Adrenocortical carcinoma mimicking hepatocellular carcinoma: A case report.

INTRODUCTION AND IMPORTANCE: Adrenocortical carcinoma (ACC) is a relatively rare tumor arising in the adrenal cortex. Its imaging and histopathologic findings are not well known to be similar to those of hepatocellular carcinoma (HCC). We report here a case of ACC with hepatic resection in the preoperative diagnosis of HCC. CASE PRESENTATION: A 46-year-old woman was noted to have a tumor 45 mm in size in the segment 7 of the liver on CT during a medical checkup. The tumor had consistent imaging findings as HCC on Ultrasound, CT, and MRI examinations, and the result of the liver tumor biopsy was a diagnosis of intermediate differentiated HCC. We considered the tumor to be HCC and performed a posterior segmentectomy with combined resection of the right adrenal gland, which had adhesions suspected to direct invasion. The pathology of the resected specimen confirmed the diagnosis of ACC with direct invasion into the liver. CLINICAL DISCUSSION: ACC may show a contrast pattern similar to that of HCC on imaging, and histopathology may show atypical cells with eosinophilic sporulation, similar to that of HCC. Our case serves to alert physicians that ACC should be considered a differential diagnosis in patients with suspected HCC in the posterior segment. CONCLUSION: Tumors suspected of HCC in the dorsal posterior segment of the liver should be considered as possible ACC.

Trifecta versus Perimount Magna Ease aortic valves: Failure mechanisms.

BACKGROUND: There are increasing reports of early externally mounted pericardial Trifecta bioprosthesis failure. We compared the hemodynamic performance of Trifecta and Carpentier-Edwards Perimount Magna Ease valves to determine the failure mechanism. METHODS: We retrospectively included 270 consecutive patients (age: 73.4 ± 8.2 years; 57.5% male; mean follow-up: 48.0 ± 20.3 months) who underwent aortic valve replacement from 2014 to 2021 at a single center and compared the Trifecta (N = 137) and Carpentier-Edwards Perimount Magna Ease valve (N = 133) patients. RESULTS: The prosthetic valve major aortic regurgitation incidence was higher for the Trifecta than that for the Carpentier-Edwards Perimount Magna Ease valve (6.3% vs. 0%, P < 0.009). Among the Trifecta failures, 33% developed structural valve deterioration, but all requiring redo aortic valve replacement developed major prosthetic valve aortic regurgitation. Freedom at 5 years from redo aortic valve replacement due to structural valve deterioration was significantly lower for Trifecta (89.4% vs. 100%, P = 0.003). The reoperation hazards were determined for Trifecta (vs. Carpentier-Edwards Perimount Magna Ease): 11.6 (1.47-90.9; P = 0.02), prosthetic valve aortic regurgitation: 2.38 (1.70-3.32; P < 0.01), structural valve deterioration: 20.82 (4.08-106.2; P < 0.01), 5-year mean transprosthetic pressure gradient: 1.14 per 1-point increase (1.03-1.24; P = 0.007), and urgent surgery: 10.1 (2.59-39.0; P = 0.001). The Cox regression analysis identified that prosthetic valve aortic regurgitation solely contributed to redo aortic valve replacement (hazard ratio: 2.38; confidence intervals: 1.70-3.32). CONCLUSIONS: Significantly, more early failures occurred with the Trifecta valve than the Carpentier-Edwards Perimount Magna Ease valve but the Trifecta showed reasonable mean transprosthetic pressure gradient over time. Prosthetic valve aortic regurgitation and calcific structural valve deterioration synergistically contributed to Trifecta valve failure alternatively.

Detection of Endogenous DNA Double-strand Breaks in Oral Squamous Epithelial Lesions by P53-binding Protein 1.

