Mediastinal Widening Research Articles

Two cases of aortic emergency presenting with neurologic manifestations, aided by POCUS

Acute aortic dissection and aneurysm are lethal vascular emergencies and may present with various clinical presentations including neurological manifestation. Thus, the diagnosis of aortic dissection and aneurysm can be challenging as it may mimic other disorders whereby misdiagnosis can be fatal. In district general hospitals where advanced radiological modalities are not widely available, Point of Care Ultrasound (POCUS) is a tool to diagnose aortic dissection and aneurysm rapidly and accurately. The first case was a 60-year old, Chinese gentleman presented with alternating conscious level. He had a history of syncope that was precipitated by shortness of breath. On examination, his initial GCS was E1,V3, M5 but he regained full consciousness when we laid him supine for intubation. He complained of severe tearing chest pain. He demonstrated radio-radial and radio-femoral delays. Chest X ray showed mediastinal widening, and bedside echocardiography revealed aortic root dilatation with intimal flaps. Patient was sent to a tertiary centre for Computed Tomography of Aorta that confirmed the diagnosis, and vascular repair was planned.The second case was a 70-year old, Malay gentleman presented with recurrent tonic-clonic seizure. On examination, there was a palpable pulsatile mass over epigastric and umbilical region. Bedside ultrasound revealed aortic aneurysm measuring 5.4 x 5.8 cm with peri-aortic haematoma. Despite intense resuscitation, pulseless electrical activity ensued while awaiting for tertiary referral. The presentation of aortic dissection and aneurysm can vary and mimic other deadly diseases in which misdiagnosis can be fatal. Most common neurological manifestations are transient ischaemic attack and ischaemic stroke. POCUS is increasingly used by emergency physician in acute care as it is rapid, non-invasive, widely available and allows accurate measurement of the aorta. Aneurysmal rupture between 4.5 to 5.5 cm is a useful guide for surgical prophylaxis. Intimal flaps visualisation has a sensitivity of 67-80 % and a specificity of 99 -100 %with demonstration of colour flow in both true and false lumens in Doppler, strengthening the diagnosis of aortic dissection. Clinicians should be aware of the unique presentations of aortic dissection and aneurysm, as both can mimic other serious diseases whereby misdiagnosis can be fatal. In district setting where advanced radiological imaging is not readily available, the utility of POCUS in the ED can be crucial to diagnose aortic dissection and aneurysm.

Manejo terapéutico de hematoma mediastinal después de un acceso venoso central

Introducción: el hematoma mediastinal es una causa rara de complicación en el abordaje de la vía venosa central (VVC). Estudio de Gupta 2011 reportó uno y revisó varios casos clínicos analizando la conducta terapéutica. Caso clínico: mujer de 51 años con ventilación mecánica invasiva en terapia intensiva por encefalitis viral, que después de la colocación de VVC subclavia presentó inestabilidad hemodinámica, dificultad para ventilar con resistencias elevadas y compliance disminuida, hace paro cardiaco que revierte con maniobras, se precisa diagnóstico de hematoma mediastinal y se indica manejo conservador con el que evoluciona satisfactoriamente. Discusión: el hematoma de mediastino siempre debe sospecharse y es evidente en radiografías de tórax en los casos graves, con ensanchamiento mediastinal. La ventilación mecánica establece una presión positiva intratorácica permitiendo contener el hematoma, el manejo conservador se reporta beneficioso. Conociendo que las complicaciones de colocación de VVC son mínimas pero con elevado riesgo de inestabilidad, prolongación de hospitalización y aumento de la mortalidad, se recomienda evitar abordajes innecesarios, en especial subclavios.

Vena cava superior syndrome in the children with mediastinal tumors: Single-center experience.

OBJECTIVE:Vena cava superior syndrome comprises various symptoms of compression of vena cava superior. The results of increased venous pressure in the upper body may cause edema of the head and neck associated with cyanosis, plethora and distended subcutaneous vessels. Vena cava superior syndrome is rare in childhood. Therefore, we planned this retrospective study.METHODS:The retrospective study was carried out on the children with mediastinal tumors in the Department of Pediatric Hematology-oncology, Erciyes University Faculty of Medicine, Kayseri, Turkey , from January 2010 to December 2017. Diagnostic procedures included hematological investigations, chestradiography, thoracic computed tomography, echocardiography and lymph node or mediastinal biopsy.RESULTS:In this study, 19 (five were female) of 41 patients with mediastinal tumors had Vena cava superior syndrome. Diagnosis included Hodgkin’s lymphoma in seven (37%), non-Hodgkin’s lymphoma in six (32%), acute T- lymphoblastic leukemia in four (21%), neuroblastoma and anaplastic round cell sarcoma in one each respectively. All of the 19 patients’ facial swelling, venous distention and mediastinal widening. All patients received intravenous corticosteroids (0.6 mg/kg dexamethasone). Furthermore, the patient with anaplastic round cell sarcoma received emergency radiotherapy. No patients died because of Vena cava superior syndrome.CONCLUSION:Vena cava superior syndrome is a medical emergency that requiresurgent treatment. Vena cava superior syndrome studies in children are rare. In this retrospective study, we found that the most common cause of Vena cava superior syndrome was Hodgkin’s lymphoma different from literature.

