Neuroblastoma in southern Brazil: an 11-year study

The authors report on the incidence and clinical characteristics of neuroblastoma in southern Brazil. The aims of the study were to evaluate the age at diagnosis, tumor stage, MYCN status, and tumor histopathology, and to relate these factors to survival. All patients with neuroblastoma, 15 years old or younger (n = 125), admitted to the three major pediatric oncology hospitals in the state of Parana over a period of 11 years (between January 1990 and December 2000), were included in the analysis. All patients were followed for at least 5 years. In addition, a FISH evaluation for MYCN status was conducted in a subset of 34 tumors. Overall survival for tumor stages 1, 2, 3, and 4 was 100%, 72%, 59%, and 17%, respectively. Sixty-two percent (77/125) of all patients were older than 2 years; these represented 71% (57/80) of the patients with stage 4 disease. Children who presented with an unfavorable histopathology had a significantly worse prognosis (20% survival) than children with a favorable histopathology (67% survival). MYCN amplification was detected most commonly in stages 3 and 4 tumors (13/16). These data showed a delayed diagnosis of neuroblastoma in children in southern Brazil, and consequently survival was considerably lower in these patients.     

Myasthenia gravis developing in a patient with CNS lymphoma

A patient was initially diagnosed with right basal ganglia lymphoma causing left hemiparesis. His disease was resistant to intravenous methotrexate, so he received radiation therapy with remarkable regression of the mass. However, 6 months after his initial diagnosis, he developed symmetric weakness of the proximal muscles. Electromyography was consistent with myasthenia gravis and anti-acetylcholine receptor antibodies were elevated. Treatment with pyridostigmine and corticosteroids improved his symptoms. The residual lymphoma was further treated with rituximab and temozolomide with complete resolution. Pyridostigmine and corticosteroids were stopped after 14 months of initiation with good results. Our case is the first case report of paraneoplastic myasthenia gravis developing in a patient with primary CNS lymphoma. The diagnosis of paraneoplastic myasthenia should be considered in patients with CNS lymphoma who develop muscle weakness and speech problems.     

Tricuspid Annular Velocity Assessed by Doppler Tissue Imaging as a Marker of Right Ventricular Involvement in the Acute and Late Phase after a First ST Elevation Myocardial Infarction

Background: Right ventricular (RV) involvement in patients with ST elevation myocardial infarction (STEMI) is a clinically important problem. The aim of this study was to evaluate the use of tricuspid annular velocity assessed by Doppler tissue imaging (DTI) as a marker of RV involvement in patients with a first STEMI. Methods: Seventy‐one patients with a first STEMI were examined by echocardiography before discharge and after 6 months. The patients were compared to 31 healthy subjects (HS). The diagnosis of RV infarction was based on the presence of ≥ 1 mm ST elevation in the right precordial ECG lead V4R. Tricuspid annular systolic and diastolic velocities using pulsed‐wave DTI were recorded at the RV free wall. Results: At day 1, tricuspid annular velocities were significantly reduced in patients with, compared to those without, ST elevation in V4R (11.1 vs. 13.7 cm/sec, 9.4 vs. 13.1 cm/sec and 14.1 vs. 15.9 cm/sec for systolic, early, and late diastolic velocities, respectively; P < 0.01). With a cutoff value for a tricuspid annular systolic velocity of 13 cm/sec, sensitivity and specificity for identifying patients with ST elevation in V4R were 89% and 71%, respectively. After 6 months, both tricuspid annular systolic and diastolic velocities in patients with RV infarction had increased significantly, but only tricuspid annular systolic velocity was still reduced compared to HS (12.3 vs. 14.7 cm/sec; P < 0.01). Conclusion: Tricuspid annular velocities assessed by DTI may be used as a marker of RV involvement in the acute and the late phase after a first STEMI. (ECHOCARDIOGRAPHY 2010;27:139‐145)     

Acute arthritis revealing Hashimoto's thyroiditis

Rheumatic manifestations can reveal hypothyroidism, such as arthritis and nonspecific musculoskeletal symptoms. We report herein the case of an acute polyarthritis revealing Hashimoto''s thyroiditis (HT). Hormone replacement therapy leads to the resolution of arthritis related to HT, suggesting the role of thyroid hormone in the pathogenesis of arthritis.     

Synovial hemangioma: A rare cause of chronic knee pain

Synovial hemangioma is a rare benign vascular tumor responsible for chronic knee pain and swelling. Given its non‐specific symptoms, synovial hemangioma is often misdiagnosed. We report a case of synovial hemangioma of bilateral knee joints occurring in a young military man.