Validation of the Diagnostic Criteria for IgG4-Related Periaortitis/Periarteritis and Retroperitoneal Fibrosis (IgG4PA/RPF) 2018, and Proposal of a Revised 2023 Version for IgG4-Related Cardiovascular/Retroperitoneal Disease.

BACKGROUND: In 2018, diagnostic criteria were introduced for IgG4-related periaortitis/periarteritis and retroperitoneal fibrosis (PA/RPF). This study assessed the existing criteria and formulated an improved version.Methods and Results: Between August 2022 and January 2023, we retrospectively analyzed 110 Japanese patients diagnosed with IgG4-related disease (IgG4-RD) involving cardiovascular and/or retroperitoneal manifestations, along with 73 non-IgG4-RD patients ("mimickers") identified by experts. Patients were stratified into derivation (n=88) and validation (n=95) groups. Classification as IgG4-RD or non-IgG4-RD was based on the 2018 diagnostic criteria and various revised versions. Sensitivity and specificity were calculated using experts' diagnosis as the gold standard for the diagnosis of true IgG4-RD and mimickers. In the derivation group, the 2018 criteria showed 58.5% sensitivity and 100% specificity. The revised version, incorporating "radiologic findings of pericarditis", "eosinophilic infiltration or lymphoid follicles", and "probable diagnosis of extra-PA/-RPF lesions", improved sensitivity to 69.8% while maintaining 100% specificity. In the validation group, the original and revised criteria had sensitivities of 68.4% and 77.2%, respectively, and specificities of 97.4% and 94.7%, respectively. CONCLUSIONS: Proposed 2023 revised IgG4-related cardiovascular/retroperitoneal disease criteria show significantly enhanced sensitivity while preserving high specificity, achieved through the inclusion of new items in radiologic, pathological, and extra-cardiovascular/retroperitoneal organ categories.

Sarcopenia Aneurysm Scoring System Predicts Early and Midterm Mortality After Endovascular Repair for Abdominal Aortic Aneurysm.

OBJECTIVE: The objective of the study was to develop a better model of prediction after EVAR using the psoas muscle index (PMI). SUMMARY BACKGROUND DATA: The Glasgow Aneurysm Score (GAS), the modified Leiden Score (mLS), the Comorbidity Severity Score (CSS), and the Euro Score (ES) are known prognostic scoring after EVAR. Similarly, sarcopenia measured by PMI has been reported to be an important predictor. This study investigated a new scoring system using PMI predicting short and midterm overall mortality after EVAR. METHODS: Three hundred ten patients were retrospectively evaluated. The primary endpoint was all-cause death. One hundred three patients were assigned to the derivation cohort and 207 patients to the validation cohort. RESULTS: The all-cause mortality rates were 8.8% at 1 year, 23.5% at 3 years, and 32.8% at 5 years. In a multivariate analysis, age, aneurysm diameter, eGFR, and PMI were associated with all-cause mortality in the derivation cohort. The SAS system was defined as the sum of the following factors: elderly (75 years), large aneurysm (65 mm), low eGFR (30 mL/min/1.73m 2 ), and low PMI (males: 48.2 cm 2 /m 2 , females: 36.8 cm 2 /m 2 ). We compared the SAS with the other prognostic scoring for 5-year mortality evaluating the area under the receiver operating characteristic curves in the validation cohort (GAS: 0.731, mLS: 0. 718, CSS: 0. 646, ES: 0.661, and SAS: 0.785, P = 0.013). CONCLUSION: We developed the SAS to predict all-cause mortality after elective EVAR and this scoring showed excellent predictive performance.

Risk factors for refractory enterocutaneous fistula following button jejunostomy removal and its treatment using a novel extraperitoneal approach in patients with oesophageal cancer: a retrospective cohort study.