PULMONARY HYPERTENSION AND ISOLATED RIGHT HEART FAILURE: AN UNUSUAL PRESENTATION OF THYROID STORM

SESSION TITLE: Pulmonary Manifestations of Systemic Disease 1 SESSION TYPE: Med Student/Res Case Report PRESENTED ON: 10/09/2018 03:45 PM - 04:45 PM INTRODUCTION: Graves' disease and other hyperthyroid states are known to have significant effects on the cardiovascular system. These may include increase in heart rate, increase in cardiac output, and decrease in systemic vascular resistance[1]. When heart failure is present, left ventricular dysfunction is frequently reported[2,3]. Thyrotoxicosis rarely presents with pulmonary hypertension and isolated right heart failure. CASE PRESENTATION: A 42-year-old African-American female with no past medical history was sent into the hospital by her outpatient physician for tachycardia. History revealed unintentional 70-pound weight loss over one year. Vital signs were notable for tachycardia identified as atrial fibrillation with rapid ventricular response(>150bpm) on ECG[Figure 1]. Physical exam revealed exophthalmos, symmetrically enlarged and non-tender thyroid, JVD 11 cm, irregularly-irregular tachycardia, decreased breath sounds right lung base, bilateral 2+ pitting edema of lower extremities, and hyperreflexia. Laboratory values indicated suppressed TSH(<0.004 uIU/mL) with elevated T3 Total, T4, and TSH-receptor antibody(494ng/dL, 23.14ug/dL, 111.10IU/L respectively). Diagnosis of Graves' disease was made. Burch-Wartofsky Point Scale of 55 suggesting thyroid storm. Treatment initiated with propranolol, methimazole, glucocorticoids. Chest x-ray showed right pleural effusion, cardiomegaly, and mediastinal widening[Figure 2]. Ultrasound thyroid showed enlarged, heterogeneous, hypervascular gland. Radioactive iodine scan showed diffuse uptake. Echocardiogram showed severe pulmonary hypertension(PASP 88mmHg), right ventricular systolic dysfunction, severe tricuspid regurgitation, left ventricular ejection fraction of 60%. Workup for causes of pulmonary hypertension negative for HIV, connective tissue disease, drugs, toxins, underlying pulmonary disease, and thromboembolic disease. Patient discharged after radioiodine ablation of thyroid gland. Follow up echocardiogram approximately six months after treatment showed significant improvement of both pulmonary hypertension (PASP 35 mmHg) and right heart systolic function. DISCUSSION: This case demonstrates that thyrotoxicosis can induce pulmonary hypertension and right-sided heart failure independent of left-heart disease. Proposed mechanisms of pulmonary hypertension in thyrotoxicosis include autoimmune endothelial dysfunction, endothelial damage from high cardiac output, changes in metabolism of vasodilators or vasoconstrictors, and sympathetic pulmonary vasoconstriction. CONCLUSIONS: It is important to consider thyrotoxicosis in patients with pulmonary hypertension and right heart failure since normalizing thyroid hormones can reverse even severe cardiopulmonary disease. Reference #1: Klein, I., & Ojamaa, K. (2001). Thyroid hormone and the cardiovascular system. The New England Journal of Medicine, 344(7), 501-509. https://doi.org/10.1056/NEJM200102153440707 Reference #2: Klein, I., & Danzi, S. (2007). Thyroid Disease and the Heart. Circulation, 116(15), 1725-1735. https://doi.org/10.1161/CIRCULATIONAHA.106.678326 Reference #3: Siu, C.-W., Yeung, C.-Y., Lau, C.-P., Kung, A. W. C., & Tse, H.-F. (2007). Incidence, clinical characteristics and outcome of congestive heart failure as the initial presentation in patients with primary hyperthyroidism. Heart (British Cardiac Society), 93(4), 483-487. https://doi.org/10.1136/hrt.2006.100628 DISCLOSURES: No relevant relationships by Mina Bushra, source=Admin input No relevant relationships by Kevin Rabii, source=Web Response No relevant relationships by Sally Ziatabar, source=Web Response

