Merkel cell polyomavirus and Langerhans cell neoplasm.

BACKGROUND: The relationship between various external agents such as pollen, food, and infectious agents and human sensitivity exists and is variable depending upon individual's health conditions. For example, we believe that the pathogenetic potential of the Merkel cell polyomavirus (MCPyV), the resident virus in skin, is variable and depends from the degree of individual's reactivity. MCPyV as well as Epstein-Barr virus, which are normally connected with humans under the form of subclinical infection, are thought to be involved at various degrees in several neoplastic and inflammatory diseases. In this review, we cover two types of Langerhans cell neoplasms, the Langerhans cell sarcoma (LCS) and Langerhans cell histiocytosis (LCH), represented as either neoplastic or inflammatory diseases caused by MCPyV. METHODS: We meta-analyzed both our previous analyses, composed of quantitative PCR for MCPyV-DNA, proteomics, immunohistochemistry which construct IL-17 endocrine model and interleukin-1 (IL-1) activation loop model, and other groups' data. RESULTS: We have shown that there were subgroups associated with the MCPyV as a causal agent in these two different neoplasms. Comparatively, LCS, distinct from the LCH, is a neoplastic lesion (or sarcoma) without presence of inflammatory granuloma frequently observed in the elderly. LCH is a proliferative disease of Langerhans-like abnormal cells which carry mutations of genes involved in the RAS/MAPK signaling pathway. We found that MCPyV may be involved in the development of LCH. CONCLUSION: We hypothesized that a subgroup of LCS developed according the same mechanism involved in Merkel cell carcinoma pathogenesis. We proposed LCH developed from an inflammatory process that was sustained due to gene mutations. We hypothesized that MCPyV infection triggered an IL-1 activation loop that lies beneath the pathogenesis of LCH and propose a new triple-factor model.

Changes in care managers' positive attitudes toward dying patients compared to that of nurses by one-day online advance care planning communication training.

Background: Culturally appropriate communication training programs for a wide range of professions that can be used during infection epidemics are crucial for advance care planning implementation. Starting in 2018, the Japanese Ministry of Health, Labour and Welfare made a major policy change, and doctors, nurses, and social workers, and care managers were identified in the guidelines as the professions that promote advance care planning. Motivated by the lack of online programs for Japanese care managers, we proposed a new one-day program. Objectives: This study aimed to determine the changes in the positive attitude of care managers toward dying patients compared to that of nurses, which has been used in past literature as an outcome of advance care planning educational interventions, after administering the program in Japan. Design: Before-after comparison study. Methods: Care managers were recruited through our website, ACP-Piece, http://plaza.umin.ac.jp/~acp-piece/piece.html. A questionnaire survey concerning positive attitudes toward dying patients was administered before and after the program on 28 August 2021. Sixty-six subjects participated in the training and 60 participants, including 14 care managers, consented to the study and completed the questionnaire surveys before and after the program. Results: The Frommelt attitude toward care of the dying scores for care managers increased after the program (p-values, confidence intervals, and effect sizes: p < 0.001, -11.90 to -4.388, -1.252). After training, care managers had a significantly higher maximum score occurrence than nurses. Older care managers with advance care planning experience may have had a higher maximum score occurrence compared to younger, inexperienced participants. Conclusion: To our knowledge, this is the first to demonstrate the increased positive attitude scores toward dying patients after online communication training for Japanese care managers. The limitations of this study include the lack of evidence regarding reasons for score changes, long-term score changes, and effectiveness for patients and their families.

Concordance of the histological diagnosis of type 1 autoimmune pancreatitis and its distinction from pancreatic ductal adenocarcinoma with endoscopic ultrasound-guided fine needle biopsy specimens: an interobserver agreement study.

