A Primary Primitive Neuroectodermal Tumor Arising from Left Subclavian Vein and Extending along Left Brachiocephalic Vein and Superior Vena Cava into Right Atrium.

Primitive neuroectodermal tumor (PNET) is an extremely rare malignancy thought to be derived from fetal neuroectodermal precursor cells. It usually occurs in central and peripheral nervous system or soft tissue and bone, while intravenous or intracavitary PNET is considered as an extremely rare tumor. We reported a case of a 44-year-old woman who presented with the left unilateral facial and neck swelling. Magnetic resonance imaging revealed a tape-shaped solid mass within left subclavian vein, left brachiocephalic vein, superior vena cava, and right atrium; the proximal end proportion occupied almost the entire right atrium with a pedicle flip protruded into the right ventricle. Ultrasonography revealed an irregular hypoechnoic mass arising from the left subclavian vein, which extended along the left brachiocephalic vein and superior vena cava into the right atrium and up to the right ventricle. Positron emission tomography-computed tomography revealed several hypermetabolic thyroid nodules with no evidence of intravenous hyperactive lesion. The patient underwent tumor resection under cardiopulmonary bypass. At 15 days postoperatively, total thyroidectomy and resection of the left subclavian vein were simultaneously performed. The patient received chemotherapy and radiotherapy later. Histologically, the neoplasm displayed small, round, blue cells with hyperchromatic nuclei and scant cytoplasm. The neoplastic cells showed a strong immunopositivity for CD99, synaptophysin, CD56, CD57, and friend leukemia integration 1, thus confirming a diagnosis of the PNET. Histopathological examination of the thyroid showed papillary carcinoma. Thus, this PNET had no definitive organ or tissue of origin, which primarily originated from the left subclavian vein with tumor extension along the superior vena cava to the right ventricle.

Systematic Oxidative Stress Indexes Associated with the Prognosis in Patients with T Lymphoblastic Lymphoma/Leukemia.

Background: T lymphoblastic lymphoma/leukemia (T-LBL/ALL) is an aggressive malignant tumor with 5-year overall survival (OS) rate reached 80% after high-dose chemotherapy. Due to the relatively low incidence of T-LBL/ALL, only a few risk factors have been identified. The occurrence and prognosis of malignant tumors are closely related to oxidative stress, but the prognostic relationship between T-LBL/ALL and systematic oxidative stress indexes has not been reported yet. Methods: A total of 258 T-LBL/ALL patients were retrospectively analyzed. The relationship between systematic oxidative stress indexes, such as creatinine (CRE), gamma-glutamyl transpeptidase (γ-GGT), albumin (ALB), alkaline phosphatase (ALP), fibrinogen (FBG), C-reactive protein (CRP) and total bilirubin (TBIL), and survival of T-LBL/ALL patients, was analyzed. The weight of indexes was used to calculate the patients' oxidative stress risk score. The independent prognostic value of oxidative stress risk grouping (OSRG) was analyzed. Results: Higher CRE, CRP, and lower ALB were associated with poorer OS in T-LBL/ALL patients. The OSRG established by CRE, ALB, and CRP was an independent prognostic factor for OS of T-LBL/ALL patients. Patients in the high-risk group were more likely to be patients older than 14 years old and patients with superior vena cava obstruction syndrome (SVCS), pleural effusion, pericardial effusion, and mediastinal mass. Conclusion: For OS in T-LBL/ALL patients, OSRG was observed as an independent prognostic factor, which provided a new idea for accurate prognostic prediction.

Does catheter material affect functional performance of intravenous ports via the superior vena cava?

INTRODUCTION: The catheter is the only intravascular portion of an implanted port and plays a crucial role in catheter related complications. Both polyurethane and silicone are biocompatible materials which are utilized for catheter manufacturing, but their correlation to complications remains controversial. The aim of this study was to try to analyze the relationship between catheter materials and complications. MATERIALS AND METHODS: A total of 3144 patients who underwent intravenous port implantation between March 2012 and December 2018 at Chang Gung Memorial Hospital, Linkou, Taiwan were recruited. Of these, 1226 patients received silicone catheter port implantation and 1679 received polyurethane catheter ports. Case matching was done prior to analysis and catheter related complications and cumulative complication incidence for each group were compared. RESULTS: Intergroup differences were identified in entry vessel (p = 0.0441), operation year (p < 0.0001), operation method (p = 0.0095), functional period (p < 0.0001), patient follow up status (p < 0.0001), operating time for vessel cutdown (p < 0.0001) and wire assisted approach (p = 0.0008). Stratified by specific entry vessel, no statistical difference was found in complication rate or incidence between the silicone and polyurethane groups. We further compared the cumulative complication incidence of the silicone and polyurethane groups, and also found no statistical difference (p = 0.4451). CONCLUSION: As long as external stress forces generated by surrounding structures and focused on potential weak points are avoided, both silicone and polyurethane materials provide sufficient structural stability to serve as reliable vascular access for patients.

