A Case of Myocardial Infarction with Non-obstructive Coronary Arteries Complicated by a Ventricular Septal Rupture: The Challenge of Achieving Hemodynamic Stability Using Impella Devices.

The prognosis of myocardial infarction with non-obstructive coronary arteries (MINOCA) is reported to be as worse as myocardial infarction with obstructive coronary arteries. However, its mechanical complications are still poorly understood. A 71-year-old woman developed MINOCA after ascending aortic replacement surgery. During treatment, the patient experienced cardiogenic shock due to a ventricular septal rupture (VSR). The introduction of Impella devices reduced the left-to-right shunt and improved the patient's hemodynamics. Finally, a scheduled surgical repair was performed under stable conditions. In this report, we focused on the pathophysiology of MINOCA-related VSR and discussed the effectiveness of Impella devices as a bridge to surgical repair and circulatory backup during the perioperative period.

Erratum to "Ependymoma of the broad ligament mimicking an ovarian surface epithelial tumor" [Radiology Case Reports 16 (2021) 210-214].

[This corrects the article DOI: 10.1016/j.radcr.2020.10.046.].

Total synthesis and antimicrobial evaluation of (+)-hygrophorone B12 and its analogues.

This paper describes the synthesis and evaluation of lead compounds with a new chemical skeleton that is not found in conventional antimicrobial agents. The biologically attractive cyclopentenoid (+)-hygrophorone B12, isolated from the fruiting bodies of Hygrophorus abieticola, and its analogues were synthesized in a longer linear sequence of twelve steps, starting from a cyclopentenone derivative. This synthesis involved the following crucial steps: (i) oximation of a ketone to stabilize the requisite aldehyde to install a side chain and (ii) coupling of an aldehyde with a side chain to assemble the desired hygrophorone. Then, the antimicrobial activity of these hygrophorones towards clinically relevant bacterial pathogens was evaluated. The results showed that hygrophorone B12 and its analogues are especially effective in preventing the proliferation of gram-positive bacteria. In addition, it was found that some structural features such as the presence of the enone moiety as well as the carbon-carbon triple bond on the hydrocarbon chain were pivotal to increase the antimicrobial activity of hygrophorone B. This study is expected to support the development of novel antimicrobial agents by flexibly synthesizing hygrophorone B analogues with a carbon five-membered ring skeleton from the common intermediate.

Hemoptysis after coil embolization for pulmonary arteriovenous malformation: Histopathological confirmation of bronchial epithelium extension.

Coil embolization is widely performed for pulmonary arteriovenous malformations (PAVMs). We describe herein 2 cases of hemoptysis during long-term follow-up after coil embolization for PAVMs. For both cases, lobectomy was performed and histopathological examinations revealed chronic inflammation and bronchial epithelium extension into the sac of the PAVM. In addition, we performed a systematic review of previous reports of hemoptysis after embolization for PAVMs.

Anticoagulant Use as an Independent Risk Factor and Higher In-Hospital Mortality in Patients Showing Alveolar Hemorrhage in Diffuse Lung Disease.

Background and objectives: Bronchoalveolar lavage (BAL) is commonly performed to evaluate diffuse lung disease and occasionally to identify alveolar hemorrhage. However, the clinical impact of alveolar hemorrhage and its risk factors in patients with diffuse lung disease have not been clarified. Materials and Methods: We retrospectively analyzed the medical records of all patients who underwent BAL to evaluate diffuse lung disease from January 2017 to December 2020. Alveolar hemorrhage was defined as progressive hemorrhagic BAL fluid or the presence of ≥20% hemosiderin-laden macrophages in the BAL fluid. Logistic regression analysis was performed to assess the association between alveolar hemorrhage and other factors. Results: Sixty subjects were enrolled in this study. Alveolar hemorrhage was observed in 19 subjects (31.7%) with idiopathic interstitial pneumonia, acute respiratory distress syndrome, interstitial pneumonia with autoimmune features, drug-induced lung injury, eosinophilic pneumonia, adenocarcinoma, and systemic lupus erythematosus. The use of anticoagulants was a significant risk factor for alveolar hemorrhage (odds ratio 7.57, p = 0.049). Patients with alveolar hemorrhage required intubated mechanical ventilation more frequently (63.2% vs. 24.4%, p = 0.005) and had higher in-hospital mortality rates (26.3% vs. 4.9%, p = 0.028) than those without alveolar hemorrhage. Conclusions: Alveolar hemorrhage was observed in various etiologies. The use of anticoagulants was a significant risk factor for alveolar hemorrhage. Patients with alveolar hemorrhage showed more severe respiratory failure and had higher in-hospital mortality than those without alveolar hemorrhage.

