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Superior vena cava syndrome in primary mediastinal B-cell lymphoma

2013; Elsevier BV; Volume: 24; Linguagem: Inglês

10.1016/j.ejim.2013.08.456

1879-0828

L. Ruzickova, Catarina Canha, L. Geraldes, Paulo Cesar, José Pedro Carda, Nuno Vasco Costa, B. Barbosa, Manuel Teixeira Veríssimo, J. Costa,

Venous Thromboembolism Diagnosis and Management

Introduction: Superior vena cava syndrome (SVCS) is a group of symptoms that occurs when vena cava superior is partly or completely blocked. Obstruction can occur due to external pressure, involvement of the vessel by tumour tissue, or thrombotic event. Most SVC syndromes are associated with malignant disease. Primary Mediastinal B-cell lymphoma is a distinctive subtype of Non-Hodgkin lymphoma with specific clinicopathologic aspects and aggressive behaviour. This malignancy is characterized by rapid growth, large bulk, and early local and extranodal extension in the absence of generalized adenopathy. Most patients demonstrate symptoms, often present for only several days, from a rapidly enlarging mediastinal mass. It is predominantly a disease of young adults, particularly women. Superior vena cava syndrome is common, as is involvement of the pericardium. Case summary: We present the case of 33-year-old, Caucasian woman with medical history of recently diagnosed hypothyroidism medicated with Levothyroxin, who presented to the Emergency Department with dyspnea, swelling and flushing of the face and neck and upper extremities which increased overnight when she lay in a horizontal position. In addition, she had developed odynophagy and low back pain and bilateral axillar pain within the course of approximately one week. Ultrasound exam shows multiple bilateral ganglionar formations along the cervical glands chains, without pathology of thyroide gland or other structures. Computed tomography was performed disclosing anterior mediastinal mass (10 × 9.5 × 4.5 cm) involving superior cava vein, causing severe obstruction on the level of the right atrium entry, small adenopathies on the periphery of the mass and small pleural effusion. Histopathological examination of the mass revealed sclerosing mediastinal B- cell lymphoma (CD3 and CD20 histochemical positivity). PET-Tomography revealed supradiapharagmatic ganglionar involvement without other hypermethabolic alterations. Initial medical management consisted of pain relief medication and corticotherapy with quick symptomatic relieve. At present, the patient is undergoing chemotherapy. Discussion: SVCS is considered an emergency, however, its presentation is rarely an acute life-threatening event. As the effectiveness of therapy depends on underlying disease, considering the definitive diagnosis may be more important than the actual care when making therapeutic decisions. Conclusion: Facial swelling and respiratory complaints are frequent symptoms in Emergency Department patients. They requires quick and complex diagnostic evaluation including differential diagnosis of SVCS. The malignant cause of SVCS should be always considered even in younger patients.