Journal of Nippon Medical School
Online ISSN : 1347-3409
Print ISSN : 1345-4676
ISSN-L : 1345-4676
Volume 90, Issue 1
Displaying 1-17 of 17 articles from this issue
Reviews
  • Akihisa Matsuda, Takeshi Yamada, Ryo Ohta, Hiromichi Sonoda, Seiichi S ...
    2023 Volume 90 Issue 1 Pages 2-10
    Published: February 25, 2023
    Released on J-STAGE: March 11, 2023
    Advance online publication: May 30, 2022
    JOURNAL FREE ACCESS

    Surgical site infections (SSIs) remain one of the most common serious surgical complications and are the second most frequent healthcare-associated infection. Patients with SSIs have a significantly increased postoperative length of hospital stay, hospital expenses, and mortality risk compared with patients without SSIs. The prevention of SSI requires the integration of a range of perioperative measures, and approximately 50% of SSIs are preventable through the implementation of evidence-based preventative strategies. Several international guidelines for SSI prevention are currently available worldwide. However, there is an urgent need for SSI prevention guidelines specific to Japan because of the differences in the healthcare systems of Japan versus western countries. In 2018, the Japan Society for Surgical Infection published SSI prevention guidelines for gastroenterological surgery. Although evidence-based SSI prevention guidelines are now available, it is important to consider the appropriateness of these guidelines depending on the actual conditions in each facility. A systemic inflammatory host response is a hallmark of bacterial infection, including SSI. Therefore, blood inflammatory markers are potentially useful in SSI diagnosis, outcome prediction, and termination of therapeutic intervention. In this review, we describe the current guideline-based perioperative management strategies for SSI prevention, focusing on gastroenterological surgery and the supplemental utility of blood inflammatory markers.

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  • Ryuzo Bessho
    2023 Volume 90 Issue 1 Pages 11-19
    Published: February 25, 2023
    Released on J-STAGE: March 11, 2023
    Advance online publication: May 30, 2022
    JOURNAL FREE ACCESS

    Neuroprotection is important in open aortic arch surgery because of the dependence of brain tissues on cerebral perfusion. Therefore, several techniques have been developed to reduce cerebral ischemia and improve outcomes in open aortic arch surgery. In this review, I describe various neuroprotective strategies, such as profound and deep hypothermic circulatory arrest, selective antegrade cerebral perfusion, retrograde cerebral perfusion, and lower body circulatory arrest; compare their advantages and disadvantages, and discuss their evolution and current status by reviewing relevant literature.

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  • Hiroshi Yoshida, Tetsuya Shimizu, Masato Yoshioka, Akira Matsushita, Y ...
    2023 Volume 90 Issue 1 Pages 20-25
    Published: February 25, 2023
    Released on J-STAGE: March 11, 2023
    JOURNAL FREE ACCESS

    As liver disease progresses, intrahepatic vascular resistance increases (backward flow theory of portal hypertension) and collateral veins develop. Adequate portal hypertension is required to maintain portal flow into the liver through an increase in blood flow into the portal venous system (forward flow theory of portal hypertension). The splenic artery resistance index is significantly and selectively elevated in cirrhotic patients. In portal hypertension, a local hyperdynamic state occurs around the spleen. Splenomegaly is associated with a poor prognosis in cirrhosis and is caused by spleen congestion and by enlargement and hyperactivation of splenic lymphoid tissue. Hypersplenism can lead to thrombocytopenia caused by increased sequestering and breakdown of platelets in the spleen. The close relationship between the spleen and liver is reflected in the concept of the hepatosplenic axis. The spleen is a regulatory organ that maintains portal flow into the liver and is the key organ in the forward flow theory of portal hypertension. This review summarizes the literature on the role of the spleen in portal hypertension.

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Originals
  • Shigeru Matsuda, Masao Ichikawa, Hanako Kaseki, Kenichiro Watanabe, Sh ...
    2023 Volume 90 Issue 1 Pages 26-32
    Published: February 25, 2023
    Released on J-STAGE: March 11, 2023
    Advance online publication: October 21, 2022
    JOURNAL FREE ACCESS