The histological diagnosis of type 1 autoimmune pancreatitis (AIP) based on the findings obtained by an endoscopic ultrasound-guided fine needle biopsy (EUS-FNB) is feasible, but the diagnostic consistency of this method has not been confirmed. We determined the interobserver agreement among 20 pathologists regarding the diagnosis of type 1 AIP, including the distinction from pancreatic ductal adenocarcinoma (PDAC) using large tissue samples obtained by EUS-FNB. After guidance for diagnosing AIP with biopsy tissues was provided, a round 2 was performed. The median sensitivity and specificity for diagnosing PDAC vs. non-neoplastic diseases were 95.2% and 100%, respectively. In groups of specialists (n = 7) and the generalists (n = 13), Fleiss' к-values increased from 0.886 to 0.958 and from 0.750 to 0.816 in round 2. The concordance was fair or moderate for obliterative phlebitis and storiform fibrosis but slight for ductal lesion of type 1 AIP. Discordant results were due to ambiguous findings and biopsy tissue limitations. Among the specialists, the ratio of cases with perfect agreement regarding the presence of storiform fibrosis increased in round 2, but agreement regarding obliterative phlebitis or ductal lesions was not improved. Although the histological definite diagnosis of type 1 AIP was achieved by most observers in > 60% of the cases, the confidence levels varied. Because some ambiguities exist, the histological diagnostic levels based on the diagnostic criteria of type 1 AIP should not be taken for granted. Guidance is effective for improving accurate PDAC diagnoses (notably by recognizing acinar-ductal metaplasia) and for evaluating storiform fibrosis.

Laryngeal malignant peripheral nerve sheath tumor mixed with high- and low-grade malignancies.

Malignant peripheral nerve sheath tumors (MPNSTs), as defined by immunohistochemical evaluation, are identified along a spectrum ranging from atypical neurofibroma to high-grade MPNST because these tumors are similar in terms of cell shape and tissue components on hematoxylin-eosin (HE) staining. The patient was a 57-year-old male referred to our hospital, with a recurrent red tumor at the anterior commissure of the larynx and submucosal swelling of the right vocal fold. A surgical specimen from a right horizontal partial laryngectomy was evaluated immunohistochemically. A high-grade MPNST lesion was included in the submucosal white tumor, whereas a low-grade MPNST lesion was encountered around the high-grade MPNST lesion. This tumor may involve different malignancies even when it is small. Although intra-tumor heterogeneity in cancers has been reported recently, careful immunohistochemical examination can be important and beneficial for eradicating the tumor while preserving vocal function.

Tenosynovial and Cardiac Transthyretin Amyloidosis in Japanese Patients Undergoing Carpal Tunnel Release.

Background: Wild-type transthyretin cardiac amyloidosis (ATTRwt-CA) is a life-threatening progressive disease. Recent studies have shown that the detection of transthyretin (TTR) amyloid in tenosynovial tissue may play an important role in the diagnosis of cardiac amyloidosis. The aim of this study was to determine the prevalence of TTR amyloid deposits in surgical tissue of patients undergoing carpal tunnel surgery and to clarify the clinical significance of concomitant cardiac examination with 99 mTc-labeled pyrophosphate (99 mTc-PYP) scintigraphy in those patients with TTR deposition. Methods and Results: We evaluated 79 consecutive patients undergoing carpal tunnel release surgery and biopsy of tenosynovial tissue. The mean (±SD) age of the patients at surgery was 71.6±12.5 years (range 30-95 years); 32 patients (41%) were male. TTR amyloid deposition in tenosynovial tissue was observed in 27 patients (34%). Sixteen of those 27 patients underwent 99 mTc-PYP scintigraphy. Of those 16 patients, 3 (19%) had Grade 2 uptake on 99 mTc-PYP scintigraphy. None of the 3 patients with a diagnosis of ATTRwt-CA had apparent cardiac symptoms and left ventricular wall thickness >13 mm. Conclusions: Concomitant cardiac examination with 99 mTc-PYP scintigraphy in patients who had TTR amyloid deposition in tenosynovial tissue resulted in the identification of 19% of patients with a diagnosis of ATTRwt-CA. This diagnostic approach seems to be useful for the early diagnosis of the disease.

Thoracic endovascular aortic repair of an aberrant arterial aneurysm with pulmonary sequestration.