BACKGROUND/AIM: P53-binding protein 1 (53BP1) is one of the DNA damage response (DDR) molecules. This study aimed to assess 53BP1 expression by immunofluorescence (IF) as a biomarker to differentiate between oral squamous epithelial lesions (OSELs). MATERIALS AND METHODS: We analyzed 129 archival oral biopsy samples, including 18 benign squamous lesions (BSLs), 37 low-grade dysplasias (LGDs), 22 high-grade dysplasias (HGDs), and 52 oral squamous cell carcinomas (OSCCs). 53BP1 and Ki-67 expressions were examined by double IF to assess the type of 53BP1 expression. RESULTS: We found that OSCC exhibited several 53BP1 nuclear foci, particularly high-DNA damage response (HDDR) and large focus (LF)-type, suggesting the presence of endogenous DNA double-strand breaks in the cancer genome, which could disrupt DDR and induce genomic injury. We also found a difference in 53BP1 expression between LGD and HGD, but not between BSL and LGD. Among the Ki-67-positive cells, HDDR- and LF-type expressions were higher in OSELs of higher grades. CONCLUSION: 53BP1 expression can be a valuable biomarker for OSELs to help estimate the grade of oral epithelial dysplasia.

Age-dependent effects on radiation-induced carcinogenesis in the rat thyroid.

Childhood radiation exposure is a known thyroid cancer risk factor. This study evaluated the effects of age on radiation-induced thyroid carcinogenesis in rats irradiated with 8 Gy X-rays. We analyzed cell proliferation, cell death, DNA damage response, and autophagy-related markers in 4-week-old (4W) and 7-month-old (7M) rats and the incidence of thyroid tumors in 4W, 4-month-old (4M), and 7M rats 18 months after irradiation. Cell death and DNA damage response were increased in 4W rats compared to those in controls at 1 month post-irradiation. More Ki-67-positive cells were observed in 4W rats at 12 months post-irradiation. Thyroid tumors were confirmed in 61.9% (13/21), 63.6% (7/11), and 33.3% (2/6) of irradiated 4W, 4M, and 7M rats, respectively, compared to 0%, 14.3% (1/7), and 16.7% (1/6) in the respective nonirradiated controls. There were 29, 9, and 2 tumors in irradiated 4W, 4M, and 7M rats, respectively. The expression of several autophagy components was downregulated in the area surrounding radiation-induced thyroid carcinomas in 4W and 7M rats. LC3 and p62 expression levels decreased in radiation-induced follicular carcinoma in 4W rats. Radiosensitive cells causing thyroid tumors may be more prevalent in young rats, and abrogation of autophagy may be associated with radiation-induced thyroid carcinogenesis.

Incidence Patterns of Sequential or Composite Lymphoma: A Population-Based Cancer Registry Study.

The development of multiple histologic types of lymphoma in a single patient has been sporadically reported as sequential or composite lymphoma. However, the incidence pattern of such patients has been rarely evaluated in a large population-based setting. We investigated the incidence of sequential or composite lymphoma based on 11,174 lymphoma records from a population-based cancer registry between 1985-2012 in Nagasaki Prefecture, Japan. We identified 99 lymphoma records were of 49 independent patients other than relapse. The prevalence of the sequential or composite lymphomas in a single patient was 0.44% (95% confidence interval [95% CI], 0.32-0.56%) without sex difference. Among the 49 patients, five (10.2%) were composite/discordant lymphoma. The most frequent "composite lymphoma" was a combination of diffuse large B-cell lymphomas (DLBCL) and adult T-cell leukemia (n = 3). A case of "discordant lymphoma" was a combination of follicular lymphoma on spleen and Waldenström macroglobulinemia on bone marrow. The rest of the patients (n = 44, 89.8% of all composite lymphoma) were "sequential lymphoma" with various combination of lymphoma subtypes on different dates. The major combination of the sequential lymphoma was DLBCL after marginal zone lymphomas (n = 4). In the era of improved survival of lymphoma patients, hematologists should be aware of the development of additional lymphomas.

A case of cystic duct syndrome.