BACKGROUND: Enterocutaneous fistula after removal of the jejunostomy tube leads to multiple problems, such as cosmetic problems, decreased quality of life, electrolyte imbalances, infectious complications, and increased medical costs. However, the risk factors for refractory enterocutaneous fistula (REF) after button jejunostomy removal remain unclear. Therefore, in this study, we assessed the risk factors for REF after button jejunostomy removal in patients with oesophageal cancer and reported the surgical outcomes of the novel extraperitoneal approach (EPA) for REF closure. METHODS: This retrospective cohort study included 47 patients who underwent button jejunostomy removal after oesophagectomy for oesophageal cancer. We assessed the risk factors for REF in these patients and reported the surgical outcomes of the novel EPA for REF closure at the International University of Health and Welfare Hospital between March 2013 and October 2021. The primary endpoint was defined as the occurrence of REF after removal of the button jejunostomy, which was assessed using a maintained database. The risk factors and outcomes of the EPA for REF closure were retrospectively analysed. RESULTS: REFs occurred in 15 (31.9%) patients. In the univariate analysis, REF was significantly more common in patients with albumin level < 4.0 g/dL (p = 0.026), duration > 12 months for button jejunostomy removal (p = 0.003), and with a fistula < 15.0 mm (p = 0.002). The multivariate analysis revealed that a duration > 12 months for button jejunostomy removal (odds ratio [OR]: 7.15; 95% confidence interval [CI]: 1.38-36.8; p = 0.019) and fistula < 15.0 mm (OR: 8.08; 95% CI: 1.50-43.6; p = 0.002) were independent risk factors for REF. EPA for REF closure was performed in 15 patients. The technical success rate of EPA was 88.2%. Of the 15 EPA procedures, fistula closure was achieved in 12 (80.0%). The complications of EPA (11.7%) were major leakages (n = 3) and for two of them, EPA procedure was re-performed, and closure of the fistula was finally achieved. CONCLUSION: This study suggested that duration > 12 months for button jejunostomy removal and fistula < 15.0 mm are the independent risk factors for REF after button jejunostomy removal. EPA for REF closure is a novel, simple, and useful surgical option for patients with REF after oesophagectomy.

The masseter muscle thickness is a predictive marker for postoperative pneumonia after endovascular aneurysm repair.

PURPOSE: Oral frailty is characterized by a decrease in the oral and swallowing function and is a risk factor for pneumonia. In the current study, we analyzed the association between the masseter muscle thickness (MMT) and postoperative pneumonia and mortality after endovascular aneurysm repair (EVAR) for abdominal aortic aneurysm. METHODS: Overall, 247 patients were retrospectively evaluated. The primary end point was postoperative pneumonia. The MMT was measured as the maximum thickness of the masseter muscle 2 cm caudal to the zygomatic arch using computed tomography images obtained within 3 months before EVAR. Pneumonia was defined as the presence of progressive infiltrates, consolidation, or cavitation on imaging and a fever or leukocytosis. RESULTS: Twenty (8.1%) cases of postoperative pneumonia occurred within 1 year after EVAR. We found that patients with a low MMT (≤ 30th percentile: males, 10.4 mm; females: 8.8 mm) had a significantly higher risk of developing postoperative pneumonia within 1 year after elective EVAR than those with a high value. A comparison of the utility of the MMT and psoas muscle index (PMI) for predicting the 1-, 3-, and 5-year all-cause mortality revealed that the MMT had superior predictive performance. CONCLUSION: The MMT before elective EVAR predicted postoperative pneumonia and life expectancy, and its predictive performance was superior to that of the PMI.

New Scoring System for Predicting the Risk of Surgical Site Infections Following Stoma Reversal.