    Background: Accurate diagnosis of retroflexed uterus in daily practice is essential because this condition is related to pelvic pain and deep endometriosis. Uterine flexion can be measured by transvaginal ultrasonography (TVUS), a cost-effective primary test, but the accuracy required for diagnosing retroflexed uterus is unclear. This study assessed the accuracy of TVUS for diagnosis of retroflexed uterus in patients with endometriosis and compared it with that of magnetic resonance imaging (MRI) -the gold standard for measuring the uterine axis. Methods: The study included 123 patients who underwent endometriosis surgery in our department between 2012 and 2017. Uterine flexion angles were measured by retrospectively examining TVUS and MRI images, and the correlation was analyzed. Analysis of anteverted and retroverted uterine subgroups identified aspects of diagnosing uterine flexion with TVUS. Results: Uterine flexion angles on TVUS were strongly positively correlated (r = 0.86) with MRI results. Additionally, TVUS yielded no false-positive diagnoses and 28 false-negative diagnoses of retroflexion. All false-negative diagnoses occurred in patients with anteverted retroflexed uteruses. Conclusions: TVUS was generally accurate for measuring uterine flexion angle, as indicated by its strong correlation with MRI. Misdiagnosis of anteverted retroflexed uterus was a limitation of using TVUS for retroflexion diagnosis.

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  • Ryohei Nishiguchi, Shinichi Asaka, Takeshi Shimakawa, Teppei Kono, Sac ...
    2023 Volume 90 Issue 1 Pages 33-40
    Published: February 25, 2023
    Released on J-STAGE: March 11, 2023
    Advance online publication: October 21, 2022
    JOURNAL FREE ACCESS

    Background: Totally extraperitoneal (TEP) repair is a recommended procedure for inguinal hernia repair in European hernia guidelines. However, technical challenges have limited its uptake in Japan, where transabdominal preperitoneal (TAPP) repair is more common. We evaluated the association of preoperative subcutaneous fat area (preSFA) with surgical outcomes and identified factors associated with the difficulty of TEP repair. Methods: Clinical data from 62 patients undergoing TEP repair were collected retrospectively. Using the median for the preoperative subcutaneous fat index (preSFI; 45.9 cm2/m2), we classified patients as having a high SFI (HSFI) (n=31) and low SFI (LSFI) (n=31). Surgical outcomes and perioperative complications were then compared between these groups. Additionally, TEP repair was divided into five phases (e.g., Phase 1: dissection of the caudal side of the preperitoneal space), and operative time was measured during each phase. Phase 1 was divided into two sub-phases (1A: insertion of the first port, 1B: reaching Cooper's ligament). Results: Operative time was longer (133 min vs 111 min, P = 0.028) and the peritoneal injury rate was higher (35.5% vs 9.7%, P = 0.015) for the HSFI patients. Furthermore, operative time for HSFI patients was significantly longer during Phase 1 (P = 0.014) and Phase 1A (P = 0.022). Conclusions: preSFA was associated with a higher peritoneal injury rate and longer operative time in HSFI patients, suggesting that the presence of abundant subcutaneous fat increases the difficulty of TEP repair.

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  • Jung In Lee, Tae-Gyu Ahn, Ji Hyun Choi
    2023 Volume 90 Issue 1 Pages 41-49
    Published: February 25, 2023
    Released on J-STAGE: March 11, 2023
    JOURNAL FREE ACCESS

    Background: Photodynamic therapy (PDT) -a minimally invasive anti-cancer therapy-is undergoing experimental studies to increase its anti-cancer effects. This study investigated the influence of iron on the anti-cancer effects of PDT. Methods: PDT was performed in a cancer-bearing mouse model, which was created by using a murine colon carcinoma (CT26) cell line after administration of Photolon and iron. Tumor volume and the results of TdT-mediated dUTP-biotin nick end labeling (TUNEL), 8-OHdG, and TBARS assays were used to measure anti-cancer effect. Results: On day 14, tumor volume had increased by 49% in the PDT group and decreased by 72% in the iron+PDT group. The percentage of TUNEL-positive cells in tumor tissues was 45% in the PDT group and 69% in the iron+PDT group, suggesting that the proportion of TUNEL-positive cells had increased in the iron+PDT group. The 8-OHdG content in tumor tissues was 33% higher in the iron+PDT group than in the PDT group. The TBARS content in tumor tissues was 46% higher in the iron+PDT group than in the PDT group. Conclusions: Iron enhances the anti-cancer effect of PDT using Photolon, most likely by increasing oxidative damage.

