[A Case of Ileal Perforated Peritonitis 50 Years after Radiotherapy for Uterine Cancer].

Here we report a rare case of peritonitis caused by radiation enteritis. The 82-year-old woman who underwent surgery and radiotherapy for uterine cancer in her 30s. Emergency operation was performed for the perforation of the ileum. The small intestine showed changes of radiation enteritis extensively on macroscopy. The first surgery was performed to resect the perforated ileum and make intestinal anastomosis at the change of radiation enteritis. However, suture failure was occurred, reoperation was performed after conservative therapy. Reoperation was performed extensively resection of the intestinal tract and made anastomosis where was mild change of radiation enteritis. Pathological findings of the intestinal stump revealed that the arterial vessels of the submucosal layer were highly thicken and the lumen of artery was stenosis and occlusion with severe changes of radiation enteritis at the first operation. Blood flow disorders by irradiation were presumed to be the cause of suture failure. On the other hand, the intestinal stump did not indicate thickened of vascular wall and lumen stenosis of the vessels, only edematous changes in the submucosal layer were observed at the reoperation. It was important to determine the surgical procedure with the change of radiation enteritis for gastrointestinal operation with abdominal irradiation.

Hematopoietic stem cell transplantation for progressive combined immunodeficiency and lymphoproliferation in patients with activated phosphatidylinositol-3-OH kinase δ syndrome type 1.

BACKGROUND: Activated phosphatidylinositol-3-OH kinase δ syndrome type 1 (APDS1) is a recently described primary immunodeficiency syndrome characterized by recurrent respiratory tract infections, lymphoid hyperplasia, and Herpesviridae infections caused by germline gain-of-function mutations of PIK3CD. Hematopoietic stem cell transplantation (HSCT) can be considered to ameliorate progressive immunodeficiency and associated malignancy, but appropriate indications, methods, and outcomes of HSCT for APDS1 remain undefined. OBJECTIVE: Our objective was to analyze the clinical manifestations, laboratory findings, prognosis, and treatment of APDS1 and explore appropriate indications and methods of HSCT. METHODS: We reviewed retrospectively the medical records of cohorts undergoing HSCT at collaborating facilities. RESULTS: Thirty-year overall survival was 86.1%, but event-free survival was 39.6%. Life-threatening events, such as severe infections or lymphoproliferation, were frequent in childhood and adolescence and were common indications for HSCT. Nine patients underwent HSCT with fludarabine-based reduced-intensity conditioning. Seven patients survived after frequent adverse complications and engraftment failure. Most symptoms improved after HSCT. CONCLUSION: Patients with APDS1 showed variable clinical manifestations. Life-threatening progressive combined immunodeficiency and massive lymphoproliferation were common indications for HSCT. Fludarabine-based reduced-intensity conditioning-HSCT ameliorated clinical symptoms, but transplantation-related complications were frequent, including graft failure.

Validation of the fibrosis-4 (FIB-4) index in the diagnosis of liver disease of rheumatoid arthritis patients treated with methotrexate.

Objectives: To validate the usefulness of a hepatic fibrosis scoring system fibrosis-4 (FIB-4) index to diagnose liver diseases in rheumatoid arthritis (RA) patients treated with methotrexate (MTX).Methods: The FIB-4 index (age(years) × AST(U/L)/platelet (PLT) (109/L) × âˆšALT(U/L)), proposed as a predictor for liver fibrosis in HIV/HCV coinfection, was evaluated in this study. RA patients on MTX treatment were screened by FIB-4 index values to detect fibrotic change in the liver. Liver biopsy specimens were examined histologically in patients with high values.Results: Thirteen of 14 patients showed histology closely resembling non-alcoholic steatohepatitis. In three of them, two biopsies were performed: 1st, during MTX treatment; and 2nd, after discontinuation of MTX. All of them showed improvement in histology along with decreased FIB-4 values. Age, AST/√ALT, and 1/PLT, as well as creatinine levels and cumulative MTX doses were significantly higher in the high FIB-4 group compared with the low FIB-4 group. In the high FIB-4 group, 1/PLT and AST/√ALT were significantly correlated with FIB-4 values, but age was not.Conclusions: The FIB-4 index is simple to calculate and a valuable marker to diagnose liver disease in RA patients treated with long-term MTX administration.

[Airway obstruction due to localized tracheal lesion of extranodal NK/T-cell lymphoma, nasal type].