AN INCIDENTAL FINDING OF MASSIVE MEDIASTINAL DENSITY FROM A CHEST RADIOGRAPH

SESSION TITLE: Cardiothoracic Surgery SESSION TYPE: Med Student/Res Case Rep Postr PRESENTED ON: 10/09/2018 01:15 PM - 02:15 PM INTRODUCTION: Bicuspid aortic valve (BAV) is one of the most common congenital heart diseases, occurring in about one percent of the population. Patients with BAV have higher frequency of aortic root and ascending aortic dilatation on echocardiogram compared to patients with normal tricuspid aortic valve. The aortic dilatation caused by BAV generally involves aortic root and ascending thoracic aorta, but it may also involve transverse aorta (1). The following case demonstrates an isolated aortic arch aneurysm, which has not been reported in the literature to our knowledge yet. CASE PRESENTATION: A 23-year-old African American male with past medical history of ventricular septal defect repair had a routine chest radiograph, which showed mediastinal widening (Figure 1). Transesophageal echocardiography showed a bicuspid aortic valve, moderate to severe aortic regurgitation, severely dilated right atrium and right ventricle, dilated aortic root, and moderately dilated proximal ascending aorta measuring 4.4cm. Computed tomography angiography (CAT) showed massive dilatation of the transverse aortic arch extending from the origin of the brachiocephalic artery to just distal to the left subclavian artery, followed by a sharp transition to a normal descending aorta without any collateral circulation (Figure 2, Figure 3). DISCUSSION: Aortic root and ascending aorta dilatation are known complications of bicuspid aortic valve, but an isolated aneurysm of the aortic arch has not been previously reported. This is favored to represent a congenital anomaly given his BAV, history of VSD, and relatively early albeit incidental presentation of his massive aneurysm (2). Coarctation of the aorta is a narrowing of the aorta near the ductus arteriosus, which is associated with bicuspid aortic valve. Our patient’s CTA showed no enlarged intercostal arteries or other collateral circulation. A variation of pseudocoarctation of the aorta, which is a rare congenital deformation and elongation of the aortic arch at the level of ligamentum arteriosum without obstruction or pressure gradient is also on the differential (3). Cervical aortic arch can present similarly, but the patient’s chest radiograph showed aortic arch located unequivocally below the clavicle, making this diagnosis less likely. The treatment for aortic aneurysm depends on the location and the extent of the aortic dilatation. The patient required complex aortic surgery with possible elephant trunk repair and was referred to a tertiary care center for further surgical evaluation. CONCLUSIONS: Aneurysmal dilatation of the aortic root and ascending aorta is common among BAV patients. An isolated aortic arch aneurysm with relatively minimal dilatation of the aortic root and ascending aorta has not been reported in the literature. This may represent part of a spectrum of congenital disorder, and pathological studies would help to further evaluate this unknown entity. Reference #1: Fazel, Shafie S., et al. "The aortopathy of bicuspid aortic valve disease has distinctive patterns and usually involves the transverse aortic arch." The Journal of thoracic and cardiovascular surgery 135.4 (2008): 901-907. Reference #2: Caglayan, Ahmet Okay, and Munis Dundar. "Inherited diseases and syndromes leading to aortic aneurysms and dissections." European Journal of Cardio-Thoracic Surgery 35.6 (2009): 931-940. Reference #3: D’Cruz, Ivan A., et al. "Noninvasive diagnosis of right cervical aortic arch." Chest 83.5 (1983): 820-822. DISCLOSURES: No relevant relationships by william bates, source=Web Response No relevant relationships by jayanth keshavamurthy, source=Web Response No relevant relationships by Haesung Lee, source=Web Response No relevant relationships by Ilkyu Oh, source=Web Response No relevant relationships by Allan Wang, source=Web Response