In the diagnosis of gallbladder induced pain without gallstones, drip-infusion cholangiographic computed tomography, and endoscopic retrograde cholangiography are useful in differentiating cystic duct syndrome.

Cricopharyngeal myotomy for cricopharyngeus muscle dysfunction after esophagectomy.

BACKGROUND: Pharyngoesophageal dysphagia sometimes develops after esophagectomy. However, severe dysphagia after esophagectomy due to cricopharyngeus muscle dysfunction is a rare complication. There are no recommended clinical treatments for cricopharyngeus muscle dysfunction after esophagectomy. We report a case of myotomy for cricopharyngeus muscle dysfunction after esophagectomy. CASE PRESENTATION: A 75-year-old man with mild dysphagia diagnosed with advanced esophageal cancer by esophagogastroduodenoscopy at a clinic was admitted to our hospital. He had occasional mild dysphagia when he swallowed solid foods. After chemotherapy, the patient underwent minimally invasive esophagectomy with regional lymph node dissection and was reconstructed with a gastric conduit and cervical anastomosis by the retrosternal route. Aspiration pneumonia developed after esophagectomy without paralysis of the vocal cords. In esophagoscopy, there was no stricture around the anastomosis. However, severe pharyngoesophageal dysphagia with cricopharyngeus muscle dysfunction was revealed by videofluoroscopic examination. Bilateral cricopharyngeal myotomy was performed because balloon dilations had failed. The histological findings revealed atrophy and fibrosis of the cricopharyngeus muscle fibers. Pharyngoesophageal dysphagia improved immediately after myotomy. The patient swallowed solid food easily without dysphagia 12 months after myotomy. CONCLUSION: Dysphagia after esophagectomy was worsened by cricopharyngeus muscle dysfunction. Cricopharyngeus myotomy may lead to long improvement of pharyngo-oesophageal dysphagia after esophagetomy.

Nondegenerated cystic neuroendocrine tumor of the pancreas: a case report.

BACKGROUND: Pancreatic neuroendocrine tumors (PNETs) are typically solid neoplasms but, in very rare cases, present as cystic lesions. We describe a case of a cystic neuroendocrine tumor that developed as a small cystic lesion. CASE PRESENTATION: In 2011, a 66-year-old Japanese woman underwent computed tomography (CT) that revealed a cystic lesion in the tail of the pancreas measuring 9 mm. She did not have any symptoms. She underwent a CT scan every year thereafter. The cystic lesion gradually increased and was 40 mm in 2019; endoscopic retrograde pancreatography (ERP) was then performed. Cytological examination demonstrated class IIIb adenocarcinoma, and we conducted laparoscopic distal pancreatectomy. Pathological examination showed PNET. CONCLUSION: Although cystic change of PNET is generally caused by ischemia or necrosis inside the tumor, in our case, PNET occurred as a small cyst that increased without changing form.

[Falx Meningioma Presenting with Intratumoral Hemorrhage and Interhemispheric Acute Subdural Hematoma:A Case of Report].

A 68-year-old male with a sudden headache while defecation was transferred to our hospital. He was initially diagnosed with intracerebral hemorrhage in the right occipital lobe and acute subdural hematoma(ASDH)in the right interhemispheric fissure. A CT angiography(CTA)showed stenosis in the superior sagittal sinus(SSS)and the vein of Galen(VG)near the hematoma, which were considered to be due to compression of the hematoma. In the source image of CTA, the enhancement effect of the hematoma part was not clear. MRI revealed a heterogeneous mixed signal intensity in the hematoma area, suggesting a mixture of hematoma components that had bled at different times. Cerebral angiography performed two weeks after onset showed a tumor shadow imaged from the middle meningeal artery. Therefore, the presence of hemorrhagic meningioma was suspected. This was confirmed by contrast-enhanced MRI. One month after the onset, tumor resection was performed after the embolization of the feeding artery. Part of the tumor around the SSS and VG was left due to severe adhesion. Postoperatively, stenosis of the SSS and VG significantly improved. In this case, the increase in venous pressure may be related to the bleeding mechanism. Hemorrhagic onset meningioma with interhemispheric ASDH is extremely rare, and only 4 cases have been reported. It is easy to misdiagnose if only non-contrast CT is used. It should be noted that in cases of intratumoral hemorrhage, CTA may not show an enhancing effect in the acute phase. Since contrast-enhanced MRI may be useful for a definitive diagnosis, it should be performed at the time of initial imaging.

