Goblet cell carcinoid of the appendix: Six case reports.

BACKGROUND: Goblet cell carcinoid (GCC) of the appendix is a rare tumor characterized by neuroendocrine and adenocarcinoma features. Accurate preoperative diagnosis is very difficult, with most patients complaining mainly of abdominal pain. Computed tomography shows swelling of the appendix, so diagnosis is usually made incidentally after appendectomy based on a preoperative diagnosis of appendicitis. Even if a patient undergoes preoperative colonoscopy, accurate endoscopic diagnosis is very difficult because GCC shows a submucosal growth pattern with invasion of the appendiceal wall. CASE SUMMARY: Between 2017 and 2022, 6 patients with GCC were treated in our hospital. The presenting complaint for 5 of these 6 patients was abdominal pain. All 5 patients underwent appendectomy, including 4 for a preoperative diagnosis of appendicitis and the other for diagnosis and treatment of an appendiceal tumor. The sixth patient presented with vomiting and underwent ileocecal resection for GCC diagnosed from preoperative biopsy. Although 2 patients with GCC underwent colonoscopy, no neoplastic changes were identified. Two of the six patients showed lymph node metastasis on pathological examination. As of the last follow-up (median: 15 mo), all cases remained alive without recurrence. CONCLUSION: As preoperative diagnosis of GCC is difficult, this possibility must be considered during surgical treatments for presumptive appendicitis.

A Case Report of Neoadjuvant Gemcitabine Plus Cisplatin for Locally Advanced Unresectable Ampulla of Vater Carcinoma.

INTRODUCTION: Ampulla of Vater carcinoma (AVC) with para-aortic node (PAN) metastasis is considered unresectable and is equivalent to distant metastasis, contributing to poor outcomes. CASE PRESENTATION: A 60-year-old man was referred to our hospital and was diagnosed with an unresectable ampulla of Vater carcinoma that had metastasized to the para-aortic nodes. The patient received a systemic chemotherapy regimen comprising a combination of gemcitabine and cisplatin. Following five cycles of treatment, imaging studies revealed a significant reduction in the primary tumor and para-aortic node metastasis, rendering detection difficult. Pancreatoduodenectomy with para-aortic node dissection was performed as a radical surgery. Upon pathological examination, no residual tumors were identified in the resected specimen, indicating that the systemic chemotherapy achieved a complete pathological response. The postoperative course of the patient was uneventful, and he was discharged on the 25th postoperative day. The patient was followed up as an outpatient and remained stable without any recurrence for two months after surgery. CONCLUSION: Neoadjuvant chemotherapy with gemcitabine and cisplatin was useful for downstaging the ampulla in patients with Vater carcinoma. This finding may help physicians manage patients with similar presentations.

A Case of Gallbladder Cancer with Trousseau Syndrome Successfully Treated Using Radical Resection.

Trousseau syndrome is characterized by cancer-associated systemic thrombosis. We describe the first case of a successfully treated gallbladder adenocarcinoma accompanied by Trousseau syndrome. A 66-year-old woman presented with right hemiplegia. Magnetic resonance imaging identified multiple cerebral infarctions. Her serum carbohydrate antigen 19-9 and D-dimer levels were markedly elevated, and a gallbladder tumor was detected via abdominal computed tomography. Venous ultrasonography of the lower limbs revealed a deep venous thrombus in the right peroneal vein. These findings suggested that the brain infarctions were likely caused by Trousseau syndrome associated with her gallbladder cancer. Radical resection of the gallbladder tumor was performed. The resected gallbladder was filled with mucus and was pathologically diagnosed as an adenocarcinoma. Her postoperative course was uneventful, and she received a one-year course of adjuvant therapy with oral S-1. No cancer recurrence or thrombosis was noted 26 months postoperatively. Despite concurrent Trousseau syndrome, a radical cure of the primary tumor and thrombosis could be achieved with the appropriate treatment.

Can right ventricular endomyocardial biopsy predict left ventricular fibrosis beforehand in dilated cardiomyopathy?