A 76-year-old man presented with a tracheal tumor associated with severe respiratory obstruction. A tracheotomy was performed due to respiratory failure. F-fluorodeoxyglucose (FDG) -positron emission tomography/computed tomography revealed an abnormal accumulation of FDG (maximum standardized uptake value: 16) in the trachea. A histopathological examination of the tracheal biopsy revealed extranodal NK/T-cell lymphoma, nasal type (ENKL). He was treated with concurrent radiotherapy (50 Gy) for the tracheal tumor and three courses of two-thirds dose ofdexamethasone, etoposide, ifosfamide, and carboplatin. Although the tumor responded remarkably well to this therapy, the patient died of an ENKL recurrence in the lungs and liver 11 months post therapy.

M1 macrophage infiltrations and histological changes in the liver after portal vein embolization using fibrinogen and OK432 in the rat.

The mechanism of anti-tumor effect of transarterial Immuno-Embolization (TIE) using OK-432 has not been well elucidated. In this study, we aimed to investigate the tissue injury and immune response after portal venous embolization (PVE) with/without OK-432. Embolic materials (L group: lipiodol, LF group: lipiodol+fibrinogen, LO group: lipiodol+OK-432, LFO group: lipiodol+fibrinogen+OK-432) were administered via the right portal vein in Wistar rats. The histological findings in LFO group demonstrated liver damage with severe architectural changes. The concentrations of CD68(+) cells were observed in a time-dependent manner; it was significantly increased in the LO group on day 1 and in the LFO group on day 3. CD68(+)CD163(-) macrophages significantly increased in the LFO group on day 7 (P<0.05). In conclusion, PVE with fibrinogen and OK-432 markedly increased the CD68(+)CD163(-) infiltrating macrophages around the peri-portal area in the liver. This novel technique could be applied as immune-enhanced chemo-embolization of liver tumors.

[A Case of Primary Poorly Differentiated/Small Cell Carcinoma of the Breast in a Patient with Von Recklinghausen's Disease].

We report a case of neuroendocrine carcinoma and poorly differentiated/small cell carcinoma ofthe breast in a patient with von Recklinghausen's disease. The patient was a 46-year-old woman who was diagnosed with von Recklinghausen's disease when she was 22 years old. She presented with left breast pain, and physical examination revealed a firm mass in the left breast. A core needle biopsy of the tumor revealed triple negative breast cancer with neuroendocrine features. We performed a simple mastectomy with lymph node dissection. We did not plan neoadjuvant chemotherapy because the tumor would be possibly inoperative if neoadjuvant chemotherapy was not effective for this neuroendocrine cancer. The tumor was diagnosed as a neuroendocrine carcinoma and poorly differentiated/small cell carcinoma. The patient was treated with CDDP and CPT- 11, which is a regimen often used to treat small cell lung cancer.

[Surgical Resection for a Metachronous Liver Metastasis of an Esophagogastric Junction Adenocarcinoma].

The patient was a 56-year-old man with advanced esophagogastric junction cancer. He received neoadjuvant chemotherapy with 5-FU plus CDDP followed by lower esophagectomy and total gastrectomy via the left thoracoabdominal approach in October 2011. Pathological examination revealed EGJ adenocarcinoma (ypT4aN1M0, Stage ⅢA, Japanese Classification of Gastric Carcinoma ver.14), and histological analysis indicated Grade 0 (no change). Adjuvant chemotherapy with S-1 was administered. Nevertheless, 6 months after the operation, a solitary hepatic metastasis (f: 32 mm) was detected in S7 of the liver. The patient underwent proton beam irradiation of the liver metastasis, resulting in a complete response, and he was followed up without any chemotherapy. However, 21 months after the irradiation, regrowth of the previous lesion with FDG re-accumulation was noted. Given the absence of any neoplasms other than the liver metastasis, right hepatic lobectomy was performed. Pathological examination revealed a small cluster of viable tumor cells surrounded by extensive fibrotic tissue (Grade 2). At 45 months after the initial operation (10 months after the liver lobectomy), the patient is living without any signs of recurrence. Surgical resection for liver metastasis of EGJ cancer may be feasible after careful selection.

Hepatic failure in a patient with rheumatoid arthritis treated with methotrexate: A case report.