BACKGROUND: Surgical site infections (SSI) are one of the most frequent complications following stoma reversal (SR-SSI) and lead to multiple problems such as decreased mobility of the patients or increased hospital costs. Several risk factors for SR-SSI have been reported, but there are no risk scoring systems for predicting SR-SSI. The current study aimed to analyze the risk factors for SR-SSI and develop a scoring system. MATERIALS AND METHODS: Multivariate analysis of risk factors for SR-SSI was performed in patients who underwent elective SR and were followed-up during the first month after surgery. A logistic regression model was used to identify risk factors and construct a predictive score. RESULTS: Of the 182 patients, 53 (29.1%) developed SSI. In multivariate analysis, three variables as preoperative risk factors were associated with increased SR-SSI incidence: subcutaneous fat thickness (≥ 20 mm) (odds ratio [OR]: 8.46 [95% confidence interval (CI): 3.45-20.7], P <0.001), period from stoma creation (≤ 20 weeks) (OR: 2.88 [95% CI: 1.14-7.28], P = 0.025), and SSI after the primary operation (OR: 3.06 [95% CI: 1.19-7.90], P = 0.021). Each of these variables contributed 2,1, and 1 points to the risk score, respectively. The SR-SSI rate was 2.9%, 20.3%, 34.2%, 54.5%, and 81.8% for the scores of 0,1,2,3, and 4 points, respectively. The area under the receiver operating characteristic curve was 0.773 (95% CI: 0.703-0.844). CONCLUSIONS: A simple clinical scoring system based on three preoperative variables may be useful in predicting the risk of SR-SSI.

Dermatomyositis associated with prostate adenocarcinoma with neuroendocrine differentiation.

BACKGROUND: Although it is known that malignancies can be associated with dermatomyositis, there are few reports on dermatomyositis associated with prostate cancer with neuroendocrine differentiation. CASE PRESENTATION: A 63-year-old man visited our hospital due to pollakiuria. High levels of PSA and NSE were observed, and prostate biopsy revealed an adenocarcinoma with neuroendocrine differentiation. Multiple metastases to the lymph nodes, bones, and liver were identified, and androgen deprivation therapy (ADT) was started immediately. Following 2 weeks of treatment, erythema on the skin, and muscle weakness with severe dysphagia appeared. The patient was diagnosed with dermatomyositis, and high-dose glucocorticoid therapy was initiated. ADT and subsequent chemotherapy with etoposide and cisplatin (EP) were performed for prostate cancer, which resulted in decreased PSA and NSE and reduction of all metastases. After the initiation of EP therapy, dermatomyositis improved, and the patient regained oral intake function. Although EP therapy was replaced by docetaxel, abiraterone, and enzalutamide because of adverse events, no cancer progression was consistently observed. Dermatomyositis worsened temporarily during the administration of abiraterone, but it improved upon switching from abiraterone to enzalutamide and dose escalation of glucocorticoid. CONCLUSIONS: We successfully treated a rare case of dermatomyositis associated with prostate adenocarcinoma with neuroendocrine differentiation.

Vertical distance from navel as a risk factor for bowel obstruction associated with feeding jejunostomy after esophagectomy: a retrospective cohort study.

BACKGROUND: Placement of feeding jejunostomy (PFJ) during esophagectomy is an effective method to maintain adequate nutrition, but is associated with serious complications such as bowel obstruction and jejunal torsion. The purpose of the current study was to analyze the incidence, clinical features, and risk factors of bowel obstruction associated with feeding jejunostomy (BOFJ) after PFJ. METHODS: This was a retrospective cohort study of 70 patients who underwent esophagectomy with three-field lymph node dissection for esophageal cancer and treated with PFJ between March 2013 and December 2019 in our hospital. Abdominal dissection was performed under hand-assisted laparoscopic surgery (HALS) from March 2013 to March 2015, and was changed to complete laparoscopic surgery in April 2015. We compared patients with and without BOFJ, and the incidence of BOFJ was evaluated. The primary endpoint was incidence of BOFJ after PFJ. RESULTS: Six patients (8.5%) were diagnosed with BOFJ, all of whom were symptomatic and in the HALS group. In addition, 3 cases displayed histories of recurrent BOFJ (3, 3, and 5 times). Laparotomy was performed in all cases. Subgroup analysis of the HALS group showed a significant difference only in straight-line distance between the jejunostomy and navel as a significant pre- and perioperative factor (117 mm [101-130 mm] vs. 89 mm [51-150 mm], p < 0.001). Furthermore, dividing straight-line distance between the jejunostomy and navel into VD and HD, only VD differed significantly (107 mm [93-120 mm] vs. 79 mm [28-135 mm], p = 0.010), not HD (48 mm [40-59 mm] vs. 46 mm [22-60 mm], p = 0.199). CONCLUSIONS: VD between the jejunostomy and navel was associated with BOFJ after PFJ with HALS esophagectomy. PFJ < 9 cm above the navel during HALS esophagectomy might effectively prevent BOFJ.