AIMS: Myocardial fibrosis of the left ventricle (LV) is a prognostic factor in dilated cardiomyopathy (DCM). This study aims to evaluate whether fibrosis of right ventricular (RV) endomyocardial biopsy (EMB) can predict the degree of LV fibrosis beforehand in DCM. METHODS AND RESULTS: Fibrosis extent in 70 RV-EMB specimens of DCM diagnosis was compared with that in the whole cross-sectional LV of excised hearts in the same patients (52 explanted hearts for transplant and 18 autopsied hearts). The median interval between biopsy and excision was 4.1 (0.13-19.3) years. The fibrosis area ratio of the EMBs and excised hearts were evaluated via image analysis. The distribution of cardiovascular magnetic resonance-late gadolinium enhancement (LGE) in the intraventricular septum was classified into four quartile categories. The fibrosis area ratio in RV-EMB correlated significantly with that in the short-axis cut of the LV of excised hearts (r = 0.82, P < 0.0001) and with a diffuse pattern of LGE (r = 0.71, P = 0.003). In a multivariate model, after adjusting for the interval between biopsy performance and heart excision, the fibrosis area ratio in RV-EMB was associated with that in LV-excised heart (regression coefficient, 0.82; 95% confidence interval, 0.68-0.95; P < 0.0001). CONCLUSIONS: The fibrosis observed in RV-EMB positively correlated with the extent of fibrosis in the LV of excised hearts in patients with DCM. The study findings may help predict LV fibrosis, considered a prognostic factor of DCM through relatively accessible biopsy techniques.

18F-FDG PET/CT in left atrial undifferentiated pleomorphic sarcoma with osteosarcomatous differentiation.

Primary cardiac sarcomas are rare and sometimes difficult to discern from benign tumors and intracardiac thrombi. We describe the ultrasound, computed tomography (CT), magnetic resonance imaging (MRI), and 18F-fluorodeoxyglucose (18F-FDG) positron emission tomography (PET)/CT findings in a case of left atrial undifferentiated pleomorphic sarcoma with osteosarcomatous differentiation, presenting with severe mitral regurgitation and pulmonary hypertension. The tumor presented as a broad-base mass protruding into the cardiac lumen, accompanied by punctate calcification-like high attenuation on CT. 18F-FDG PET/CT revealed high 18F-FDG uptake in the mass. Severe mitral regurgitation, a rare manifestation, was caused by tumor extension to the mitral valve leaflets and subvalvular tissue, which was best visualized on transesophageal echocardiography. This case illustrates the importance of multimodal diagnostic approaches including 18F-FDG PET/CT, which can facilitate accurate diagnosis and timely initiation of curative treatment, ultimately saving the patient's life. Learning objective: Firstly, cardiac sarcomas, particularly those with calcification/ossification, are rare and may mimic benign tumors and chronic intracardiac thrombi. Multimodal imaging approach, including 18F-FDG PET/CT, may be helpful in the accurate diagnosis of malignancies. Second, left atrial undifferentiated pleomorphic sarcoma has the potential to extensively spread along the endocardium and can extend to involve the valve leaflets, resulting in mitral regurgitation and pulmonary hypertension.

The Clinical Features of Late Postoperative Cholangitis After Hepaticojejunostomy Brought on by Conditions other than Cancer Recurrence.

PURPOSE: Postoperative cholangitis and anastomotic strictures (AS) are long-term complications of biliary-enteric anastomosis (BEA). METHODS: We retrospectively reviewed data of patients who underwent bile duct resection with or without hepatectomy and investigated the risk factors for postoperative cholangitis, benign AS, and incidence of Clavien-Dindo (C-D) >Grade III complications. RESULTS: Overall, data of 189 patients (115 men and 74 women) were retrospectively analyzed. The median patient age was 73 years. Thirty-five patients (18.5%) developed postoperative cholangitis, and 16 (8.4%) developed postoperative AS. Male sex and serious postoperative complications (C-D ≥ Grade III) were independent risk factors for cholangitis. The incidence of serious postoperative complications was 32.3%. Hypertension, preoperative biliary drainage, C-reactive protein-albumin ratio ≥.22, and bile duct resection with hepatectomy were potential risk factors for serious postoperative complications. CONCLUSIONS: The incidence rates of postoperative cholangitis and AS after BEA were 18.5% and 8.4%, respectively. Male sex and serious postoperative complications (C-D ≥ Grade III) were independent risk factors for postoperative cholangitis.

Favorable Outcome of Repeated Salvage Surgeries for Rare Metastasis to the Ligamentum Teres Hepatis and the Upper Abdominal Wall in a Stage IV Gastric Cancer Patient.

