Diffuse large B-cell lymphoma (DLBCL) of the lung

Definition

A diffuse large B-cell lymphoma that is localised to the lung at the time of presentation.

Epidemiology

DLBCL constitutes 5-20% of primary pulmonary lymphomas. Most patients are older adults.

Clinical features

DLBCL may occur in association with collagen vascular disease, fibrosing alveolitis2, immunosuppressed transplant patients, in association with AIDS or other immunodeficiency states. Patients present with cough, haemoptysis, dyspnea or systemic "B" symptoms.

Radiology

Masses are solid and usually multiple.

Macroscopic appearances

Histopathology

As for DLBCL at other sites. Some cases show areas of low-grade MALT lymphoma1.

Immunohistochemistry

As for DLBCL at other sites.

Differential diagnosis

Primary mediastinal B-cell lymphoma extending into the lung: often occurs in young women.
Lymphomatoid granulomatosis (LG): both may show angiocentricity and necrosis. In LG, the T-cell component is much more marked and the atypical B-cells are positive for Epstein-Barr virus.
Undifferentiated carcinoma
Anaplastic large cell lymphoma
Germ cell tumours

Management

Chemotherapy.

Prognosis

Five year survival is up to 60%.

References

0 Tumours of the Lung, Pleura, Thymus and Heart. WHO Classification of Tumours. IARC Press 2004.

1 Fiche M, Caprons F, Berger F, et al. Primary pulmonary non-Hodgkin's lymphomas. Histopathology 1995; 26:529-37

2 Nicholson AG, Wotherspoon AC, Jones AL, et al. Pulmonary B-cell non-Hodgkin's lymphoma associated with autoimmune disorders: a clinicopathological review of six cases. Eur Respir J 1996; 9:2022-5

This page last revised 21.6.2005.