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  • Tomoko Suzuki, Masahiro Sakurai, Hirotaka Suzuki, Takae Kawamura
    2023 Volume 90 Issue 1 Pages 50-57
    Published: February 25, 2023
    Released on J-STAGE: March 11, 2023
    JOURNAL FREE ACCESS

    Background: Postoperative complications of thoracoabdominal aortic aneurysm include paraplegia due to impaired blood flow in the spinal cord. Sivelestat sodium hydrate (ONO-5046), a specific neutrophil elastase inhibitor, can prevent neuropathy after ischemia-reperfusion of the spinal cord; however, the underlying mechanism remains unclear. Here, we examined whether ONO-5046 elicits its protective effects in spinal cord ischemia by affecting endoplasmic reticulum (ER) stress. Methods: Forty-five male Japanese white rabbits (weight 2.5-3.0 kg) were assigned to three groups: a sham control group (n = 5), and two other groups (n = 20, respectively; n = 5 each time point) that were subjected to spinal cord ischemia-reperfusion for 15 min and administered saline or ONO-5046 intravenously. From 8 h to 7 d after resumption of blood flow, a neurological evaluation, histological evaluation of the spinal cord, and immunohistochemical evaluation based on the expression of GRP78 and caspase12 were performed. Results: Rabbits treated with ONO-5046 had fewer functional deficits and more surviving motor neurons after ischemia than did rabbits in the saline and control groups. In rabbits treated with ONO-5046, histological findings of the spinal cord showed a high number of viable motor nerves, whereas induction of GRP78, an ER stress response-related protein, was prolonged. Furthermore, caspase12 expression was activated by excessive ER stress and was downregulated in rabbits treated with ONO-5046, as compared with that in rabbits administered saline. Conclusions: ONO-5046 exerts a protective effect on the spinal cord by relieving ER stress during spinal cord ischemia.

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  • Shuang Qiu, Jie Liao, Xin Luo, Xiu Chen
    2023 Volume 90 Issue 1 Pages 58-63
    Published: February 25, 2023
    Released on J-STAGE: March 11, 2023
    JOURNAL FREE ACCESS

    Background: The prognostic value of the neutrophil-to-lymphocyte ratio (NLR), an inflammatory indicator, for 90-day outcomes has not been determined for patients with acute ischemic stroke aged ≥ 80 years. Therefore, this study examined the predictive significance of the NLR for estimating the 90-day outcomes of older patients with acute ischemic stroke. Methods: Data from patients aged ≥ 80 years were recorded within 7 days of ischemic stroke onset during the period from January 2019 to June 2021. A Kaplan-Meier curve was drawn based on the incidence of adverse outcomes to compare the survival probability of each group at different time periods. Cox multivariate regression was used to explore adverse events affecting patient prognosis. Results: A total of 560 patients were initially recruited; of these, 476 were eligible for inclusion. The log-rank test showed that the survival rates of the groups differed. The 90-day survival rate was significantly lower in the group with the highest NLR than in the group with the lowest NLR. Multivariate Cox regression analysis showed that after adjusting for risk factors, a high NLR and a high National Institutes of Health Stroke Scale score were independent risk factors for 90-day mortality. According to the receiver operating characteristic analysis, the area under the curve for predicting mortality at 90 days was 0.74. Conclusion: This study confirmed that a high NLR is an independent risk factor for acute ischemic stroke and has predictive value for 90-day prognosis in patients aged ≥ 80 years.

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  • Kentaro Maejima, Nobuhiko Taniai, Hiroshi Yoshida
    2023 Volume 90 Issue 1 Pages 64-68
    Published: February 25, 2023
    Released on J-STAGE: March 11, 2023
    Advance online publication: November 25, 2022
    JOURNAL FREE ACCESS

    Background: Leakage at the esophagojejunal anastomosis site is an important postoperative complication of total gastrectomy. We analyzed our surgical cases to determine the risk factors for esophagojejunal anastomotic leakage. Methods: This study included 309 patients who underwent total gastrectomy and esophagojejunal anastomosis. The onset of esophagojejunal anastomotic leakage according to age, gender, performance status, American Society of Anesthesiologists classification, body mass index, presence or absence of diabetes, invasion depth, lymph node metastasis, histological type, presence or absence of esophageal infiltration, operative duration, amount of blood loss, experience of blood transfusion, procedural approach, and the prognostic nutritional index was analyzed. Results: Univariate analyses revealed a significant difference in the rate of esophagojejunal anastomotic leakage due to advanced age, male gender, the presence of diabetes, the presence of esophageal infiltration, and blood loss ≥1,100 g. In the multivariate analysis, which included factors identified in the univariate analyses, advanced age, male gender, the presence of diabetes, and blood loss ≥1,100 g were identified as independent risk factors for esophagojejunal anastomotic leakage. Conclusions: Advanced age (≥68 years), male gender, diabetes, and massive blood loss are risk factors for esophagojejunal anastomotic leakage.