INTRODUCTION: Chronic liver damage from methotrexate (MTX) is not uncommon, and fatal outcome is rare. We experienced a case of hepatic failure leading to death. We considered the cause of death through this case and proposed a method to prevent the progression of this liver injury. PATIENT CONCERNS: We report the case of a patient with rheumatoid arthritis treated with MTX for 15 years. DIAGNOSIS, INTERVENTIONS, AND OUTCOMES: A liver biopsy revealed histological changes similar to those of advanced nonalcoholic steatohepatitis (NASH), most likely induced by MTX. MTX was discontinued after 4 years. Two years after the discontinuation, the patient died of irreversible hepatic failure. Her obesity, complicated by type 2 diabetes mellitus, might have aggravated MTX-induced NASH-like liver injury. CONCLUSION: Early diagnosis and immediate MTX discontinuation following NASH diagnosis and strict type 2 diabetes mellitus control might have prevented the irreversible progression of liver injury.

Prospective evaluation of the optimal number of 25-gauge needle passes for endoscopic ultrasound-guided fine-needle aspiration biopsy of solid pancreatic lesions in the absence of an onsite cytopathologist.

INTRODUCTION: A prior study with 22-gauge needles recommended more than seven needle passes for endoscopic ultrasound-guided fine-needle aspiration biopsy (EUS-FNA) of solid pancreatic lesions (SPL) without onsite cytopathology for optimal acquisition of cytopathological diagnosis. The feasibility of this recommendation should be re-evaluated considering the later development and popularity of 25-gauge EUS-FNA needles. We aimed to determine the optimal number of needle passes for cytopathological specimen acquisition with 25-gauge needles for EUS-FNA of SPL. METHODS: A preliminary prospective study of 22 patients with an onsite cytopathology technician showed a sensitivity of 93.3% and a specificity of 100% with four needle passes that was not statistically different from five needle passes. Based on our preliminary study, we fixed the number of needle passes to four (Group A). As a control group, we carried out sampling in consecutive patients using 25-gauge needles with an onsite cytopathologist (Group B). Sampling rate, diagnostic value and complications were evaluated. RESULTS: We enrolled 20 patients in each group. Sampling rate was higher in Group B (20/20, 100%) than in Group A (19/20, 95%), but there was no statistical difference between them (P-value = 0.31). In Group A, sensitivity, specificity and accuracy were 100% among 19. In Group B, sensitivity was 94.1%, specificity 100%, accuracy 95%. There were also no statistical differences between the groups. No complications were seen. CONCLUSION: Our study suggests that four needle passes using a 25-gauge needle may be sufficient for EUS-FNA of SPL where onsite cytology is not available.

Endoscopic ultrasound-guided fine-needle aspiration of solid pancreatic masses with rapid on-site cytological evaluation by endosonographers without attendance of cytopathologists.

BACKGROUND: Endoscopic ultrasound-guided fine-needle aspiration (EUS-FNA) with rapid on-site evaluation (ROSE) has been reported to provide a more accurate diagnosis than EUS-FNA without such evaluation. However, even endosonographers can evaluate ROSE regarding sample adequacy. The aim of this study was to evaluate the diagnostic accuracy of EUS-FNA with ROSE by endosonographers compared to ROSE by cytopathologists in patients with solid pancreatic masses. METHODS: Between September 2001 and October 2005, of the 73 EUS-FNA procedures with the final diagnoses, 38 procedures after the introduction of ROSE by endosonographers (September 2001-September 2003, period 1), and 35 procedures after the introduction of ROSE by cytopathologists (October 2003-October 2005, period 2) were included. The specimens were stained with Diff-Quik stain and assessed. When the on-site assessors (endosonographers or cytopathologists) indicated that the amounts of cell samples were adequate, the procedure was stopped. RESULTS: Results are presented with 95% confidence limits. The average numbers of needle passes were 4.0 +/- 1.6 and 3.4 +/- 1.5 in periods 1 and 2, respectively (P = 0.06). The specimen collection rates were 97.4 and 97.1% in periods 1 and 2, respectively (P = 0.51). Sensitivity, specificity, positive predictive value, negative predictive value, and accuracy for malignancy and benign were 92.9, 100, 100, 83.3, and 94.7%, respectively, in period 1, and 93.1, 100, 100, 75.0, and 94.3%, respectively, in period 2 (P = 0.97, P = 1.0, P = 1.0, P = 0.65, P = 0.93, respectively). No complications were seen. CONCLUSIONS: For accurate diagnosis, ROSE should be performed during EUS-FNA by the endosonographer, if no cytopathologist is available.