Protective effects of a cystine and theanine mixture against acute radiation injury in rats.

This study aims to examine the effects of cystine and theanine (CT), which increases glutathione biosynthesis, on the survival rate and acute radiation injury of the small intestine and bone marrow using a rat model. CT pre-treatment (280 mg/kg for 5 days) significantly improved weight loss and survival rate of rats as compared with the control group after 5 Gy. CT pre-treatment significantly increased the rate of mucosa and crypt length, and decreased the number of apoptotic cells, TUNEL and cleaved caspase-3 positive cells, while increasing the number of mitotic cells and Ki-67 positive cells in jejunal crypts and villi compared to control rats post-irradiation. CT also suppressed bone marrow cell loss and reduced the number of apoptotic cells in bone marrow. These results suggest a protective effect of CT pre-treatment for acute injury after irradiation through apoptosis inhibition and increased proliferative activity in jejunal crypt cells and bone marrow cells.

Clinical impact of pathology-proven etiology of severely stenotic aortic valves on mid-term outcomes in patients undergoing surgical aortic valve replacement.

BACKGROUND: The use of transcatheter or surgical aortic valve replacement (AVR) for severe aortic stenosis (AS) has considerably increased in recent years. However, the association between AS etiology and mid-term clinical outcomes after surgical AVR has not been fully investigated. METHODS AND RESULTS: We retrospectively included 201 patients (mean age, 75 years; 43%, men) who underwent surgical AVR for severe native AS (aortic valve area ≤1.0 cm2 on preoperative transthoracic echocardiography examination). The following valve etiologies were postoperatively identified on pathological examination: post-inflammatory (n = 28), congenital (n = 35), and calcific/degenerative (n = 138). The median follow-up interval was 4.1 years following surgical AVR. Of the 201 patients, 27% were asymptomatic, 40% had a history of heart failure, and 11% underwent previous heart surgery. The cumulative incidence of cardiac events (all-cause death, aortic valve deterioration requiring repeated AVR, and hospitalization for heart failure) and combined adverse events, which included non-fatal stroke, unplanned coronary revascularization, pacemaker implantation, and gastrointestinal bleeding along with cardiac events, was significantly higher in the calcific/degenerative group (p = 0.02 and p = 0.02, respectively). In multivariate analysis adjusted for age, sex, renal function, heart failure, atrial fibrillation, concomitant surgical procedures, and EuroSCORE II, AS etiology was independently associated with an increased risk of combined adverse events (congenital vs. post-inflammatory: hazard ratio [HR], 4.13; p = 0.02 and calcific/degenerative vs. post-inflammatory: HR, 5.69; p = 0.002). CONCLUSIONS: Pathology-proven AS etiology could aid in predicting the mid-term outcomes after surgical AVR, supporting the importance of accurate identification of severe AS etiology with or without postoperative pathological examination.

Cutaneous pilomatrical carcinosarcoma: a case report with molecular analysis and literature review.