LEFT SUBCLAVIAN ARTERY RUPTURE DUE TO BLUNT THORACIC TRAUMA: A CASE REPORT

Abstract. Blunt thoracic trauma highest incidence is in adult, 20% to 50 % of the trauma cause death. Injuries to the vascular structures of the thoracic outlet, especially left subclavian artery, are rare and typically accompanied by massive hemorrhage. Close observation of vital sign and serial chest x-ray are very important. We describe an unusual presentation of a patient who suffered traumatic rupture ofleft subclavian artery. No clinical presentation of supraclavicular hematoma, unilateral absence of radial artery pulse and mediastinal widening in chest x-ray was found, but there is a massive hemothorax. Left posterolateral thoracotomy was performed, the source of bleeding was found in the apex of the lung, suspected a rupture of the left subclavian artery. The procedure continued with median sternotomy extended to left supraclavicular incision, a rupture was found in the left subclavian artery, 1,5 cm proximal to aortic arch. Primary repair was done with a good result. Key Words : Blunt thoracic trauma, massive hemothorax, left subclavian artery rupture

Value of CD, MPO, Ki-67 and C-MYC Positive Rate in the Pathological Tissues and C-MYC Gene of Patients with T-LBL/ALL for Predicting Prognosis

To explore the value of of CD, MPO, Ki-67, C-MYC positive rates in the pathological tissues and C-MYC gene of patients with T-LBL/ALL for predicting Prognosis. Ninty cases of T-LBL/ALL patients in our hospital were selected and included in the T-LBL/ALL group, and 30 cases of lymphnode reactive hyperplasia were selected as control group. Immunohistochemical staining was used to detect the changes of CD, MPO, Ki-67 and C-MYC positive rate in 2 groups, and the changes of C-MYC gene were detected by fluorescence in situ hybridization. In 90 patients with T-LBL/ALL, there were CD1a+ 34 cases (37.8%), CD3+ 67 cases (74.4%), epsilon CD3+ 47 cases (52.2%), CD7+ 85 cases (94.4%), CD10+ 33 cases (36.7%), CD34+ 22 cases (24.4%), CD43+ 48 cases (53.3%), CD45RO+ 46 cases (51.1%), CD99+ 88 cases (97.8%), TDT+ 85 cases (94.4%); and CD23, CD20, and MPO all were negative; Ki-67>80% 47 cases (52.2% cases), Ki-67≤80%, 43 cases (47.8%). In 90 T-LBL/ALL patients, the positive rate of C-MYC (66.7%) was significantly higher than the control group (positive rate 0.0%) (P< 0.05); the Ki-67 index, mediastinal widening of T-LBL/ALL patients and the positive rate of C-MYC positively were correlated (P< 0.05). The overall survival rate (44.0%) of C-MYC negative patients was significantly higher than that of C-MYC positive patients (0.0%). The overall survival rate of C-MYC negative patients was significantly higher than that of C-MYC positive patients (P< 0.05).Ann Arbor staging, LDH, bone marrow involvement, mediastinal widening, Ki-67 positive index, and C-MYC protein expression of patients with T-LBL/ALL did not correlated with increased C-MYC gene breakage and copy number (P> 0.05). The overall survival rate of C-MYC positive patients decreases, which positively correlates with Ki-67 positive index and mediastinal width, suggesting that the prognosis of the patients with C-MYC protein expression is poorer.

The diagnostic accuracy of the mediastinal width on supine anteroposterior chest radiographs with nontraumatic Stanford type A acute aortic dissection.

BackgroundNontraumatic Stanford type A acute aortic dissection is a life‐threatening condition; thus, the ability to make a precise diagnosis of nontraumatic Stanford type A acute aortic dissection is essential for the emergency physician. Several reports have shown that the mediastinal widening on a chest radiograph is useful for the diagnosis of nontraumatic Stanford type A acute aortic dissection; however, the exact cutoff value of the mediastinal width on plain radiographs is rarely defined.MethodsA single‐center retrospective case‐control study was conducted between October 1, 2013, and March 31, 2015. We evaluated the maximal mediastinal width of the anteroposterior chest X‐ray at the level of the aortic knob in the supine position between patient groups with and without nontraumatic Stanford type A acute aortic dissection.ResultsWe enrolled 72 patients (36 patients with nontraumatic Stanford type A acute aortic dissection and 36 patients without nontraumatic Stanford type A acute aortic dissection). The median mediastinal width of patients with nontraumatic Stanford type A acute aortic dissection was significantly larger than that of patients without nontraumatic Stanford type A acute aortic dissection (100.7 mm vs 77.7 mm, P < .01). The optimal cutoff level was 87 mm (sensitivity, 81%; specificity, 89%). Using multivariable logistic regression, the odds ratio of a mediastinal width of >87 mm for a diagnosis nontraumatic Stanford type A acute aortic dissection was 57.1 (95% confidence interval, 11.2‐290.2).ConclusionA mediastinal width of >87 mm showed high sensitivity in the diagnosis of probable nontraumatic Stanford type A acute aortic dissection.