Aortic Wall Enhancement Detected by Contrast Computed Tomography Scan Predicts Aortic Remodeling after Conservative Therapy for Acute Uncomplicated Type B Dissection.

BACKGROUND: The Investigation of Stent Grafts in Aortic Dissection with extended length of follow-up trial showed that pre-emptive thoracic endovascular aneurysm repair (TEVAR) for the uncomplicated type B dissection (TBAD) in the subacute phase promotes aortic remodeling and avoids aortic-related death 5 years after onset. However, there are some patients with complete aortic remodeling (CAR) with optimal medical treatment (OMT) and severe complications after TEVAR such as retrograde type A dissection. Therefore, which patients should undergo pre-emptive TEVAR and optimal surgical timing are still under debate. We reported that aortic wall enhancement (AWE) after endovascular aneurysm repair for abdominal aortic aneurysm was associated with sac shrinkage. However, there is no report about the relationship between AWE and aortic dissection. Herein, we evaluated the relationship between AWE and acute TBAD. METHODS: From March 2012 to May 2018, consecutive patients with acute TBAD were retrospectively collected. We retrospectively analyzed 35 patients with acute TBAD who were treated with OMT and without pre-emptive TEVAR in the subacute phase. AWE was defined as an increase of more than 20 Hounsfield units in mean computed tomography (CT) values, comparing images in delayed contrast-enhanced scans with those in plain scans evaluated within 3 months from onset. The measurement points were all slices including the wall of the false lumen. The patients with traumatic dissection, type A dissection, acute complicated type B dissection, chronic (>12 weeks) dissection, and those lost to follow-up within 3 months from onset were excluded. The primary end point was spontaneous CAR under OMT, as determined by the latest contrast-enhanced CT scan. RESULTS: The median follow-up period from onset was 86 weeks and there were 25 cases (71.4%) with AWE. Under OMT, CAR was observed in 20 patients (57.1%); this was significantly associated with abdominal branch dissection (6/15 [40%] vs. 2/20 [10%], P = 0.050), number of tears more than 2 at onset (11/15 [73%] vs. 4/20 [20%], P = 0.003), multiple tears at 1 month after onset (9/15 [60%] vs. 4/20 [20%], P = 0.020), maximal false lumen diameter at 1 month after onset (14 vs. 8 mm, P = 0.025), and AWE within 3 months of onset (7/15 [47%] vs. 18/20 [90%], P = 0.010). Multivariate analysis demonstrated a significant difference with multiple tears at onset (P = 0.014) and AWE within 3 months of onset (P = 0.047). CONCLUSIONS: AWE was associated with CAR under OMT for acute TBAD which is out of indication of pre-emptive TEVAR. Presence of AWE may be useful in predicting prognosis of TBAD.

Initial results of thoracic endovascular repair for uncomplicated type B aortic dissection involving the arch vessels using a semicustom-made thoracic fenestrated stent graft.