Examination on the necessity of pericardial fat tissue resection in extended thymectomy for myasthenia gravis.

BACKGROUND: Extended thymectomy with pericardial fat tissue resection has been a mainstay in the treatment for myasthenia gravies (MG), but few studies have examined the necessity of the pericardial fat tissue resection in extended thymectomy. METHODS: We pathologically examined the distribution of germinal centers in the resected thymus including the thymus-surrounding fat tissue. Patients who underwent extended thymectomy using subxiphoid thoracoscopy for generalized MG or thymoma with anti-acetylcholine receptor antibody (anti-AchR Ab) positivity from March 2015 to January 2021 were included in this study. RESULTS: A total of 20 patients underwent extended thymectomy (generalized nonthymomatous MG, n=5; generalized MG with thymoma, n=6; thymoma with anti-AchR Ab positivity, n=9). The resected specimens were divided to 6 parts and were pathologically examined to investigate the distribution of the geminal centers in all lesions. The number and distribution of germinal centers in the thymus varied depending on the patient, and no germinal centers were identified in the right or left pericardial fat tissues. CONCLUSIONS: It is necessary to resect the pericardial fat tissue in patients whose preoperative images show the presence of pericardial fat tissue hyperplasia or ectopic thymoma. However, extensive pericardial fat tissue resection might not be necessary for all MG patients who undergo extended thymectomy with opening of the bilateral pleural by video-assisted or robot-assisted thoracoscopic surgery (RATS) via a subxiphoid approach.

[Cystic fibrosis with focal biliary cirrhosis and portal hypertension in Japan: a case report].

A 9-year-old Japanese girl was found to have persistently elevated hepatic enzymes, chronic bronchitis, chronic sinusitis, and poor weight gain beginning at 5 months of age. Chest computed tomography (CT) revealed diffuse bronchial wall thickening and peripheral bronchiectasis. Abdominal CT showed pancreatic atrophy, liver cirrhosis, a dilated splenic vein, and splenomegaly. Her sweat chloride concentration was 117mmol/l (normal, <60mmol/l). CFTR gene analysis revealed the presence of the Y517H variant on one allele and the 1540del10 variant one the other allele. These findings established a definitive diagnosis of cystic fibrosis (CF). While CF is the most common autosomal recessive genetic disorder among Europeans, it is quite rare in Southeast Asia including Japan. It is important that CF be considered in the work-up of children with chronic hepatic and respiratory disorders even if it is uncommon among children of a similar background.

Myelolipoma mimicking osteosarcoma in the distal femur.

INTRODUCTION AND IMPORTANCE: Periosteal reactions indicate malignant bone tumors, including osteosarcoma; establishing an accurate diagnosis is key to determining the most appropriate treatment strategy. We describe a rare case of myelolipoma in the distal femur metaphysis with massive extraskeletal lesions and periosteal reactions. CASE PRESENTATION: A 25-year-old woman was referred to our hospital to treat a gradually expanding mass around her knee that grew to the size of a baby's head. She had a history of hydrocephalus caused by congenital cytomegalovirus infection and was bedridden for life. Radiography showed a prominent osteoblastic rim and osteolytic lesion with a moth-eaten appearance. Osteosarcoma was suspected due to excessive extraskeletal invasion and periosteal reactions. T1- and T2-weighted magnetic resonance images showed a high-signal-intensity homologous lesion. Biopsy specimens contained adipose and hematopoietic tissues. A myelolipoma was diagnosed. Due to her fragility, surgical intervention was suspended. Two years after diagnosis, the tumor size did not change. CLINICAL DISCUSSION: Myelolipomas are benign tumors that typically arise from the adrenal gland and rarely develop in the extremities. This type of tumor typically does not cause any tumor-related symptoms or endocrine disturbances and has been reported as a type of incidentaloma. To effectively manage myelolipoma patients, differential diagnosis of tumors mimicking malignant bone tumors is important. CONCLUSION: We successfully managed a destructive ectopic myelolipoma in the distal femoral metaphysis, with massive extraskeletal lesions and periosteal reactions. Clinicians should appropriately differentiate myelolipoma from tumors mimicking malignant bone tumors.

