. This TS-1/cisplatin chemotherapy regimen might be a useful treatment for metastatic esophageal cancer.

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A 75-year-old male patient with small cell carcinoma of the stomach and liver metastasis was treated by combined chemotherapy of TS-1 and CDDP. One course consisted of TS-1 (120 mg/day) administered for 14 days followed by 14 days rest. CDDP (108 mg/day) was administered by 24-hour continuous intravenous infusion at day 8 after the start of TS-1. After 3 courses, endoscopic examination revealed complete disappearance of the primary tumor with no cancer cells detected by endoscopic biopsy. CT-scan showed that the metastasis of the left lobe of the liver had disappeared and also that the metastasis of the right lobe of the liver was remarkably reduced (75%). The primary lesion was estimated CR, the metastasis PR, and the synthesis PR. The TS-1/CDDP chemotherapy regimen is considered effective for small cell carcinoma of the stomach with liver metastasis.

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A 70-year-old patient with advanced esophageal cancer with invasion to the aorta was treated by combined chemotherapy of TS-1 and CDDP with radiotherapy. TS-1 (80 mg/m2) was administered for 14 days followed by 14 days rest, CDDP (70 mg/m2) was administered by 24 h continuous intravenous infusion at day 8 after the start of TS-1. Radiotherapy (5 days/week) at 2 Gy/day was concurrently started from the beginning of chemotherapy for 3 weeks. After the end of the first course, leukocytopenia of grade 2 and thrombocytopenia of grade 2 were observed. The second course of chemoradiotherapy was suspended for 1 week. After recovery from the toxicity, the second course was started. After the 2 courses of chemoradiotherapy, endoscopic examination with biopsy revealed the disappearance of the esophageal cancer. Combined chemotherapy of TS-1 and CDDP was administered 2 times after chemoradiotherapy. After this therapy, endoscopy and a CT showed a complete clinical response. No severe adverse effects were observed during this therapy. Combined chemotherapy of TS-1 and CDDP with radiotherapy can be effective for advanced esophageal cancer.

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BACKGROUND: This study evaluated zoom endoscopic findings according to four components in comparison with the histologic findings of acute cellular rejection (ACR) in a living-related small bowel transplantation (SBTx) patient. METHODS: A 16-year-old boy with microvillus inclusion disease underwent SBTx with a 150-cm-long ileal graft from a blood-identical living-related donor. The endoscope was inserted into the distal stoma of the graft, and the mucosal architecture was observed under zoom. The observed findings were expressed by the following four components and graded as 0 to 2: H, homogeneity of mucosal change, minimal (H-0), patchy (H-1), diffuse (H-2); V, appearance of villi, stringlike (V-0), tonguelike (V-1), domelike (V-2); W, widening of crypt area, narrow (W-0), slightly widened within a width of one villus (W-1), markedly widened beyond a width of one villus (W-2); E, erythema in crypt area: no redness (E-0), sporadic erythema (E-1), diffuse erythema (E-2). RESULTS: Histologic ACR was grade 0 in 27, grade 1 in 6, grade 2 in 3, and grade 3 in 0 occasions during 11 months after SBTx. In grade 0, 1, and 2 histology, H-0/H-1/H-2 was 85.2%/14.8%/0%, 33.3%/66.7%/0%, and 0%/100%/0%, respectively, with a significant difference among the groups by grade (P<0.05). V-0/V-1/V-2 was 48.1%/51.9%/0%, 0%/100%/0%, and 0%/66.7%/33.3% (P<0.05), W-0/W-1/W-2 was 85.2%/14.8%/0%, 0%/100%/0%, and 0%/66.7%/33.3% (P<0.05), and E-0/E-1/E-2 was 100%/0%/0%, 66.7%/33.3%/0%, and 0%/100%/0% (P<0.05), respectively. CONCLUSION: Zoom endoscopic findings, graded on four components, may reflect the histologic severity of ACR and minimize the performance of biopsies in SBTx.

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OBJECTIVE: This study attempted to establish echocardiographic measurements of left ventricular (LV) mass and LV systolic and diastolic function, particularly in rats with hypertensive heart. METHODS: M-mode LV echograms and Doppler mitral flow were obtained in Dahl salt-sensitive rats placed on 0.3% or 8% sodium chloride diet. Echo Doppler measurements were compared with catheterization and pathologic measurements in 54 rats for LV mass and in 45 rats for LV systolic and diastolic function. RESULTS: Echocardiographic measurement of LV mass correlated well with pathologic measurement (r = 0.94, P <.01, n = 54, SEE = 0.08 mg), independent of LV size, aging, and therapeutic intervention. Endocardial fractional shortening (FS) correlated with LV peak + dP/dt (r = 0.56, n = 45, P <.01), and the correlation was improved to r = 0.71 if 11 rats with marked LV hypertrophy were excluded. Midwall FS correlated well with LV peak + dP/dt (r = 0.72, n = 45, P <.01) even if rats with extremely thickened ventricular wall were included. If midwall FS was lower than 14%, LV systolic dysfunction was very likely (sensitivity 67%, specificity 91%). Association of mitral E/A ratio of 2.0 or greater with deceleration time of shorter than 35 ms was an accurate indicator of elevated LV end-diastolic pressure (sensitivity 82%, specificity 86%) and increased lung weight because of congestive heart failure (sensitivity 89%, specificity 96%) in rats with hypertension. CONCLUSION: LV mass, LV systolic function, and LV end-diastolic pressure were assessable with echo Doppler in rats with hypertensive heart.

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