OBJECTIVE: Thoracic endovascular aortic repair (TEVAR) for selected type B aortic dissection (TBAD) is a standard treatment; however, TBAD involving the aortic arch is difficult to treat because of the need for arch vessel reconstruction. We report our initial results of TEVAR for uncomplicated TBAD involving the arch vessels using a semicustom-made fenestrated stent graft. METHODS: This is a retrospective study of 24 patients treated by fenestrated (F group) or debranching (D group) TEVAR from August 2011 to July 2017. The patients in the F group received the Najuta semicustom-made fenestrated stent graft (Kawasumi Laboratories, Tokyo, Japan). The fenestrated graft ensures sufficient sealing at the proximal healthy aorta without the need for arch vessel reconstruction. The primary end point was aorta-related mortality; the secondary end points were technical success and major adverse events (stroke, type IA endoleak, retrograde type A aortic dissection, and secondary intervention). RESULTS: During the study period, we treated 65 TBAD cases by TEVAR, including 17 complicated cases. Of the 48 uncomplicated cases, 24 underwent TEVAR with arch vessel involvement (13 in the F group and 11 in the D group). The technical success rates in the F and D groups were 92.3% and 100.0%, respectively (P > .99, NS). The mean operation time was significantly shorter in the F group (158 minutes) than in the D group (202 minutes; P = .0426), and the mean postoperative hospital stay was also significantly shorter in the F group (7 days) than in the D group (22 days; P = .0168). The primary patency rate of the reconstructed branch vessel was 100%, and there were no aorta-related deaths or retrograde type A aortic dissection in either group. One patient had a type IA endoleak in the F group. In the D group, one patient had a postoperative stroke and two patients required secondary interventions for stent graft-induced new entry at the descending aorta. The median follow-up period was 14.1 months (range, 1-37 months). The rate of freedom from aorta-related death was 100% in both groups (P > .99, NS); the rate of freedom from major adverse events at 24 months was 92.3% in the F group and 72.7% in the D group (P = .749, NS). CONCLUSIONS: The initial results of TEVAR with aortic arch vessel reconstruction for uncomplicated TBAD were acceptable. The fenestrated graft may be a less invasive option for the treatment of TBAD involving the aortic arch.

Risk Factor Analyses of Abdominal Aortic Aneurysms Growth in Japanese Patients.

BACKGROUND: This study aimed to retrospectively demonstrate the growth rate (mm/year) of abdominal aortic aneurysm (AAA) diameters (ADs) and to analyze risk factors for AAA expansion. METHODS: We retrospectively investigated the clinical data of 319 patients with AAAs who were followed up as outpatients for >2 years after their initial visit and who underwent computed tomography >4 times. RESULTS: The mean follow-up period was 3.7 ± 1.5 years. The annual average growth rates according to varying ADs were as follows: 1.9 ± 0.8 (AD 30-34 mm), 2.6 ± 1.2 (AD 35-39 mm), 2.8 ± 1.1 (AD 40-44 mm), 3.1 ± 1.3 (AD 45-49 mm), 3.4 ± 1.6 (AD 50-54 mm), and 3.5 ± 1.4 mm (AD ≥55 mm). Factors associated with AAA expansion were smoking (P = 0.017), hypertension (P < 0.001), and ADs (P < 0.001). In the subgroup analysis, data regarding growth rates of ≥3 mm were extracted, and a statistically significant difference between smoking status and ADs of ≥40 mm was observed. CONCLUSIONS: Factors associated with AAA expansion in Japanese patients included smoking, hypertension, and ADs, and a statistically significant difference was observed between smoking status and ADs of ≥40 mm.

Prophylactic retention suture for surgical site infection: a retrospective cohort study.

BACKGROUND: Surgical site infection (SSI) is a common complication of gastrointestinal surgery. Because retention suture is known to prevent abdominal wound dehiscence, it is only considered indicated in high-risk patients. At present, there are no clear indications for retention suture. The purpose of this study was to analyze the effect of prophylactic retention suture and to determine what situations indicate prophylactic retention suture against SSI. MATERIAL AND METHODS: Between January 2014 and January 2016, 135 patients who underwent midline laparotomy in our hospital were analyzed. Inclusion criteria for this study were patients with American Society Anesthesiologists' physical status classification system (ASA-PS score) ≥ 3 or emergent surgery. RESULTS: Of the 135 patients, 30 (22.2%) received prophylactic retention suture. Diabetes mellitus, surgical wound classification, large incision, and retention suture were associated with SSI in multivariate analysis. In subgroup analysis, SSI risk factors were analyzed in each surgical wound classification. Only in surgical wound classification class II and III did retention suture significantly reduce the risk of SSI (odds ratio = 0.100 [0.012-0.837], P = 0.034). In class IV, however, half the patients developed SSI, regardless of retention suture. Table 3 summarizes the results of the subgroup analysis. CONCLUSIONS: The present data suggest that prophylactic retention suture reduces SSI for surgical wound classification class II or III. For class IV operations, however, other methods to prevent SSI are necessary.