Gastric cancer with peritoneal metastases is typically a devastating diagnosis. Ligamentum teres hepatis (LTH) metastasis is an extremely rare presentation with only four known cases. Herein, we report salvage surgery of successive metastases to the abdominal wall and LTH in a patient originally presenting with advanced gastric cancer with peritoneal metastasis, leading to long-term survival. A 72-year-old man with advanced gastric cancer underwent curative-intent distal gastrectomy with D2 lymph node dissection for gastric outlet obstruction. During this procedure, three small peritoneal metastases were detected in the lesser omentum, the small mesentery, and the mesocolon; however, intraoperative abdominal lavage cytology was negative. We added cytoreductive surgery for peritoneal metastasis. The pathological diagnosis of the gastric cancer was tubular adenocarcinoma with pT4aN1pM1(PER/P1b)CY0 stage IV (Japanese classification of gastric carcinoma/JCGC 15th), or T4N1M1b stage IV (UICC 7th). Post-operative adjuvant chemotherapy with S-1 (TS-1)+cisplatin (CDDP) was administered for 8 months followed by S-1 monotherapy for 4 months. At 28 months after the initial surgery, a follow-up computed tomography (CT) detected a small mass beneath the upper abdominal wall. The ass showed mild avidity on 18F-fluorodeoxyglucose positron-emission (FDG-PET) CT. Salvage resection was performed for diagnosis and treatment, and pathological findings were consistent with primary gastric cancer metastasis. At 49 months after the initial gastrectomy, a new lesion was detected in the LTH with a similar level of avidity on FDG-PET CT as the abdominal wall metastatic lesion. We performed a second salvage surgery for the LTH tumor, which also showed pathology of gastric cancer metastasis. There has been no recurrence up to 1 year after the LTH surgery. With multidisciplinary treatment the patient has survived almost 5 years after the initial gastrectomy. Curative-intent gastrectomy with cytoreductive surgery followed by adjuvant chemotherapy for advanced gastric cancer with localized peritoneal metastasis might have had a survival benefit in our patient. Successive salvage surgeries for oligometastatic lesions in the abdominal wall and the LTH also yielded favorable outcomes.

Case Report: Postmortem brain and heart pathology unveiling the pathogenesis of coexisting acute ischemic stroke and electrocardiographic abnormality.

Electrocardiography abnormalities have been occasionally reported at the onset of stroke. Simultaneous electrocardiographic abnormalities and stroke require a rapid differentiated diagnosis among several diseases. However, direct causal relationships remain unclear. A 92-year-old woman presented to our emergency department in a sudden-onset coma. The patient suffered from huge acute ischemic stroke with bilateral internal carotid artery occlusion assessed by brain magnetic resonance imaging, and her electrocardiography showed ST-segment elevation at II, III, aVF and V4-6, and atrial fibrillation (AF). However, the etiology of the medical condition was clinically unknown. Eventually, the patient died on day 4 of hospitalization before the diagnosis could be completed. Therefore, an autopsy was performed to investigate pathological findings after obtaining informed consent from the family. A postmortem pathological evaluation demonstrated that fibrin mural thrombi in the left atrial appendage (LAA), and the cerebral and coronary arteries possessed CD31-positive endothelial cells, and CD68-positive and CD168-positive macrophages in a similar fashion, suggesting the fibrin thrombi observed in the three sites implicated to be identical. We concluded that nearly concurrent cerebral and coronary artery embolism because of the fibrin thrombi in LAA developed by AF. Simultaneous cerebral infarction and myocardial infarction are referred to as cardiocerebral infarction (CCI), a rare disorder for which clear pathomechanisms remain unknown, although several mechanisms of CCI have been proposed. We first revealed the clear pathology of CCI using the autopsy. Additional pathological studies are warranted to establish clear pathomechanisms and preventive strategies of CCI.

Two cases of unresectable hepatocellular carcinoma treated via atezolizumab and bevacizumab combination therapy.