Cytokeratin-positive interstitial reticulum cell (CIRC) tumor in the lymph node: a case report of the transformation from the epithelioid cell type to the spindle cell type.

BACKGROUND: Cytokeratin-positive interstitial reticulum cells (CIRCs), which are a subgroup of fibroblastic reticular cells (FRCs), are known to be present in the lymph nodes. There have been only a few cases of tumors derived from CIRCs. CASE PRESENTATION: We have reported a new case involving a CIRC tumor in a 75-year-old man and reviewed the literature. The resected mediastinal lymph nodes showed epithelial-like proliferation of large atypical round and polygonal epithelioid cells. The tumor cells expressed CK8, CK18, CAM5.2, AE1/AE3, epithelial membrane antigen, vimentin, fascin, and some FRC markers, which is consistent with the diagnosis of a CIRC tumor. Following chemotherapy, the CIRC tumor was observed to have responded very well and became difficult to confirm on imaging, but a small cell lung carcinoma developed 12 months later. Chemoradiotherapy was performed, but the patient passed away 29 months after the initial diagnosis. The autopsy revealed the recurrence of the CIRC tumor, residual small cell lung carcinoma, and a very small latent carcinoma of the prostate. The relapsed CIRC tumor cells had a spindle shape; they were highly pleomorphic and had invaded the superior vena cava. CONCLUSION: We first reported autopsy findings of CIRC tumors and demonstrated the transformation of the tumor from the epithelioid cell type to the spindle cell type.

Extended atrial conduction system characterised by the expression of the HCN4 channel and connexin45.

OBJECTIVE: In the heart, there are multiple supraventricular pacemakers involved in normal pacemaking as well as arrhythmias and the objective was to determine the distribution of HCN4 (major isoform underlying the pacemaker current, I(f)) in the atria. METHODS: In the atria of the rat, the localisation of HCN4 and connexins was determined using immunohistochemistry, and electrical activity was recorded using extracellular electrodes. RESULTS: As expected, HCN4 and Cx45 (but not Cx43) were expressed in the sinoatrial node extending from the superior vena cava down the crista terminalis. The same pattern of expression of HCN4 and connexins was observed in a novel tract of nodal-like cells extending from the superior vena cava down the interatrial groove. Although the sinoatrial node was usually the leading pacemaker site, the novel tract of HCN4-expressing cells was capable of pacemaking and could act as the leading pacemaker site; there was evidence of a hierarchy of pacemakers. The same pattern of expression of HCN4 and connexins was also observed in the atrioventricular ring bundle (including the atrioventricular node) encircling the tricuspid valve, but not in the atrioventricular ring bundle encircling the mitral valve. HCN4 was not expressed in the pulmonary veins. CONCLUSIONS: The widespread distribution of HCN4 can explain the widespread location of the leading pacemaker site during sinus rhythm, the extensive region of tissue that has to be ablated to stop sinus rhythm, and the widespread distribution of ectopic foci responsible for atrial tachycardia.

Markers of activated hemostasis and fibrinolysis in patients with pulmonary malignancies: comparison of plasma levels in central venous and pulmonary venous blood.

Malignancy frequently is accompanied by activated coagulation and fibrinolysis indicating a hypercoagulable state. The purpose of our study was to estimate the contribution of local tumor-induced mechanisms to the activation of hemostasis and fibrinolysis. In a prospective study, we compared the plasma levels of thrombin-antithrombin complexes, prothrombin fragment 1+2, and D-dimers in blood samples that simultaneously were drawn from the superior vena cava and the pulmonary vein of a tumor-bearing pulmonary lobe. Samples from the superior vena cava were drawn before operation and served as controls. After thoracotomy, a second group of samples was simultaneously taken from the pulmonary veins of the tumor-bearing lobe and the superior vena cava. Forty-five patients with pulmonary malignancies were included (25 adenocarcinomas and 20 squamous cell carcinomas). There were no significant differences of thrombin-antithrombin complexes, prothrombin fragment 1+2, and D-dimers levels in patients suffering from adenocarcinoma and from squamous cell carcinoma. Intraoperatively, prothrombin fragment 1+2 and D-dimers levels were markedly increased when compared with the preoperative values (p<0.0001). There was no increase of thrombin-antithrombin complexes levels due to the operative traumatization. Prothrombin fragment 1+2, thrombin-antithrombin complexes, and D-dimers plasma levels were significantly higher in the pulmonary venous blood than in the blood simultaneously drawn from the superior vena cava (p<0.0001). Our findings indicate that malignant lung tumors directly contribute to the activation of hemostasis and fibrinolysis in these clinical settings.

Ketone body ratios of the superior and inferior vena cava and of pulmonary arterial blood compared to that of arterial blood: central venous ketone body ratio as a substitute for the arterial ketone body ratio.