Aneurysm Wall Enhancement Detected by Contrast Computed Tomography Scan Is Associated With Aneurysm Shrinkage After Endovascular Aneurysm Repair for Abdominal Aortic Aneurysm.

BACKGROUND: Aneurysm expansion, and consequent endoleaks, after endovascular aneurysm repair (EVAR) is a major problem. Accurate prediction of aneurysm expansion is demanding for surgeons and remains difficult.Methods and Results:We retrospectively analyzed 157 cases of EVAR for abdominal aortic aneurysm (AAA) using a bifurcated main-body stent-graft. There were 62 cases of aneurysm shrinkage after EVAR, 63 cases of stable aneurysm, and 32 cases of aneurysm expansion. Type I endoleaks were significantly increased in the aneurysm expansion group (EXP) compared with the stable (STB) and shrinkage (SHR) groups (EXP: 15.6% vs. STB: 4.8% vs. SHR: 0%, P=0.005). Type II endoleaks were also significantly increased in EXP (EXP: 65.6% vs. STB: 36.5% vs. SHR: 6.5%, P<0.001). Aneurysm wall enhancement (AWE) on imaging, however, was significantly decreased in the EXP group (EXP: 18.8% vs. STB: 23.8% vs. SHR: 53.2%, P<0.001). In multivariate analysis, the occurrence of type II endoleaks significantly decreased (P<0.001) and that of AWE significantly increased the likelihood of aneurysm shrinkage (P=0.032). CONCLUSIONS: AWE following EVAR may be associated with aneurysm shrinkage.

Oncological and peri-surgical outcomes of radical prostatectomy for non-metastatic prostate cancer with prostate-specific antigen level of 50 ng/ml or greater.

BACKGROUND: The role of radical prostatectomy in treating non-metastatic prostate cancer patients with high prostate-specific antigen levels remains unclear. We evaluated the feasibility and oncological outcomes of radical prostatectomy in non-metastatic prostate cancer patients with prostate-specific antigen levels of 50 ng/ml or higher. METHODS: This retrospective study included 31 patients who were diagnosed as very high-risk prostate cancer (clinical stage of any T, N0-1 M0 and PSA levels ≥50 ng/ml) and underwent radical prostatectomy either as a monotherapy or as a component of multimodal therapy (RP group). Surgery-related complications were investigated. Time to castration-resistant prostate cancer, cancer-specific survival, and overall survival were estimated using the Kaplan-Meier method. A total of 47 patients with very high-risk prostate cancer who were treated with androgen deprivation therapy without local therapy served as a control group (ADT group). Survivals were compared between RP group and ADT group in exploratory analyses. RESULTS: The median pretreatment prostate-specific antigen was 87 ng/ml and 100 ng/ml in the RP and ADT groups, respectively (P = 0.67). Surgical complications of Clavien-Dindo Grade 3 were documented in nine patients (29%). Ten-year castration-resistant prostate cancer-free, cancer-specific and overall survivals were 78%, 81% and 77% in RP group, respectively, and they were significantly better than those of ADT group (54%, P = 0.006; 54%, P = 0.006 and 38%, P < 0.001). Exploratory multivariate analysis identified radical prostatectomy as the only significant factor associated with a better cancer-specific survival (hazard ratio: 0.25, P = 0.006). CONCLUSIONS: Radical prostatectomy is feasible for non-metastatic prostate cancer patients with prostate-specific antigen levels of 50 ng/ml or higher. Radical prostatectomy is a viable option for select patients with non-metastatic, very high-risk prostate cancer.

Endovascular Aneurysm Repair-First Strategy for Ruptured Aneurysm Focuses on Fitzgerald Classification and Vein Thrombosis.