BACKGROUND: Treatment of hepatocellular carcinoma (HCC) varies widely depending on the patient's condition. In recent years, combination therapy with immune checkpoint inhibitors has emerged as the treatment of choice due to its superior antitumor effects for unresectable HCC (uHCC). Conversion surgery (CS) after systemic chemotherapy is expected to be an effective treatment strategy for uHCC. Here, we report two cases of uHCC with bilateral porta hepatis invasion, in which atezolizumab plus bevacizumab therapy regressed the tumor invasion of the porta hepatis, followed by CS with R0 resection. CASE PRESENTATION: The first patient-a 71-year-old man with S4 HCC-developed porta hepatis, and the tumor compressed the right portal vein and bile duct. R0 resection with left trihepatectomy was impossible because of insufficient liver function, and combination therapy using atezolizumab and bevacizumab was initiated. After ten courses of treatment, the tumor shrunk with regression of the porta hepatis contact, and segmentectomy of S4 was performed with a sufficient surgical margin. Histopathological findings showed that the primary tumor was mostly necrotic with no residual viable tumor cells. The second patient was a 72-year-old man with an S4 HCC extending to the porta hepatis. The patient's condition was almost similar to that in the first case and required left tri-segmentectomy with R0 resection; however, insufficient liver function made liver resection impossible. An atezolizumab plus bevacizumab regimen was administered, and after seven courses of treatment, porta hepatis compression regressed, following which left lobectomy was performed with adequate surgical margins. The pathological diagnosis was moderately differentiated HCC, most of which was necrotic, and R0 resection was confirmed. CONCLUSIONS: Atezolizumab plus bevacizumab therapy has the potential to facilitate radical resection in patients with uHCC.

Morphological characteristics of cardiac myxoma causing embolism: a series of 40 years of experience at a single institute.

Systemic and cerebral embolisms are serious complications of associated with cardiac myxoma. Embolism risk reportedly depends on the gross and histological morphology. This study is aimed at analyzing the morphologic pattern of excised cardiac myxoma as a high-risk embolic cause. Between 1978 and April 2022, 116 surgical specimens of cardiac myxomas were recorded at the pathology department of our hospital. The tumors were classified into three types based on their macroscopic shapes and external morphology-round-smooth, irregular, and villous-to investigate the embolic complications. Of the 116 specimens, 106 macroscopic images of cardiac myxoma (89% were located in the left atrium) were prepared. Round-smooth types were found in 36 (34.0%) patients, irregular types in 32 (30.2%) patients, and the villous types in 38 (35.8%) patients. Multivariable analysis revealed that a villous external appearance was an independent predictor of embolic events (odds ratio: 8.7; 95% confidence interval: 2.4-42.1; p < 0.001). Villous external appearance of cardiac myxoma was associated with the highest risk of distal embolism.

Early vascular responses to abluminal biodegradable polymer-coated versus circumferential durable polymer-coated newer-generation drug-eluting stents in humans: a pathological study.

BACKGROUND: Recent clinical studies are testing strategies for short (1-3 months) dual antiplatelet therapy following newer-generation drug-eluting stent (DES) placement. However, detailed biological responses to newer-generation DES remain unknown in humans. AIMS: We sought to evaluate early pathologic responses to abluminal biodegradable polymer-coated (BP-) DES compared with circumferential durable polymer-coated (DP-) DES in human autopsy cases. METHODS: The study included 38 coronary lesions with newer-generation DES implanted for <90 days (DP-DES=24, BP-DES=14) in 26 autopsy cases. The degree of strut coverage was defined as follows: grade 0 (bare), grade 1 (with fibrin or tissues/cells without endothelium), grade 2 (with single-layered endothelium), and grade 3 (with endothelium and underlying smooth muscle cell layers). RESULTS:  The duration following implantation was similar in DP- and BP-DES (median=20 vs 17 days). A total of 2,022 struts (DP-DES=1,297, BP-DES=725) were pathologically analysed. Focal grade 2 coverage was observed as early as 5 days after the implantation in both stents. The multilevel mixed-effects ordered logistic regression model demonstrated that BP-DES exhibited greater strut coverage compared with DP-DES (odds ratio [OR]: 3.64, 95% confidence interval [CI]: 1.37-9.67; p=0.009), which remained significant after adjustment for the duration following implantation and underlying tissue characteristics (OR: 2.74, 95% CI: 1.10-6.80; p=0.030). The predictive probability of grade 2 and 3 coverage was comparably limited at 30 days (DP-DES=17.1%, BP-DES=28.7%) and increased at 90 days (DP-DES=76.5%, BP-DES=86.6%). Both stents showed low inflammation and a similar degree of fibrin deposition. CONCLUSIONS: Single-layered endothelial coverage begins in the days after newer-generation DES placement, and BP-DES potentially exhibit faster strut coverage with smooth muscle cell infiltration than DP-DES in humans. Nevertheless, vessel healing remains suboptimal in both stents at 30 days.

First case report of neoadjuvant gemcitabine and S-1 for locally advanced unresectable duodenal adenocarcinoma.