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  • Ryuta Watanabe, Yasuo Okumura, Koichi Nagashima, Yuji Wakamatsu, Akima ...
    2023 Volume 90 Issue 1 Pages 69-78
    Published: February 25, 2023
    Released on J-STAGE: March 11, 2023
    Advance online publication: November 25, 2022
    JOURNAL FREE ACCESS

    Background: Chronological changes in ablation lesions after cryoballoon ablation (CBA) and hot balloon ablation (HBA) of atrial fibrillation (AF) remain unclear. Methods: Of 90 patients who underwent initial balloon-based catheter ablation of AF and cardiac magnetic resonance imaging (cMRI) 3 months after ablation, data from 48 propensity score-matched patients (24 per group; 34 males; age 62±10 years) were analyzed. High-density pulmonary vein-left antrum (PV-LA) voltage mapping was performed after PV isolation, and low voltage areas around the PV ostia were defined as the total acute ablation lesion area (cm2). cMRI-derived dense fibrotic tissue localized around PVs was defined as the total chronic ablation lesion area (cm2). The percentage of total ablation lesion areas to total PV-LA surface area (%ablation lesion) was calculated during each phase, and %acute ablation lesion and %chronic ablation lesion areas were compared in patients who had undergone CBA and HBA. Results: The %acute ablation lesion area was larger for the CBA group than for the HBA group (30.8±5.8% vs. 23.0±5.5%, p < 0.001). There was no difference in %chronic cMRI-derived ablation lesion area (24.8±10.8% vs. 21.1±11.6%, p = 0.26) between groups. The rates of chronic AF recurrence were 12.5% and 8.3%, respectively (p = 0.45; log-rank test). LA volume and LA surface area were strongly associated with AF recurrence, but %chronic ablation lesion area was not (27±8% vs. 23±12%, p = 0.39). Conclusion: Large acute ablation lesions after CBA were smaller during the chronic phase. The size of chronic ablation lesions and the rate of AF recurrence were both similar for CBA and HBA.

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  • Shintaro Fujita, Masanobu Takeyama, Shingo Kato, Yusuke Kawabata, Yuta ...
    2023 Volume 90 Issue 1 Pages 79-88
    Published: February 25, 2023
    Released on J-STAGE: March 11, 2023
    Advance online publication: November 25, 2022
    JOURNAL FREE ACCESS
    Supplementary material

    Background: Active surveillance (AS) has been suggested for managing extra-abdominal desmoid fibromatosis (EADF), but a substantial percentage of such patients transitioned to invasive secondary treatments. The anti-keloid medication tranilast is frequently used in Japan but its effectiveness for EADF is not well understood. Methods: We retrospectively analyzed the medical records of EADF patients treated with tranilast between January 2009 and March 2021. EADF has been reported to shrink spontaneously, so the effects of all drugs must be compared with AS. To assess the effect of tranilast, we compared the clinical courses of patients receiving tranilast with those managed by AS (as identified in a systematic review). A systematic review of AS outcomes was conducted on July 22, 2021, in accordance with PRISMA guidelines. The primary endpoint was rate of conversion to secondary treatment. Secondary endpoints were progression-free survival, objective response rate (ORR), disease control rate (DCR), and adverse events. The rates of conversion to secondary treatment, ORRs, and DCRs were compared between the two groups by using the Fisher exact test. Results: Eighteen patients who received tranilast as initial treatment for EADF were included. Two patients (11.1%) underwent surgical resection for treatment of tumor growth and persistent pain. The rate of conversion to secondary treatment was significantly lower for tranilast than for a pure AS approach (40.1%; p = 0.01). ORR and DCR did not differ between groups. Conclusions: Tranilast was better than AS for initial management of EADF.

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  • Mingming Fang, Chen Zhong
    2023 Volume 90 Issue 1 Pages 89-95
    Published: February 25, 2023
    Released on J-STAGE: March 11, 2023
    JOURNAL FREE ACCESS

