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Immunoglobulin G4-related disease (IgG4-RD) is a systemic fibroinflammatory disease characterised by multiorgan lymphoplasmacytic infiltration, obliterative phlebitis and storiform fibrosis. It can be associated with cardiovascular pathology. The objective of this narrative review is to summarise the published literature on cardiovascular manifestations of IgG4-RD and to provide a basis for diagnosis and management of the condition by the practising cardiologist.We propose the following categorisations of cardiovascular IgG4-RD: aortitis, medium-vessel arteritis, pulmonary vascular disease, phlebitis, valvulopathy, pericarditis, myocardial disease and antineutrophilic cytoplasmic antibody-associated vasculitis. We also review herein developments in radiological diagnosis and reported medical and surgical therapies. Cardiovascular lesions frequently require procedural and/or surgical interventions, such as aortic aneurysm repair and valve replacement. IgG4-RD of the cardiovascular system results in serious complications that can be missed if not evaluated aggressively. These are likely underdiagnosed, as clinical presentations frequently mimic cardiovascular disease due to more common aetiologies (myocardial infarction, abdominal aortic aneurysm and so on). While systemic corticosteroids are the mainstay of IgG4-RD treatment, biological and disease-modifying agents are becoming more widely used. Cardiologists should be aware of cardiovascular IgG4-RD as a differential diagnosis, and understand the roles of corticosteroids, disease-modifying agents and biologicals, as well as their integration with surgical approaches. There are several knowledge gaps, including diagnosis, risk factors, pathogenesis and appropriate management in Ig4-RD of the cardiovascular system. Areas lacking well-conducted randomized trials include safety of steroids in the setting of vascular aneurysms and the role of disease-modifying drugs and biological agents in patients with established cardiovascular complications of this multifaceted enigmatic disease.

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BACKGROUND An epithelial inclusion cyst within a lymph node denotes a heterotopic phenomenon. Nodal epithelial inclusion cysts have been reported in a variety of anatomical locations including pelvic, abdominal, mediastinal, and axillary regions. While nodal melanocytic nevus (also known as nevus cell aggregates) is the most common heterotopic phenomena involving the axillary lymph nodes, the presence of benign epithelial inclusion cysts in axillary lymph nodes is a rare but well-reported finding. Such documentation is in part due to assessment of sentinel lymph nodes in breast cancer becoming standard of care. These epithelial inclusion cysts offer a diagnostic pitfall in evaluation of sentinel lymph node in the setting of breast carcinoma. They also complicate assessment of sentinel lymph node during intraoperative frozen sections analysis. CASE REPORT We report a case of co-existent of benign squamous-type and glandular-type epithelial inclusions cysts in 2 sentinel lymph nodes in a patient with grade III invasive ductal carcinoma involving the left breast. There have been at least 4 cases reported in literature in which benign epithelial inclusion cysts in sentinel lymph nodes were first mistakenly diagnosed as metastatic carcinoma both during intraoperative frozen section analysis and during review of permanent sections. The missed diagnosis could potentially occur intraoperatively during frozen section sentinel lymph node analysis secondarily due to lack of availability of the primary tumor for comparison and inability to use immunohistochemical stains. CONCLUSIONS Pathologists should be aware of this pitfall especially in frozen section analysis of sentinel lymph node to avoid misdiagnosis and its associated potential grave consequences.

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We present a case of a 73-year-old male who initially presented with night sweats, intermittent fever, worsening dry cough and shortness of breath. CT scans revealed atelectasis and calcified mediastinal lymphadenopathy, raising a suspicion for sarcoidosis. Multiple lung biopsies were performed. Microscopically, atypical lymphocytes were identified within capillaries, small arteries and veins. These lymphocytes were large with prominent nucleoli. Immunohistochemical staining demonstrated tumor cells positive for CD20, CD79a, Pax-5, CD10 and Mum-1, while negative for CD3, cytokeratin, S100, and CD34. LDH serum level was increased (480 IU/L). Extra pulmonary lymphoma was not detected elsewhere in the patient. These findings support the diagnosis of primary lung intravascular large B cell lymphoma (IVLBCL). Literature review of 52 cases demonstrated occurrence of primary lung IVBCL in patients between the ages (35-85) with a slight male predominance (1.167:1). The most common clinical presentation was fever associated with dyspnea.