To investigate the ketone body ratio (acetoacetate/3-hydroxybutyrate) of central venous blood compared to that of peripheral arterial blood, the acetoacetate and 3-hydroxybutyrate concentrations in paired peripheral arterial and central venous or pulmonary arterial blood were measured. The ketone body concentrations in superior and inferior vena cava blood were significantly (P < 0.0001) lower than those in peripheral arterial blood, whereas those in pulmonary arterial blood were almost the same as those in peripheral arterial blood. These results indicate that ketone bodies were metabolized in the muscles, which reduced their levels in vena cava blood, but ketone bodies newly produced by the liver were transported to the right side of the heart via the hepatic vein, giving concentrations in pulmonary arterial blood that were almost the same as those in peripheral arterial blood. On the other hand, the correlation coefficients (r2) of the arterial blood ketone body ratio to the ratio of superior and inferior vena cava and pulmonary arterial blood were 0.897, 0.767 and 0.882, respectively. The ratios of central venous ketone body ratio/arterial blood ketone body ratio were 0.89 +/- 0.15 in the superior vena cava, 0.64 +/- 0.18 in the inferior vena cava and 1.01 +/- 0.15 in the pulmonary artery.

Differential lectin binding on walls of thoraco-cervical blood vessels and lymphatics in rats.

Lectin binding in the walls of large to medium-sized blood vessels and lymphatics in the rat thoraco-cervical region was examined histochemically. The tunica intima of the aorta and superficial cervical artery showed positive reactions with wheat germ agglutinin (WGA) and Concanavalin A (ConA) but not with Dolichus biflorus agglutinin (DBA). The tunica media of the aorta exhibited intense WGA binding, especially on the smooth muscle cells, but the tunica media of the superficial cervical artery did not react with the lectin. Neither ConA nor DBA bound to the tunica media of the aorta and superficial cervical artery. The tunica adventitia of both arteries contained sites binding the three lectins, although DBA reactivity declined as the vascular diameter decreased. The tunica intima of the superior vena cava and azygos vein exhibited positive WGA and ConA binding, whereas DBA binding was noted on only part of the tunica intima of the superior vena cava and not on that of the azygos vein. The tunica media and tunica adventitia were reactive for all three lectins. The WGA and ConA binding sites in the tunica adventitia showed loose networks, suggesting lectin binding on connective tissue elements interlacing among smooth muscle bundles. Lectin binding sites in the walls of lymphatics exhibited an arrangement similar to those in the walls of the veins. Moreover valves protruding into the lumen showed intense WGA and ConA binding and scattered DBA binding. Three other lectins (Ulex europaeus agglutinin, peanut agglutinin, Maclura pomifera) were examined, but they showed no reactions with the vessels. Thus, the differential binding of lectins on the walls of blood vessels and lymphatics of various sizes suggests the functional complexity of monosaccharide residues in the vascular walls.

Balloon angioplasty for disruption of tunneled dialysis catheter fibrin sheath.

PURPOSE: Management of failing tunneled hemodialysis catheters, sometimes the only vascular access for hemodialysis, presents a difficult problem. In spite of various techniques having been developed, no consensus has been reached about the preferred technique, associated with the longest catheter patency. METHODS: We report disruption of the fibrin sheath covering dysfunctional tunneled hemodialysis catheter by means of angioplasty, followed by over guidewire catheter exchange. RESULTS: Following the procedure, the catheter placed in the recovered lumen of the vessel presented correct function. CONCLUSIONS: The described procedure allowed maintenance of vascular access in our patient. Additionally, dilatation of the concomitant central vein stenosis opens an option for another attempt for arteriovenous fistula creation.

Epithelioid haemangioendothelioma of the superior vena cava.

Primary calcified tumours of major central veins are extremely rare. Epithelioid haemangioendotheliomas (EHs) are malignant tumours of vascular origin with very limited reports in the literature. The aetiology is unknown. Immunohistochemically, tumours are often positive for at least one endothelial marker. We present a unique presentation of an EA in the superior vena cava.

The tissue plasminogen activator and urokinase response in vivo during natural resolution of venous thrombus.

PURPOSE: The aim of this study was to measure the distribution of endogenous plasminogen activators during thrombolysis with an endothelial-conserving model of laminated thrombosis. METHODS: Thrombi were raised in the inferior vena cava of rats with thrombin and flow reduction. The thrombi, adjacent vein wall, and distant veins (the superior vena cava) were removed at intervals from 1 hour to 21 days from formation and then cryohomogenized and assayed with specific bioimmunoassays for tissue-type (t-PA) and urokinase-type plasminogen activators (u-PA). RESULTS: The measured t-PA activity of the vein wall around the thrombus was reduced compared with the control inferior vena cava at 4 days. Both the u-PA and t-PA content of the thrombus increased progressively during thrombolysis. The t-PA activity increased significantly in the distant vein walls in the animals with thrombi. Immunocytochemistry and in situ hybridization localized the t-PA to a mononuclear cell infiltrate and showed up-regulation of mRNA for rat t-PA in these monocytes. CONCLUSIONS: The local plasminogen activator response was predominantly within the thrombus itself. Increased t-PA activity was additionally found in distant veins but was reduced in the vessel wall adjacent to the thrombus. This is the first report to show that u-PA activity is increased within organizing thrombus in vivo and that most of the t-PA activity is localized to a monocyte infiltrate.