BACKGROUND: Recent study have demonstrated the good results of endovascular aneurysm repair (EVAR) for ruptured abdominal aortic aneurysms (RAAAs). We report on the results of our EVAR-first strategy for RAAAs focuses on Fitzgerald (F) classification and vein thrombosis. MATERIALS AND METHODS: From 2011 to 2017, 31 patients with RAAA underwent EVAR at our hospital. We compared F-1 patients (group A) with F-2 to F-4 patients with obvious retroperitoneal hematoma (group B). RESULTS: The baseline characteristics in group A (n = 9) and group B (n = 22) were similar. In group B, there were 8 cases of F-2, 10 cases of F-3, and 4 cases of F-4. Of the 22 cases in group B, 16 (73%) cases involved preoperative shock. Operation time was not significantly different (group A: 147 min and group B: 131 min, P = 0.48). The total mortality rate of group A and group B combined was 77.4%. The 30-day mortality was 0% for group A and 23.8% for group B, in which there were 2 F-4 cases and 3 F-3 cases. In group B, hematoma-related complications developed in 6 cases (deep vein thrombosis: 4 cases, abdominal compartment syndrome: 1 case, and hematoma infection: 1 case), and 1 case with deep vein thrombosis developed a pulmonary embolism that resulted in cardiac arrest. The 3-year survival rate was significantly higher for group A (100% vs. 52.3%, P = 0.016), but the freedom from aortic death rate was not significantly different (100% vs. 66.7%, P = 0.056). CONCLUSIONS: Using EVAR for RAAA is a valid strategy. Certain complications that are associated with peritoneal hematoma, especially venous thrombosis, should receive particular attention.

[Acquisition of IgH/CCND1 translocation during the natural disease course in a patient with chronic lymphocytic leukemia].

A 69-year-old man visited a doctor because of systemic lymphadenopathy. Peripheral blood examination revealed leukocytosis, anemia, and decreased platelet count (WBC, 103,060/µl; lymph, 92.2%; Hb, 8.9 g/dl; and Plt, 4.1×104/µl). Bone marrow biopsy revealed that approximately 70% of nucleated cells were small, mature lymphoid cells with positive immunostaining for CD5, CD20, and CD23. He was diagnosed with chronic lymphocytic leukemia (CLL). The IgH/CCND1 translocation and ATM locus loss in 20% and 95% peripheral cells, respectively, were detected by fluorescence in situ hybridization. Immunostaining revealed that cyclin D1 was positive in approximately 30% bone marrow cells. As the positive rate of CCND1 fusion signal was low, the diagnosis of mantle cell lymphoma was excluded. In contrast, signals of ATM locus deletion were detected in most tumor cells. Therefore, we assessed that IgH/CCND1 translocations occurred during the natural clinical course of CLL with ATM locus deletion from the onset of disease. The secondary IgH/CCND1 translocation in CLL is rare, and all reported cases with such translocations received treatments with alkylating agents. This is the first report regarding secondary IgH/CCND1 translocation during the natural clinical course of CLL and may provide insights into CLL pathogenesis.

Crus incision without repair is a risk factor for esophageal hiatal hernia after laparoscopic total gastrectomy: a retrospective cohort study.

BACKGROUND: Although postoperative esophageal hiatal hernia (EHH) is primarily considered a post-operative complication of esophagectomy, it is also a rare post-operative complication of laparoscopic total gastrectomy (LTG), with a reported incidence rate of 0.5 %. The purpose of this study is to analyze the incidence, clinical features, and prevention of EHH following LTG for gastric cancer. METHODS: Between October 2008 and July 2014, 78 patients who underwent LTG for gastric cancer in our hospital were analyzed. We compared the crus incision group (in which the left crus of the diaphragm was incised without suture repair) with the crus conserving or repair group (in which the crus was preserved or the crus was incised and underwent suture repair). The primary endpoint was incidence of postoperative EHH. RESULTS: Of the 78 patients, 7 (9.0 %) developed postoperative EHH. Three of seven patients (42.9 %) were symptomatic and required an emergency operation for intestinal obstruction. Four of seven patients (57.1 %) were asymptomatic and did not require an operation. Incising the left crus of the diaphragm without suture repair during LTG was considered the only risk factor for postoperative EHH (0 of 29 for preserving the crus or incising and performing suture repair of the crus vs. 7 of 49 in crus incision without suture repair; p = 0.033). CONCLUSIONS: The present data suggest that incision of the crus without suture repair is associated with EHH after LTG. If crus incision is required, crus repair may be effective for the prevention of postoperative EHH.

q-space MR imaging of gastric carcinoma ex vivo: Correlation with histopathologic findings.