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Metastatic carcinomas to the thyroid are quite rare in daily cytology practice. However, when present they may produce a diagnostic dilemma, particularly when they share some morphologic similarities with primary thyroid lesions and when occurring in patients with occult malignant history. Herein, we report a case of metastatic gastric signet ring cell carcinoma to the thyroid. Our patient presented with an isolated right thyroid nodule, which was clinically considered to be a primary thyroid neoplasm. Fine-needle aspiration (FNA) cytology of the nodule revealed a cellular specimen with cohesive fragments and scattered individual neoplastic cells. The neoplastic cells had enlarged nuclei, fine chromatin, and inconspicuous nucleoli. Nuclear crowding, molding, and grooving were prominent. Intranuclear inclusion-like clearance was identified. Some tumor cells also had eccentric nuclei, creating a signet ring cell appearance. The colloid was scant. These cytological features may be seen in cases of papillary thyroid carcinoma or signet ring cell follicular adenoma; however, the presence of the signet ring cells is unusual in primary thyroid lesions and raises the possibility of a metastatic lesion to the thyroid. In our case, the tumor cells were positive for AE1/AE3, mucicarmine, and periodic acid-Schiff, but negative for thyroglobulin and thyroid transcription factor-1. The patient was also found to have a 3.7-cm mass in the distal esophagus/proximal stomach. Biopsy of this mass showed an invasive signet ring cell carcinoma. The purpose of our study is to discuss the cytological features and the differential diagnosis of this unusual thyroid FNA case.

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Well-differentiated papillary mesothelioma (WDPM) is an uncommon subtype of epithelioid mesothelioma. In contrast to malignant epithelioid mesothelioma, WDPM has a low malignant potential and an indolent clinical course. WDPM may be difficult to diagnose and differentiate from benign reactive mesothelial cells and other malignant neoplasm on cytology specimens due to the presence of papillary or tubulopapillary clusters of tumor cells. We report a case of a 63-year-old Asian male with a slowly growing left inguinal hernia mass for several years and a concurrent 8 cm mass in the peritoneal wall. The cytology of ultrasound-guided fine-needle aspiration (FNA) of the left inguinal hernia and peritoneal masse reveal cellular specimens with numerous individual and tubulopapillary clusters of epithelioid mesothelial cells in a background of scant hyalinized material. Tumor cells show minimal cytological atypia. The differential diagnoses are broad and include reactive mesothelial cells, WDPM, and other malignant neoplasm. The follow-up surgical resection of masses reveals features of WDMP. It is important to recognize this entity in the differential diagnosis, because the clinical management of WDPM is quite different from that of malignant neoplasm. On the basis of the published data in the literature, it suggests that in male patients, the WDPM occurs predominantly in pleural cavity of older men in their 50s, and about half of the patients have history of asbestos exposure. However, the data is limited and insufficient for a definitive conclusion.

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A patient with microscopic metastatic renal cell carcinoma within liver focal nodular hyperplasia (FNH) is reported here. The initial CT scan was classic for liver FNH yet the following liver resection showed a microscopic metastatic renal cell carcinoma (0.48 cm) within the liver nodule. FNH in a patient with existing primary tumor probably warrants further investigation as the coexistence of metastatic disease may significantly alter treatment. The high arterial blood flow in the liver FNH may predispose the region to develop a metastatic tumor. Also possible is that a metastatic tumor may cause a local vascular flow abnormality which induces hepatocellular hyperplasia, resulting in a secondary FNH.

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A case of the combination of tetralogy of Fallot, hypertrophic cardiomyopathy, and Down's syndrome is reported here and is the first report on the combination of the 3 entities. Tetralogy of Fallot is often associated with chromosomal aberration, while hypertrophic cardiomyopathy associates with certain gene loci. Our experience with treating this patient, although ultimately unsuccessful, may provide useful information in any future cases.

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VEGF, an endothelial-specific mitogen, is an important tumor angiogenesis growth factor. The major receptor for VEGF on endothelial cells is KDR. We hypothesized that an intrabody could bind newly synthesized KDR and block receptor transport to the cell surface, thereby inhibiting important VEGF effects. We expressed a single chain antibody (p3S5) to KDR with or without the endoplasmic reticulum (ER) retention signal (KDEL), using either a plasmid (p3S5-HAK) or a tet-off adenoviral system (Ad-HAK). Plasmid-mediated expression of the tethered intrabody significantly reduced KDR expression (from 82.5+/-12.5% to 27.9+/-13.6% of cells; P<0.01) and thymidine incorporation in successfully transfected cells. Ad-HAK infection resulted in intrabody expression in >90% of human umbilical vein endothelial cells (HUVECs), producing marked (80%) apoptosis at 48 h postinfection. The intrabody was essential for these effects, as confirmed by inhibiting its expression with doxycycline or by expressing irrelevant genes (lacZ, GFP). Cell death was dependent on KDR, because Ad-HAK infection of cell lines with minimal or no KDR had little effect on cell viability. Infected HUVECs were unable to form tubes on Engelbreth Holm-Swarm (EHS) tumor gel matrix. These results demonstrate the potential for development of an intrabody-based strategy to block angiogenesis and prevent tumor growth.

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