Palpable thoracoabdominal aortic aneurysm through the chest wall: A late and ominous presentation

Thoracoabdominal/thoracic aortic aneurysm is often diagnosed incidentally while evaluating for dyspnea or atypical chest pain of long duration. Late presentation in various forms such as palpable pulsatile swelling in this patient is quite rare and fatal and foreruns impending rupture. We present a 53-year-old female who presented with acute-onset left-sided chest and back pain with swelling on the left side of the chest wall for 2 weeks. She was evaluated with chest X-ray which showed mediastinal widening and computed tomography scan thorax showed a large thoracic aortic aneurysm protruding out through seventh intercostal space onto the chest wall. She underwent open repair of aneurysm and was doing well at 6 months of follow-up. Thoracic aortic aneurysm mandates early diagnosis and prompt treatment as delay in the same leads to fatal rupture. Open surgical repair is the treatment of choice in large aneurysms causing significant compressive symptoms and imminent rupture.

Dysphagia as a Presentation of Spontaneous Mediastinal Hematoma With Life-Threatening Airway Obstruction

SESSION TITLE: Disorders of the Mediastinum 1 SESSION TYPE: Affiliate Case Report Poster PRESENTED ON: Tuesday, October 31, 2017 at 01:30 PM - 02:30 PM INTRODUCTION: Spontaneous atraumatic mediastinal hematomas are rare. Considering the infrequency with which it is encountered, the clinical identification and management of them can prove to be challenging. We report a case of spontaneous mediastinal hematoma in a patient presenting with dysphagia. CASE PRESENTATION: An otherwise healthy 67-year-old female presented to the emergency department with a 4 day history of worsening dysphagia to both solids and liquids, chest pain radiating to the upper back, and anterior chest bruising. The patient denied history of trauma, vomiting, cough or other constitutional symptoms. She had no reports of antiplatelet or anticoagulant use and no known personal or family history of bleeding problems. Physical examination was only significant for ecchymosis on the anterior chest. Initial blood work was unremarkable with a hemoglobin level of 13.0 g/dl, normal platelet count and coagulation profile. Over the next 48 hours, her hemoglobin dropped to 9.4 g/dl. Initial plain chest radiograph demonstrated widening of the superior and middle mediastinum. An emergent CT chest revealed a large retrotracheal mass extending from the oropharynx to the level of the subcarinal region, with mass effect and anterior displacement on the trachea consistent with a mediastinal hematoma. A conservative approach was initiated. The patient remained stable under intensive care monitoring. Repeat CT Chest 3-days following admission confirmed a decreasing size of the mediastinal hematoma. The patient's remaining hospital course was uncomplicated and she was discharged 10 days later. Hematologic work up for a bleeding diathesis was unrevealing. DISCUSSION: Less than two dozen cases of spontaneous atraumatic mediastinal hematomas have been reported to date. Mediastinal hematomas are commonly provoked by thoracic trauma, iatrogenic procedures, sustained hypertension, hematologic conditions and transient increase in intra thoracic pressure. Although rare, spontaneous mediastinal hematomas must be recognized as a potential cause for mediastinal widening. Dysphagia as a presenting symptom of spontaneous mediastinal hematoma is infrequently reported in the literature. CONCLUSIONS: Spontaneous mediastinal hematomas pose a life-threatening risk. Timely recognition and awareness of atypical presentations is vital to appropriate treatment and management. Although the primary etiology of the hemorrhage may remain unidentified, awareness of the phenomenon is crucial to the differential diagnosis. Reference #1: Li X, Liu L, Cao D, Sun Y. Spontaneous Hematoma of Posterior Mediastinum with an Uncommon Cause: A Case Report and Review of the Literature. Clinics and Practice. 2016;6(1):838. doi:10.4081/cp.2016.838. Reference #2: Iskander M, Siddique K, Kaul A. Spontaneous Atraumatic Mediastinal Hemorrhage: Challenging Management of a Life-Threatening Condition and Literature Review. Journal of Investigative Medicine High Impact Case Reports. 2013;1(2):2324709613484451. doi:10.1177/2324709613484451 DISCLOSURE: The following authors have nothing to disclose: Phyllis Suen, Abdul Siddiqui, Paul Catella, Shimshon Wiesel, Michel Chalhoub No Product/Research Disclosure Information

Second lung malignancy and Richter syndrome in chronic lymphocytic leukemia: case report and literature review