Type A thymoma with simultaneous solitary intrapulmonary metastasis: A case report.

A 79-year-old woman was referred to our facility because of an abnormal chest shadow. Chest computed tomography (CT) showed a solitary right middle lung nodule with a maximum diameter of 3 mm and anterior mediastinal nodule with a maximum diameter of 21 mm. The lung nodule was suspected of being a primary lung cancer rather than a metastatic tumor because there were no primary malignant tumors, apart from an anterior mediastinal tumor visible on diagnostic imaging, including F18 fluorodeoxyglucose-positron emission tomography, and a solitary lung nodule. Partial lung resection by video-assisted thoracoscopic surgery (VATS) was performed, and the intraoperative frozen section of the tumor tissue resulted in a diagnosis of carcinoid tumor. As a result, right middle lobectomy by VATS was performed. The final histological diagnosis of the permanent specimen was intrapulmonary type A thymoma. VATS thymectomy was performed three months later. The histological diagnosis was type A thymoma with intrapulmonary metastasis (Masaoka stage IVb). Additional therapy was not performed because complete resection was achieved. Follow-up CT was performed once every six months after the operation. The patient has been followed up for one year without any further recurrence.

Ependymoma of the broad ligament mimicking an ovarian surface epithelial tumor.

We report a case of ependymoma of the broad ligament occurring in a 21-year-old woman. CT and MRI findings showed a 40-mm-diameter, well-demarcated cystic mass with a lobulated solid component in the right pelvis. The solid component showed heterogeneous intermediate signal intensity on T2-weighted image and prolonged mild contrast enhancement. The tumor was resected and confirmed as ependymoma based on the histologic findings along with its immunohistochemical profile. To our knowledge, this is the first report of an adnexal ependymoma describing the precise radiological characteristics that resembled those of borderline or malignant epithelial ovarian tumors.

A Case of Alveolar Haemorrhage Induced by Iodinated Contrast Medium Use.

An 81-year-old man complaining of exertional dyspnoea underwent coronary angiography using an iodinated contrast medium. After angiography, the patient required systemic corticosteroid therapy because of respiratory failure due to alveolar haemorrhage. Percutaneous coronary intervention was performed 29 days after angiography using the same contrast medium. After the intervention, the patient required intubated mechanical ventilation and renal replacement therapy. Bronchoalveolar lavage was bloody with many haemosiderin-filled macrophages. Systemic corticosteroid therapy again improved his clinical condition. Iodinated contrast media may cause alveolar haemorrhage and re-exposure to contrast media may induce a more severe adverse reaction. LEARNING POINTS: Iodinated contrast media may cause alveolar haemorrhage.Re-exposure to iodinated contrast media may induce a more severe adverse reaction.

Potential complications in patients undergoing an ethanol injection into the vein of Marshall.

BACKGROUND: Ethanol injections into the vein of Marshall (VOM) (EIM) are considered to be a good therapeutic option for atrial tachyarrhythmias, however, the safety remains to be determined. To elucidate what would affect the safety and potential complications of an EIM, we investigated the anatomical features of the VOM and patient background. METHODS: We performed the EIM before the conventional pulmonary vein isolation for drug-resistant atrial fibrillation in 88 patients and evaluated the anatomical features of the VOM and their background. RESULTS: All procedures were completed, however, other than myocardial staining, trivial contrast medium leaked out of the VOM into the pericardial space, that is, extravasation of contrast medium with capillary rupture, during the EIM in 20 patients (22.7%) regardless of the features of the VOM. No pericardial effusions requiring further intervention developed after the extravasation, which resolved by the next day on echocardiography in 18 of those patients. However, two patients who had extravasation other than during the initial contrast injection required additional therapeutic intervention for nonnegligible pericardial effusions. Their body weights were significantly lower and the latter two patients were also small lean women with heart failure and a preserved ejection fraction. CONCLUSIONS: The physical constitution, regardless of the characteristics of the VOM, could be strongly associated with adverse events during the EIM. We must take extreme care in smaller patients with poor compliant hearts during the EIM.

Testicular teratoma demanded in-depth pathological exploration to rule out malignancy: A pediatric case report.