    Background: Oxidative stress is an important mechanism in liver ischemia/reperfusion (I/R) injury. Hepatocyte apoptosis and proliferation occur in parallel with liver I/R injury, and the degree of apoptosis and proliferation determines the effects on hepatocytes. Vitamin D receptor (VDR) can lessen liver I/R injury, but previous studies focused mostly on inflammation and immunity. Methods: H2O2 was used to induce hepatocyte injury. Before treatment with H2O2, Hep-3B cells were pretreated with paricalcitol (PC) and siRNA-VDR. Rapamycin and chloroquine were also applied in the study. Results: The number of apoptotic cells was measured with an annexin V (AV) -fluorescein isothiocyanate apoptosis detection kit. Expression of proteins was measured by western blotting. As compared with the H2O2+Hep-3B group, levels of AV/PI, cleaved caspase-3, and p62 were lower, and expression levels of Bcl-2, proliferating cell nuclear antigen, and VDR were higher, in the PC+H2O2+Hep-3B group. When the VDR gene was silenced by siRNA-VDR in the siRNA-VDR+H2O2+Hep-3B group, expressions of AV/PI, cleaved caspase-3, and p62 were upregulated, and expressions of Bcl-2, proliferating cell nuclear antigen, and VDR were downregulated, as compared with values for the siRNA-NC+H2O2+Hep-3B group. Treatment with rapamycin or chloroquine partially reversed the effect of PC and siRNA-VDR on apoptosis and proliferation. Conclusions: VDR mediates hepatocyte apoptosis and proliferation through autophagy.

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  • Shun Miyazaki, Saeko Ozaki, Susumu Ichiyama, Michiko Ito, Toshihiko Ho ...
    2023 Volume 90 Issue 1 Pages 96-102
    Published: February 25, 2023
    Released on J-STAGE: March 11, 2023
    JOURNAL FREE ACCESS

    Background: We previously evaluated blood screening data, including antinuclear antibodies (ANA), before initiating biologic treatment for patients with psoriasis in a real-world setting. However, we did not analyze change in ANA titers after the start of biologics. No previous study has comprehensively investigated change in ANA titers over time in individual patients or the effectiveness of certolizumab pegol or tildrakizumab. Objectives: This study evaluated change in ANA titers in individual patients during treatment with biologics, including certolizumab pegol and tildrakizumab. Methods: 111 patients were included in this study. Change in ANA was regarded as significant when the ANA titer was ×80 or more in patients with a previously undetectable ANA titer or when it increased by fourfold or more in those with a detectable ANA titer before treatment. Results: The ratios of patients with a significant change in ANA titer who were treated with a tumor necrosis factor (TNF) inhibitor, interleukin (IL) -17 inhibitor, or IL-23 inhibitor were 34.9% (15/43), 0.0% (0/32), and 0.0% (0/36), respectively. There were 4 patterns of significant change in ANA titer: (i) an increase (n=8), (ii) a decrease after an increase (n=4), (iii) a decrease after an increase with a drug change (n=2), and (iv) an increase after a decrease after an increase (n=1). No symptom suggesting lupus syndrome was noted. Conclusions: ANA titers must be carefully monitored throughout treatment with biologics, especially TNF inhibitors, and the possibility of lupus-like syndrome should be excluded.

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  • Toru Yoshioka, Nobukazu Okimoto, Mikiya Sawa, Kei Asano, Kenji Obayash ...
    2023 Volume 90 Issue 1 Pages 103-110
    Published: February 25, 2023
    Released on J-STAGE: March 11, 2023
    JOURNAL FREE ACCESS

    Background: In vivo three-dimensional (3D) motion under weight-bearing conditions was analyzed postoperatively in medial pivot cruciate-substituting (CS) knee systems with fixed and mobile inserts. Methods: Tibiofemoral knee kinematics during squatting were captured with X-ray fluoroscopy for 4 patients in each cohort. The 3D motion of implants was analyzed with KneeMotion motion analysis software (LEXI Corporation; Tokyo, Japan). In addition, anterior-posterior (AP) movement of the distal-most points and the angle of axial rotation of the femoral component on the tibial component were assessed in both cohorts. Results: Mean AP movement of the femoral component on the tibial component was 3.8±0.5 mm on the medial side and 9.5±0.5 mm on the lateral side in the cohort with fixed prostheses and 5.9±2.1 mm on the medial side and 10.0±2.5 mm on the lateral side in the cohort with mobile prostheses. The mean angle of axial rotation of the femoral component on the tibial component was 14.4±1.1 degrees and 8.2±2.7 degrees of external rotation for fixed knees and mobile knees, respectively. Conclusions: Postoperative motion analysis confirmed that fixed and mobile CS implants, which have a similar design, guided medial pivot motion under weight-bearing conditions. However, motion differed between these implant types after mid-flexion: bicondylar rollback after medial pivot motion was noted in the mobile cohort.