BackgroundChronic lymphocytic leukemia (CLL) is the most frequent lymphoproliferative disease. Transformation into Richter disease and occurrence of second malignancies involving the lungs are rare complications. The hallmarks of any thoracic involvement are still unknown.Case presentationWe report a case of a 56-year-old male patient, with history of tobacco smoking, who presented with recurrent hemoptysis, fatigue and weight loss. Physical examination was normal except a slightly enlarged supraclavicular lymph node. Chest x-ray revealed a mediastinal widening due to enlarged paratracheal nodes and a left parahilar infiltrate. Blood tests showed a hyperlymphocytosis and a biological inflammatory syndrome. CT scan showed bilateral mediastinal and axillary lymphadenopathy, as well as left supraclavicular lymphadenopathy, with a left upper lobe alveolar attenuation and a solitary contralateral pulmonary nodule. Examination of Virchow’s node and bone marrow biopsies confirmed metastasis of a pulmonary adenocarcinoma, as well as chronic lymphocytic leukemia with Richter’s transformation. The clinical course was unfavorable since the first days of therapy as the patient passed away in a matter of a few days.ConclusionsSteady surveillance of CLL patients and systematic screening for second solid tumors, particularly lung cancer, and Richter’s transformation seem to be relevant more than ever. Early diagnosis might help us understand the pathways leading to these complications and adapt therapy.

Multiple giant mid-thoracic esophageal diverticula in a teenager

Abstract Esophageal diverticula in the pediatric population are rare. The etiologies and presentations can vary widely which can make it difficult to diagnose without imaging or direct visualization. We are reporting a case of a 14-year-old male with history of asthma, anorexia, weight loss, and chest discomfort. CXR findings of mediastinal widening prompted a more extensive evaluation. He was found to have two esophageal diverticula, phlegmonous changes, and osteomyelitis of the T5 and T6 vertebra. He was initially treated with IV antibiotic therapy, bypassing the affected area with nasogastric enteric tube feedings, and a cervical thoracic orthosis brace for spine stability. He regained his lost weight and became asymptomatic, returning to a regular diet. The family has declined to pursue surgical intervention at present.

Aortic Dissection after Intravenous Thrombolysis in Acute Cerebral Infarction

Use of recombinant tissue plasminogen activator (rt-PA) for the treatment of acute cerebral infarction secondary to aortic dissection is challenging because of a narrow time window and potential life-threatening complications. An 80-year-old woman with right middle cerebral artery infarction was treated with rt-PA. Although she had no history, symptoms, or sign of aortic dissection, carotid CT angiography revealed aortic arch dissection. Mediastinal widening, which did not show on initial chest X-ray, developed on follow-up chest X-ray. This observation indicates that physicians should monitor patient symptoms for signs of aortic dissection during thrombolysis and perform chest X-ray or carotid angiography immediately after thrombolysis even if the patient has no symptoms or signs of aortic dissection on onset of acute cerebral infarction.

Primary mediastinal large B cell lymphoma in a woman who is human immunodeficiency virus positive presenting with superior vena cava syndrome: a case report

BackgroundThe risk of non-Hodgkin lymphoma is increased 200-fold in individuals seropositive for human immunodeficiency virus compared to those free from human immunodeficiency virus. Human immunodeficiency virus-associated non-Hodgkin lymphoma is known for its atypical presentation, aggressive ability, widespread involvement, poor response to chemotherapy, and high relapse potential which makes both the diagnosis and management a difficult undertaking especially in resource-poor settings.Case presentationWe report a case of primary mediastinal large B cell lymphoma in a 46-year-old woman of African descent who is human immunodeficiency virus positive who presented with symptoms of superior vena cava syndrome. Her past medical history was remarkable for a 23-year history of systemic hypertension and a 10-year history of human immunodeficiency virus infection. A physical examination revealed an underweight woman with right-sided facial, neck, upper limb, and trunk swelling together with distended veins on her chest and abdomen draining downwards. A respiratory examination revealed a reduced chest expansion, stony dull percussion note, and absent breath sounds on her entire right side with a left-sided tracheal deviation. She had a CD4 count of 146 cells/μL. A chest X-ray revealed a homogenous opacification on her right side with a left-sided tracheal deviation while a computed tomography scan of her chest revealed a solid mass on her right side. An echocardiogram showed a huge well-circumscribed mass (4.6×3.3 cm) with spontaneous echocardiographic contrast compressing her heart inferiorly. She had severe pulmonary hypertension (right ventricular systolic pressure 58 mmHg) but preserved left ventricular systolic function, no thrombus was seen, and her pericardium was normal. A computed tomography angiography of her aorta ruled out an aortic aneurysm. Finally, she underwent mediastinoscopy and a direct biopsy of the mass was taken for histopathology. Hematoxylin and eosin staining demonstrated a dense lymphoid infiltrate of large malignant cells with pleomorphic nuclei in clusters, compartmentalized by fine bands of fibrosis, and frequent mitoses were present. A diagnosis of mediastinal large B cell lymphoma was reached.ConclusionsThe presence of a mediastinal widening coupled with a history of unintentional yet significant weight loss in an individual who is human immunodeficiency virus seropositive should raise an index of suspicion for lymphomas and warrant aggressive investigations and timely management.

