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PMLBCL : Epidemiology & Sites

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Epidemiology: Primary Mediastinal Large B-cell Lymphoma (PMLBCL) accounts for 2-3% of non-Hodgkin lymphomas and about 6% of diffuse large B-cell lymphomas. It occurs mainly in young adults (median age 35 years) with a predilection for females (F:M=2:1). Some cases occur in children and adolescents.

Sites: Most patients present with a rapidly growing bulky mass (> 10 cm size in 75% of cases) localized in the anterosuperior mediastinum. The tumor frequently invades surrounding thoracic structures such as sternum, chest wall, lungs, pleura, and pericardium. Supraclavicular and cervical lymph nodes may be involved. In advanced stages, the lymphoma spreads to extranodal sites such as CNS, liver, kidneys, and adrenals. Bone marrow involvement or leukemic phase are rare (2%) or absent. Rare cases of PMLBCL are extramediastinal with no mediastinal involvement.

This plain radiograph of chest shows an expanded cardiomediastinal contour due to a large superior mediastinal mass. Diagnosis of PMLBCL was confirmed by a CT-guided needle biopsy of the mass (same case as the previous image). Case courtesy of Assoc Prof Frank Gaillard, Radiopaedia.org. From the case rID: 8655

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