INTRODUCTION: Prepubertal testicular tumors can be both benign and malignant. Although most testicular teratomas are benign, some immature cases include malignant transformation or the mixed type with yolk sac tumor and, occasionally, it is challenging to rule out malignancy. CASE PRESENTATION: We present a case of immature testicular teratoma in a 7-month-old infant, whose alpha-fetoprotein level was sequentially elevated following orchidectomy. Since malignancy could not be ruled out, we performed whole body imaging and in-depth pathological exploration. GLYPICAN3, OCT3/4, and SOX2 staining revealed no evidence of malignancy. The patient was finally diagnosed with benign immature teratoma, and has been free from recurrence for 3 years. CONCLUSION: Here, we describe the case report, as well as all the comprehensive diagnostic tests that we performed in order to rule out the malignant component.

The inflammatory pseudotumor presenting periodic acid-Schiff-positive inclusions with acute unilateral facial nerve palsy.

Although most acute peripheral facial palsies can be attributed to Bell's palsy, other factors, such as infection, trauma, and neoplasm, can cause facial palsy as well. Among these, facial nerve tumors are rare but should be considered in the differential diagnosis of facial palsy. Palsies due to facial nerve tumors usually present with slow onset but occasionally present as acute episodes. In such cases, facial nerve decompression is the treatment of choice to allow the tumor room to grow without compressing the nerve or its blood supply. We describe a case of severe, acute facial palsy presenting with a spindle-shaped bone erosion on the mastoid portion of the facial canal. Although facial neuroma was suspected preoperatively, emergency decompression surgery revealed that an unusual inflammatory pseudotumor was responsible for the finding. Postoperative histological analysis revealed extensive destruction of the nerve fibers, with extensive infiltration of foamy macrophages containing characteristic, diastase-resistant, periodic acid-Schiff (PAS)-positive inclusions, which are hallmark of the uncommon bacterial infections. This was a case of facial palsy with an unusual etiology. The case shows the benefit of decompression surgery not only as treatment for the palsy but also as exploratory surgery in cases of facial nerve tumor.

Sinonasal inverted papilloma associated with small cell neuroendocrine carcinoma: A case report and literature review of rare malignancies associated with inverted papilloma.

We report a rare case of sinonasal inverted papilloma (IP) associated with small cell neuroendocrine carcinoma (SNEC). To our knowledge, this is the first report to describe SNEC found during the treatment of sinonasal IP. Surgery and five cycles of cisplatin plus etoposide with concurrent intensity modulated radiation therapy were performed. Neither local recurrence nor distant metastasis was noted during 6 years of post-diagnostic follow-up. The prognosis of SNEC is very poor. Treatment planning for sinonasal IP should consider a possible association with this rare but aggressive malignancy, whose treatment is completely different from that of squamous cell carcinoma, a malignancy which is commonly associated with IP. We also performed a PubMed review of the literature to identify the incidence and pathological diagnosis of associated malignancy. Among a total of 5286 cases of sinonasal IP (61 studies), the incidence of associated malignancy was 8.02% in squamous cell carcinoma, 0.19% in transitional cell carcinoma, 0.04% in mucoepidermoid carcinoma, 0.02% in verrucous cell carcinoma and 0.02% in adenocarcinoma. The incidence of associated malignancy was significantly higher in East and Southeast Asia (11.0%) and North America (10.4%) than in Europe (3.9%) (p=0.04 and p=0.03, respectively; T-test).

Blastomatoid pulmonary carcinosarcoma: A rare case report and review of the literature.

A 65-year-old never-smoking woman presented to a local hospital, because an abnormal shadow was detected at the right lower lung field by annual chest X-ray. Computed tomography (CT) revealed a 5-cm tumor in segment 6 of her right lung and an enlarged subcarinal lymph node, suggesting metastasis. The lung tumor was diagnosed as adenocarcinoma by a CT-guided percutaneous needle biopsy. She was referred to our hospital and underwent right lower lobectomy with lymph node dissection (ND2a-2). A histopathological examination of the tumor showed a biphasic proliferation made of carcinomatous and sarcomatous components. The carcinomatous component consisted of glandular structures of atypical cells that possessed chromatin-rich nuclear and clear cytoplasm, confirming high-grade fetal adenocarcinoma. The sarcomatous component consisted of immature spindle cells that differentiated into chondrosarcoma. Immunohistochemically, the glandular structures expressed membranous beta-catenin, and the ultimate diagnosis was blastomatoid variant of pulmonary carcinosarcoma. She received four courses of cisplatin plus vinorelbine as adjuvant chemotherapy and remained alive with neither recurrence nor distant metastasis at two and a half years after the operation. We experienced a rare case of blastomatoid pulmonary carcinoasarcoma.