BACKGROUND: Cutaneous pilomatrical carcinosarcoma (CS) is a very rare biphasic tumor composed of admixed epithelial and mesenchymal malignant cells, with limited information on its pathogenesis. We report a case of pilomatrical CS of the scalp with comparative immunohistochemical and molecular analysis together with a review of the literature. CASE PRESENTATION: A 74-year-old woman presented with a rapidly growing long-standing tumor of the scalp. The tumor was surgically resected. Histologically, the tumor was 25 mm in diameter, and was composed of carcinoma showing a clear pilomatrical differentiation and sarcoma with pleomorphic spindle cells and giant cells. Both epithelial and mesenchymal components shared focal cytoplasmic and/or nuclear accumulation of ß-catenin based on immunohistochemical analysis, although a mutation of exon 3 of the CTNNB1 gene was not detected. Fluorescence in situ hybridization analysis revealed gains of chromosomes 9p21, 3, and 7 in both the epithelial and sarcomatous components. CONCLUSIONS: The current case demonstrated characteristic findings of pilomatricoma and further evidence of partial clonality between the carcinomatous and sarcomatous component, suggesting the possibility of malignant transformation of pilomatricoma. Rapid growth of a pilomatrical tumor should warrant the development of a malignant tumor, including CS.

Development of hypocomplementemic urticarial vasculitis during certolizumab pegol treatment for rheumatoid arthritis: A case report.

WHAT IS KNOWN AND OBJECTIVE: Tumour necrosis factor-α-blocking agents potentially cause vasculitis. However, no study has reported on the association between hypocomplementemic urticarial vasculitis (HUV) and certolizumab pegol (CZP) usage. CASE DESCRIPTION: We present the first case of HUV development during CZP treatment for rheumatoid arthritis. Hypocomplementemic urticarial vasculitis improved after CZP was discontinued and the dose of oral prednisolone was increased. WHAT IS NEW AND CONCLUSION: Clinicians should be aware about the potential development of HUV during CZP treatment, which is presumed to be safe considering its unique structural characteristics that differ from those of other tumour necrosis factor-α-blocking agents.

Iron Deficiency Anemia-Induced Cardiomyopathy With Congestive Heart Failure: Reversible Cardiac Dysfunction Assessed by Multi-Imaging Modalities.

Iron deficiency anemia (IDA) can cause left ventricular (LV) dysfunction, causing heart failure. A 48-year-old woman with severe IDA developed congestive heart failure that was properly diagnosed, managed, and followed with multiple imaging modalities to explore potential mechanisms, highlighting the reversibility of LV function in unique cardiomyopathy. (Level of Difficulty: Intermediate.).

Significant association between 53 BP1 expression and grade of intraepithelial neoplasia of esophagus: Alteration during esophageal carcinogenesis.

BACKGROUND: Abnormal DNA damage response (DDR) leads to genomic instability and carcinogenesis. P53-binding protein 1 (53 BP1), a DDR molecule, is known to accumulate at the sites of DNA double-strand breaks. The aim of this study was to analyze the expression pattern of 53 BP1-nuclear foci (NF) in esophageal neoplasms in order to visualize the state of DDR in esophageal carcinogenesis and to clarify its significance in the molecular pathology of the disease. METHODS: A total of 61 lesions from 22 surgically resected samples of esophageal cancer, including histologically normal squamous epithelium, low-grade intraepithelial neoplasia (LG-IN), high-grade intraepithelial neoplasia (HG-IN), carcinoma in situ (CIS), and invasive squamous cell carcinoma (SCC), were included in the study. 53 BP1 and Ki-67 expression were analyzed by double-labeled immunofluorescence. RESULTS: The number of discrete 53 BP1-NF increased as the tumor progressed from normal epithelium through LG-IN, HG-IN, CIS, and SCC. 53 BP1-NF larger than 1 µm in diameter (large foci), indicating intensive DDR, also showed a stepwise increase during the progression of carcinogenesis. Of note, large foci of 53 BP1 were found in significantly higher numbers in HG-IN than in LG-IN. Furthermore, localization of 53 BP1-NF in Ki-67-positive cells, indicating the abnormal timing of DDR, also increased with malignancy progression. CONCLUSIONS: 53 BP1-NF accumulation increases during cancer progression from LG-IN to HG-IN to CIS to SCC. Detection of 53 BP1-NF by immunofluorescence, especially large foci, is a feasible method of estimating DNA instability and the malignant potential of esophageal intraepithelial neoplasia.