BACKGROUND: The usefulness of neoadjuvant chemotherapy for patients with duodenal adenocarcinoma remains unclear. We report the case of a successfully resected duodenal adenocarcinoma managed by neoadjuvant chemotherapy using gemcitabine and S-1. CASE PRESENTATION: A 72-year-old female presented with a one-week history of abdominal bloating and vomiting after meals. Esophagogastroduodenoscopy revealed a circumferential epithelial lesion in the second portion of the duodenum. Abdominal computed tomography scan revealed thickened walls and narrowing of the duodenum. Further, an adenocarcinoma was noted on biopsy. Though she was diagnosed with duodenal adenocarcinoma, pancreatic cancer could not be completely ruled out. Therefore, she underwent neoadjuvant chemotherapy using gemcitabine and S-1 after bypass surgery. After six chemotherapy cycles, the tumor significantly reduced in size. Further, lymph nodes and distant metastases were not noted on abdominal computed tomography. The patient underwent pancreaticoduodenectomy. Pathological examination revealed a 0.5-mm lesion and surrounding fibrosis at the duodenum, distal from the ampulla of Vater and the pancreas. Her postoperative course was almost uneventful, and she was discharged on the 31st postoperative day. The patient was followed up and had no tumor recurrence at 24 months after surgery. CONCLUSION: Neoadjuvant chemotherapy with gemcitabine and S-1 was useful in reducing the size of a duodenal adenocarcinoma. This finding would aid physicians in managing patients that present with a similar presentation.

Left Ventricular Dysfunction Caused by IgG4-related Small Intramural Coronary Periarteritis.

IgG4-related disease (IgG4-RD) is a systemic autoimmune disorder known to affect multiple organs. However, IgG4-RD rarely affects the myocardium. We herein report a case of left ventricular dysfunction due to cardiac involvement of IgG4-RD.

Laparoscopic Synchronous Resection for Descending Colon Cancer and Tailgut Cyst.

A 67-year-old woman underwent polypectomy for a tumor at the descending colon. Pathologically, the tumor was diagnosed as adenocarcinoma with an invasion of 2000 µm. Computed tomography showed a swollen paracolic lymph node and a mass lesion in the presacral space. Magnetic resonance imaging revealed a multio-cular cystic lesion. On diagnosis of descending colon cancer and tailgut cyst, she underwent synchronous lapa-roscopic resection. Histopathologically, the colon cancer was diagnosed as pT1bN1M0, pStage IIIa. The pre-sacral cystic lesion was diagnosed as a nonmalignant tailgut cyst with negative surgical margin. The patient is currently doing well without recurrence at 28 months.

[Successful Resection Using a Posterior Approach of a Tailgut Cyst in the Retrorectal Space].

We present the case of a Tailgut cyst occurring in the retrorectal space that was curatively resected using a posterior approach. A 40-year-old man presented to the Kochi Health Sciences Center with the chief complaint of perineal incongruity. Pelvic magnetic resonance imaging revealed a multilocular cystic lesion in the retrorectal space, with high signal intensity on T2-weighted imaging. After diagnosing a Tailgut cyst, we performed resection of the tumor using a posterior approach. The lesion was removed en bloc with the coccyx. Histopathologically, the lesion was diagnosed as a non-malignant Tailgut cyst, and the surgical margin was negative. The patient is currently doing well without recurrence at 20 months.

Reversible left ventricular dysfunction due to severe stenosis of the elephant trunk graft: A case report.

Acquired coarctation of the aorta (CoA) following total aortic arch replacement (TAR) is a rare complication inducing left ventricular (LV) dysfunction probably due to increased LV afterload and secondary hypertension caused by increased upper body and decreased renal blood flow. We describe a case of a 35-year-old male who developed atypical CoA with severe LV dysfunction with LV ejection fraction of 10%, but without secondary hypertension after TAR using conventional elephant trunk (ET) technique for acute aortic dissection. Computed tomography revealed near-occlusive CoA due to narrowed distal ET. Because the myocardial histological findings were mild, and he had no cardiac failure history, we determined that LV function might be reversible. He underwent thoracic endovascular aortic repair (TEVAR), resulting in restored LV function. However, as the descending aortic false lumen distally to the end of ET was rapidly dilated, probably due to increased cardiac output and lower body blood flow, he underwent descending aortic replacement 3 months after TEVAR. In conclusion, a narrowed distal ET may cause LV dysfunction early after TAR, even without secondary hypertension. TEVAR may be a useful therapeutic option for a narrowed distant ET but can induce distal aortic dilatation. .