PURPOSE: The purpose of this study was to establish the feasibility of q-space imaging (QSI) as a method of assessing the depth of mural invasion, histologic grade, and the presence of lymph node metastasis in gastric carcinomas. METHODS: A 7.0 Tesla MR imaging system was used to investigate 20 gastric specimens containing a carcinoma. QSI was performed by using the following parameters: 50-60 mm × 25-30 mm field of view, 2-mm section thickness, 256 × 128 matrix, 10 b values in the 0-7163 s/mm(2) range, which corresponded to q values of 0-1026/cm, and motion-probing gradients perpendicular to the gastric wall. The MR images and the histopathologic findings were then compared. RESULTS: The depth of tumor invasion of the gastric wall in all 20 carcinomas (100%) was established by using mean displacement, zero-displacement probability, and kurtosis maps. The QSI parameters were significantly correlated with the histologic grades of the gastric carcinomas (all P < 0.001). The QSI parameters made it possible to differentiate between metastatic and nonmetastatic lymph nodes (all P = 0.001). CONCLUSION: Ex vivo QSI facilitates excellent diagnostics for evaluating gastric carcinomas in terms of mural invasion, histologic grade, and the presence of lymph node metastasis. Magn Reson Med 76:602-612, 2016. © 2015 Wiley Periodicals, Inc.

Development of myelofibrosis during eltrombopag treatment in a patient with immune thrombocytopenia.

Eltrombopag, a thrombopoietin (TPO) receptor agonist, is effective for treating refractory immune thrombocytopenia (ITP). However, the development of bone marrow fibrosis is a concern. A 78-year-old man was diagnosed with ITP in 2004. His platelet count did not increase after eltrombopag treatment initiation in 2014. However, anemia progressed, along with the presence of immature myelocytes, erythroblasts, and tear drop cells. At 8 months after initiating eltrombopag treatment, the patient underwent a bone marrow biopsy that showed grade 2 myelofibrosis. Hence, eltrombopag was discontinued. In our experience with this case indicates that careful observation is required while using TPO receptor agonists.

Gastric Carcinoma: Ex Vivo MR Imaging at 7.0 T-Correlation with Histopathologic Findings.

PURPOSE: To determine the imaging detail and diagnostic information that can be obtained at 7.0-T magnetic resonance (MR) imaging with a voxel volume of 9.5-14.0 nL as a means of evaluating the depth of mural invasion by gastric carcinomas ex vivo. MATERIALS AND METHODS: This study was approved by the institutional review board, and written informed consent was obtained from each patient. Twenty gastric specimens containing 20 carcinomas were studied with a 7.0-T MR imaging system equipped with a four-channel surface coil. MR images were obtained with a 50-60 × 25-30 mm field of view, a 512 × 256 matrix, and a 1.0-mm section thickness, resulting in a voxel volume of 0.0095-0.0140 mm(3) (9.5-14.0 nL). The signal intensity of the gastric wall layers, tumor tissue, and fibrosis was described as low, intermediate, or high by comparing it with the signal intensity of the muscularis propria. Depth of invasion initially was assessed by two reviewers independently and then by the two reviewers in consensus. MR images were compared with histopathologic findings. RESULTS: The 7.0-T T2-weighted MR images clearly depicted the normal gastric wall in all 20 specimens (100%) as consisting of seven layers, which clearly corresponded to the tissue layers of the gastric wall. These MR images enabled clear differentiation between tumor tissue and fibrosis. Reviewers disagreed on the depth of invasion at the initial reading in three (15%) of 20 specimens (between mucosa and submucosa in two specimens and between muscularis propria and subserosa and serosa in one specimen); however, in all 20 gastric carcinomas, the depth of invasion could be accurately determined on T2-weighted images after consensus interpretation. CONCLUSION: Ex vivo 7.0-T MR imaging enables clear delineation of the gastric wall layers and clear differentiation of tumor tissue from fibrosis and allows one to assess the depth of mural invasion by gastric carcinomas.