Significance of CXCL12/CXCR4 expression in T-lymphoblastic lymphoma/leukemia

Objective: To investigate the significance of CXCL12/CXCR4 expression in T lymphoblastic lymphoma/leukemia (T-LBL/ALL) and its prognostic significance. Methods: Using immunohistochemical EnVision method, CXCL12, CXCR4 and Ki-67 expression were evaluated in 72 cases of T-LBL/ALL and 30 selected cases of lymph node reactive hyperplasia (LH) as control. In addition, CXCL12 and CXCR4 mRNA expression levels were examined by real-time reverse transcription polymerase chain reaction (real-time RT-PCR) method. Results: Immunohistochemical results showed that the expression rates of CXCL12 and CXCR4 in T-LBL/ALL were 84.7%(61/72) and 91.6%(66/72), respectively, and these were not different from the expression in the LH control group. The expression indexes of Ki-67 <80% and ≥80% were 25 cases (34.7%, 25/72) and 47 cases (65.3%, 47/72), respectively. Real-time quantitative PCR demonstrated that CXCL12 and CXCR4 mRNA expression in T-LBL/ALL was 62.4% and 71.5%, respectively, and was statistically different (P<0.05) from that of the control group. Single factor analysis found that CXCL12 mRNA expression in T-LBL/ALL was positively correlated with Ann Arbor staging and KPS score (P<0.05); CXCL12 protein expression was positively correlated with splenomegaly (P<0.05); CXCR4 mRNA expression was positively correlated with the IPI score, clinical symptoms, mediastinal widening and bone marrow involvement (P<0.05); CXCR4 protein expression was positively correlated with mediastinal widening (P<0.05); CXCL12 mRNA expression was positively correlated with CXCL12 protein and CXCR4 protein expression (P<0.05), but not the CXCR4 mRNA and protein levels. There was no correlation between CXCL12 and CXCR4 protein expression and CXCR4 mRNA expression. Multivariate COX regression analysis showed that high expression of CXCR4 protein, hepatosplenomegaly and bone marrow involvement were risk factors for T-LBL/ALL outcome. Conclusions: CXCL12/CXCR4 expression is associated with disease progress, mediastinal widening, bone marrow involvement and adverse outcome in T-LBL/ALL. CXCL12/CXCR4 axis plays an essential role in the occurrence and development of T-LBL/ALL. However, CXCL12 and CXCR4 protein expression are not entirely reflected by mRNA transcription levels, and there may be other molecules involved in CXCL12/CXCR4 expression and regulation. With CXCR4 antagonists undergoing clinical trials, targeting the CXCL12/CXCR4 axis may be a promising treatment strategy for T-LBL/ALL.