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  • Shu Tanaka, Jun Omori, Aitoshi Hoshimoto, Takayoshi Nishimoto, Naohiko ...
    2023 Volume 90 Issue 1 Pages 111-120
    Published: February 25, 2023
    Released on J-STAGE: March 11, 2023
    JOURNAL FREE ACCESS

    Background: Linked color imaging (LCI) improves detection of colorectal neoplastic lesions during colonoscopy. However, polyps <5 mm in diameter often do not require resection, and the benefits of LCI are unclear for detection of colorectal polyps ≥5 mm that are indicated for endoscopic resection in clinical practice. This randomized controlled trial compared rates of detection of adenoma polyps, stratified by size, for LCI and white light imaging (WLI). Methods: We compared ADR (5 mm-) and PDR (5 mm-), which were defined as the proportion of patients with at least one adenoma or polyp with a diameter of 5 mm or larger in the LCI and WLI groups. Moreover, we estimated ADR and PDR for diameters between 5 and 10 mm (ADR (5-9 mm), PDR (5-9 mm) ) and for diameters larger than 10 mm (ADR (10 mm-), PDR (10 mm-) ). Results: Data from 594 patients (LCI, n=305; WLI, n=289) were analyzed. ADR (5 mm-) and PDR (5 mm-) were significantly higher in the LCI group than in the WLI group (ADR (5 mm-): P=0.016, PDR (5 mm-): P=0.020). In the assessment of adenoma and polyp size, ADR (5-9 mm) and PDR (5-9 mm) were significantly higher in the LCI group than in the WLI group, although no significant differences were seen in ADR (10 mm-) and PDR (10 mm-) between these groups. Conclusions: Polyps ≥5 mm, which are indicated for endoscopic treatment, were more easily visualized with LCI mode than with WLI mode. The improvement in detection rate was obvious for polyps <10 mm, which are easier to miss.

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Case Reports
  • Hiroyuki Dan, Kyongsong Kim, Rinko Kokubo, Ryutaro Nomura, Daijiro Mor ...
    2023 Volume 90 Issue 1 Pages 121-125
    Published: February 25, 2023
    Released on J-STAGE: March 11, 2023
    JOURNAL FREE ACCESS

    Purpose: Pleomorphic adenomas tend to arise in the salivary glands. They are rare and histologically benign but can result in distant metastasis, and their characteristics need further investigation. We report a case of locally recurring benign primary palatal pleomorphic adenoma that resulted in spinal metastases and review the relevant literature. Case Report: A 58-year-old woman had undergone surgery for a palatal pleomorphic adenoma 22 years earlier and 6 subsequent operations for local recurrences. During follow-up, metastases to multiple organs, including the spine, were diagnosed and 4 CyberKnife treatments were performed. She suffered right flank pain and slight paralysis of the right leg; radiological findings showed a growing metastatic spinal tumor. She underwent removal of a thoracic vertebral tumor and posterolateral fusion. Postoperatively, her symptoms improved. Histopathological analysis indicated a pleomorphic adenoma and no evidence of malignancy. Although there was no local recurrence, 23 months after surgery, a fifth CyberKnife procedure was performed for a growing salivary gland tumor and she is currently being followed up. Conclusion: We described a rare case of benign pleomorphic adenoma that metastasized to the spine. Long-term follow-up for recurrence and metastasis is required for patients with benign pleomorphic adenoma.

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  • Masahiro Sakaguchi, Yasunobu Nagata, Yasuhiro Terasaki, Atsushi Takeyo ...
    2023 Volume 90 Issue 1 Pages 126-135
    Published: February 25, 2023
    Released on J-STAGE: March 11, 2023
    Advance online publication: November 25, 2022
    JOURNAL FREE ACCESS
    Supplementary material

    Hemophagocytic lymphohistiocytosis (HLH) involves pathological histiocytes and phagocytosis of normal blood cells through activation of inflammatory cytokines. We report a case of Epstein-Barr virus-HLH in a 75-year-old woman who presented with fever, thrombocytopenia, and loss of consciousness. Epstein-Barr virus-HLH was diagnosed after we identified massive hemophagocytosis in bone marrow and Epstein-Barr virus DNA in cerebrospinal fluid. The HLH-2004 protocol was applied, and lactate dehydrogenase levels-which reflect HLH disease status-decreased. However, persistent loss of consciousness and multiple organ failure led to the patient's death on day 18. Most cases of primary and secondary HLH involve pediatric patients; adult cases are rare. Few cases of central nervous system involvement in older adults have been reported. Therefore, accumulation of more data will help in developing better treatment strategies.

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