We describe a treatment strategy for an aberrant arterial aneurysm associated with pulmonary sequestration. A 58-year-old man with impending aberrant arterial aneurysm rupture underwent a 2-stage surgery that included an emergency thoracic endovascular aortic repair (TEVAR) of the descending aorta to occlude the origin of the aberrant artery, followed by lobectomy. TEVAR can lead to faster occlusion of the aneurysm and can avoid operative risk of aneurysm rupture during lobectomy. The aberrant artery was broad where it branched off the aorta and had a short neck, rendering primary ligation or stump-forming unsuitable. Pathological findings revealed the fragility of the aberrant artery; thus, its root was prone to breakdown of the stump after simple aneurysmectomy. Furthermore, TEVAR may reduce graft infection during lobectomy in the second surgery. The 2-stage surgery may be useful for aberrant aneurysms complicated by pulmonary sequestration.

Sigmoid colon perforation in the patient with granulomatosis with polyangiitis.

BACKGROUND: Granulomatosis with polyangiitis (GPA) induces respiratory tract and kidney granulomatous inflammation due to small-vessel vasculitis. However, gastrointestinal involvement, and especially colon perforation, is rare. CASE PRESENTATION: A 40-year-old man diagnosed with GPA was admitted to our hospital for GPA management. He was started on anti-cluster of differentiation 20 antibody (rituximab) therapy after admission and suffered severe abdominal pain 2 weeks later. A clinical diagnosis of sigmoid colon perforation was made, and we performed sigmoid colon resection with colostomy. Histopathological examination revealed loss of vascular wall and neutrophil infiltration. He was discharged from the hospital after intravenous immune globulin therapy. CONCLUSIONS: Although gastrointestinal involvement is rare in GPA, severe complications require surgical intervention. Bowel perforation should be considered an important complication of GPA.

Three-dimensional histologic reconstruction of remnant functional accessory atrioventricular myocardial connections in a case of Wolff-Parkinson-White syndrome.

Myocardial bundles working as accessory pathways in Wolff-Parkinson-White (WPW) syndrome are generally tiny tissues, so elucidating the culprit histology of atrioventricular (AV) myocardial connections requires careful serial sectioning of the AV junction. We performed a postmortem examination of accessory AV myocardial connections in an 84-year-old man who died from pneumonia 20 years after surgical cryoablation for WPW syndrome. Three-dimensional reconstruction images of serial histologic sections revealed accessory AV connections between the atrial and ventricular myocardium in the vicinity of the cryoablation scar. The remnant myocardial bridge was 4 mm wide and made up of multiple discontinuous fibers. This case was informative in that it provided for visualization of the histologic morphology of a remnant bundle of Kent.

Multiple coronary and cerebral aneurysms in a patient with chronic thromboangiitis.

A 73-year-old man had multiple coronary aneurysms that resulted in acute myocardial infarction on the day before surgery for cerebral aneurysms. Emergent coronary angiography revealed that the lesion that caused the myocardial infarction was a distal left circumflex artery, and two huge coronary aneurysms were also found in the left circumflex artery. A two-stage treatment strategy was planned, including coronary aneurysm surgery, followed by cerebral aneurysm surgery. He underwent coronary artery aneurysmorrhaphy with closure of the ostia of the afferent and efferent arteries, and coronary artery bypass grafting with a saphenous vein graft applied to the left circumflex artery. The pathological findings suggested chronic thromboangiitis, as the inflammatory cells were observed to have infiltrated the coronary artery wall. The tissue remodeling of the aneurysmal wall indicated a positive response to tenascin C. We report a case of multiple coronary aneurysms, focusing on the pathological findings. .

Mitral Regurgitation and Heart Failure as the First Presentation in a Patient with Features of Two Connective Tissue Disorders: A Rare Combination of Mucopolysaccharidosis and Osteogenesis Imperfecta?

Connective tissue disorders sometimes involve cardiovascular systems. This report describes the case of a middle-aged man with mitral regurgitation and heart failure. He had distinctive features of mucopolysaccharidosis type (MPS) III, but no gene mutations that were known to be associated with MPS. Meanwhile, he had a COL1A2 gene mutation that is associated with osteogenesis imperfecta (OI), and had some features that were compatible with OI. The patient might have had a rare connective tissue disorder with the characteristics of MPS III and OI, which was initially detected as a result of the cardiovascular manifestations.