A Young Man with an Abnormal Chest X-ray

Gastrointestinal stromal tumors(GIST) are an uncommon neoplasms of the GI tract. We present a rare case of esophageal GIST in a previously healthy young male. A 30 year old male presented to the ED after waking up ith cough, eigastric pain, nausea and vomiting. A chest x- ray showed upper mediastinal widening. Contrast enhanced CT scan incshowed a posterior mediastinal mass. Transbronchial needle aspiration and biopsy of a subcarinal mass was performed which revealed necrotic tissuecand was not diagnostic. Repeat biopsy with right video assisted thoracic surgery was performed. Immunohistochemistry was positive for CD117. A repeat chest x-ray 3 months later revealed resolution of previously described mass. He remained well until 10 months after initial presentation when he presented with chest pain, back pain and dry cough. A chest CT revealed a recurrent 5 cm subcarinal density. Right thoracotomy and resection of the mass was performed. Pathology results showed GIST with high mitotic rate. Patient was started on Imatinib. Endoscopic ultrasound two months later showed a residual mass. Fine needle aspiration and biopsy was positive for GIST. He underwent esophagectomy. Histologic evaluation revealed high grade epithelioid GIST. Patient has been asymptomatic without recurrence since.Figure 1Figure 2Figure 3GISTs affect mainly older individuals. It occurs mainly in stomach and express KIT or cell surface marker cd117 in 85-90% of cases, which is a tyrosine kinase receptor. Initial patient evaluation is usually with CT scan. Radical surgery is the treatment of choice in primary resectable GISTs. Almost all are associated with a risk of recurrence with rate of 40-50%. Imatinib Mesylate, inhibitor of KIT Tyrosine Kinase is used as an adjuvant therapy to decrease the risk of post operative recurrence. High risk tumors have a risk of 66% recurrence, making it obvious that surgery alone is not sufficient to provide long term survival. It is also used as neoadjuvant in cases that complete surgical resection is impossible and also for reducing tumor size. Since patients with high risk GIST relapse within 2 years, the follow up should be adopted to tumor criteria. CTS in the technique of choice for follow up. Our patient is particularly unique because of the age of presentation and location of tumor. Immunohistochemistry for CD117 has a high value of specificity for GIST. Treatment is complete resection. Recurrence is mainly dependent on mitotic activity. Imatinib is a useful adjuvant.

Aortic aneurysm and mitral regurgitation as first manifestat ions of ankylosing spondilitis

Ankylosing Spondilitis is a systemic inflammatory disease that involves the whole spine and sacroiliac joints. Cardiac manifestations include mitral regurgitation, aortic regurgitation, conduction abnormalities, atrial fibrillation, myocardial dysfunction and pericarditis. A 72 year-old male referring to our department because of dyspnea and mediastinal widening at chest radiography and having a diagnosis of aortic aneurysm and mitral regurgitation is presented as a case report.

Young patient with generalized lymphangiomatosis: Differentiating the differential.

We present the case of a 19-year-old man who was extensively evaluated in multiple centres for long-standing cough, dyspnea, and hemoptysis without a definitive diagnosis. His chest radiograph at presentation showed mediastinal widening, bilateral pleural effusions, and Kerley B lines. Computed tomography of the thorax showed a confluent, fluid-density mediastinal lesion enveloping the mediastinal viscera without any mass effect. There were bilateral pleural effusions, prominent peribronchovascular interstitial thickening, interlobular septal thickening and lobular areas of ground glass density with relative sparing of apices. There were a few dilated retroperitoneal lymphatics and well-defined lytic lesions in the bones. In this case report, we aim to systematically discuss the relevant differentials and arrive at a diagnosis. We also briefly discuss the treatment options and prognosis along with our patient's course in the hospital and final outcome.

Rapid Identification of Type A Aortic Dissection as a Cause of Acute Ischemic Stroke

Patients with acute aortic dissection (AAD) sometimes present predominantly with neurological symptoms from cerebral ischemia. Such stroke patients must not receive thrombolysis therapy, which can be fatal. However, patients remain at risk if there is a failure to notice concurrent AAD. We aimed to clarify the characteristics of AAD patients with stroke to identify markers for early AAD detection before thrombolysis. Using the single-center database of Stanford type A-AAD patients between 2007 and 2013, we selected those presenting with acute focal neurological deficits, presumably due to cerebral ischemia. Results of physical, radiological, and blood examinations were assessed in AAD patients with stroke. Of 226 AAD patients, 23 (10%) had stroke secondary to AAD. Of the 23 patients, 21 (91%) were primarily examined by stroke physicians and 2 (9%) by cardiologists. Thirteen patients (57%) were potential candidates for intravenous thrombolysis. Only 11 patients (48%) complained of chest/back pain. Positive findings indicating AAD included occlusion or intimal flap of the common carotid artery on carotid ultrasound in 18 (90%) of 20 patients, elevated serum d-dimer values (≥6.9 µg/mL) in 18 (78%) of 23, left hemiparesis as a neurological symptom in 17 (74%) of 23, systolic blood pressure differential above 20 mmHg between the arms in 15 (71%) of 21 patients, and mediastinal widening on chest radiograph in 10 (67%) of 15 patients. All 14 patients who underwent complete evaluation showed 2 or more positive diagnostic findings. The combination of physical, radiological, and laboratory findings may be a useful rapid-screening method for AAD as a cause of acute ischemic stroke.

Normalization of mediastinal widening after successful treatment of mediastinal tuberculosis

Clinical image of an asymmetrical mediastinal widening due to tuberculosis of mediastinal lymph nodes, without evidence of pulmonary tuberculosis. Image at first presentation and after successful treatment, showing normalization of the mediastinum.