Ethanol injection into the Marshall vein provoking a pericardial effusion resulting in a fatal complication in a patient with persistent atrial fibrillation.

An EIM (ethanol infusion into the vein of Marshall [VOM]) provoked a fatal complication in a chronic hemodialysis patient. Autopsy revealed a lacerated VOM covered with thrombi as the only potential cause. The EIM caused vascular damage and clots resulting in myocardial necrosis and interstitial bleeding around the lacerated VOM.

[Pulmonary Carcinosarcoma Presenting Hemothorax Caused by Pleural Invasion;Report of a Case].

A 71-year-old man presented with hemothorax with cough, sputa and worsening dyspnea. On chest X-ray and computed tomography(CT), a huge tumor in the right upper lobe with hematoma and small amount of gas suggesting hemopneumothorax was revealed. No apparent lymphadenopathy nor intrapulmonary metastases were observed. The tumor showed a little enhancement on the contrastenhanced CT. Then the resction of the tumor was performed, and the pathological evaluation revealed a carcionosarcoma (adenocarcinoma+osteosarcoma) pT3N0 (stage II B) G4 pl2. Sarcomatoid carcinoma such as carcinosarcoma should be considered as a possible cause of hemothorax in making a diagnosis of hemorrhagic hypovascular huge lung tumor.

Mammary analogue secretory carcinoma of salivary glands: a clinicopathologic and molecular study including 2 cases harboring ETV6-X fusion.

Mammary analogue secretory carcinoma (MASC) is a recently described low-grade carcinoma with morphologic and genetic similarity, including ETV6-NTRK3 fusion, to secretory carcinoma of the breast. ETV6 is frequently involved in other epithelial and nonepithelial tumors, and many fusion partners of ETV6 have been reported. In the present study, 14 Japanese MASC cases were clinicopathologically and molecularly analyzed. The median age of the patients was 39 years, and the male:female ratio was 6:8. All cases showed histopathologic findings compatible with those previously described for MASC and harbored an ETV6 split as visualized by fluorescence in situ hybridization. Two cases showed thick fibrous septa and invasive features including vascular or perineural tumor involvement, findings that are rare in MASC. In addition, in these 2 cases, non-NTRK3 genes appeared to fuse with ETV6 (ETV6-X fusion). NTRK1 and NTRK2, both members of the NTRK family, were not involved. Of the 14 MASC cases, the ETV6-NTRK3 fusion transcript was positive in 6 cases, and the relative expression level of the ETV6-NTRK3 fusion transcript was variable, ranging from 1 to 5.8. Results of the present study of MASC suggest that (1) ETV6 occasionally fuses with unknown non-NTRK3 genes, (2) ETV6-X cases might have an invasive histology, (3) for molecular diagnosis of MASC, fluorescence in situ hybridization to detect ETV6 splits is the method of choice, and (4) the expression level of the ETV6-NTRK3 fusion transcript is considerably variable. These findings provide a novel insight into the oncogenesis, histopathology, diagnosis, treatment, and prognosis of this newly recognized carcinoma.

Thymic extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue: a gene methylation study.

Although rare, thymic mucosa-associated lymphoid tissue (MALT) lymphoma is considered to be a distinct clinicopathological entity. Using a methylation-specific polymerase chain reaction, we analyzed thymic MALT lymphomas (n = 18) for their methylation of the following seven tumor suppressor genes: DAPK1, p16(INK4A), p14(ARF), CDH1, RARB, TIMP3 and MGMT. Reactive lymph nodes (n = 16) were used as a control. Of the seven genes examined, thymic MALT lymphomas had an increased number of genes that were methylated (2.9 genes) as compared with reactive lymph nodes (0.63, p = 0.0003). In particular, thymic MALT lymphomas showed a frequent methylation of DAPK1, CDH1, TIMP3 and p14(ARF). In addition, gene methylation of p14(ARF) was associated with a larger tumor size, while that of the other three genes was not associated with any clinicopathological features examined. This study suggests that methylation of tumor suppressor genes may play an important role in thymic MALT lymphoma.