Two rare cases of congenital aortic stenosis showing a discrepancy between preoperative imaging diagnosis, intraoperative findings, and histopathological diagnosis.

Unicuspid aortic valve (UAV) is an extremely rare congenital heart valve abnormality while bicuspid valve (BAV) has been reported as one of the most common cardiac anomalies. With a UAV usually showing similar presentations to a BAV, such as aortic regurgitation or aortic stenosis (AS), it is challenging to differentiate them from each other in clinical settings. Despite some features shared between both valve disorders, there can be a clinical significance in distinguishing UAV from BAV for the management of patients with these heart anomalies. Herein, we describe two cases where patients with hemodynamically severe AS were diagnosed with BAV and UAV, respectively based on preoperative examinations and intraoperative findings, but subsequent pathological examinations confirmed the opposite diagnosis in both cases. .

Impact of preprocedural atrial fibrillation on immediate and long-term outcomes after successful percutaneous mitral valvuloplasty of significant mitral stenosis.

Optimal time to perform percutaneous mitral valvuloplasty (PMV) for patients with significant mitral stenosis (MS) and atrial fibrillation (AF) remains controversial. We sought to identify prognostic factors and evaluate long-term clinical outcomes after PMV of 77 consecutive patients with MS with a mitral valve area (MVA) <1.5 cm2. According to baseline heart rhythm, these patients were divided into sinus rhythm (SR; n = 24) and AF (n = 53) groups. The study endpoint was defined as a composite of all-cause mortality, admission for heart failure, mitral valve surgery, repeated PMV, and major cerebral vascular accident during follow-up. After successful PMV, there was no significant difference between the two groups in post-MVA and post-mitral mean pressure gradient. However, the New York Heart Association Functional Classification post-procedure was worse in the AF group (p < 0.01). In the AF group, event-free survival during follow-up was significantly lower compared with that of the SR group (p = 0.016). Independent predictors of clinical events were AF [hazard ratio (HR), 2.73; 95 % confidence interval (CI), 1.04-9.36; p = 0.03] and pulmonary artery systolic pressure (HR 2.57; 95 % CI 1.18-5.47; p = 0.017). Patients with AF at baseline were significantly associated with worse symptoms and higher event rates after successful PMV compared with those with SR. The clinical benefit of PMV may be considered for patients with MVA <1.5 cm2 before the onset of AF.

Expression of Somatostatin Receptor Type 2A and PTEN in Neuroendocrine Neoplasms Is Associated with Tumor Grade but Not with Site of Origin.

Neuroendocrine neoplasms (NENs) are derived from endocrine cells in various organs and share common morphological features. This study aimed to clarify whether NENs of different organs are comparable at the molecular pathologic level. We retrospectively collected 99 cases of NENs from gastro-entero-pancreatic, lung, and other organs and reclassified these according to identical criteria. Grade, site, and molecular expression profile including NE markers, Ki-67, p53, somatostatin receptor type 2A (SSTR2A), and phosphatase and tensin homolog (PTEN) were compared. PTEN immunoreactivity was also compared with genomic copy number by fluorescence in situ hybridization (FISH) and droplet digital polymerase chain reaction (ddPCR). No significant differences were observed in the immunoreactivities of NE markers, p53, SSTR2A, or PTEN expression in NENs between the different organ sites. PTEN and p53 functional inactivation along with the loss of membranous SSTR2A expression appeared to be commonly involved in high-grade NEN. FISH results were significantly correlated with the level of PTEN immunoreactivity and with the findings of ddPCR analyses. The demonstration that these tumors are comparable at the molecular level will likely contribute to the broadening of therapeutic options such as the use of somatostatin analogues and mTOR inhibitors against NENs regardless of the affected organ, whereas molecular characterization of tumor grade will be useful for determining treatment strategy.