Primary Pulmonary Lymphoma: Diagnosis and Follow-Up of 6 Cases and Review of an Uncommon Entity

Abstract

Aims and background

Primary pulmonary lymphoma is an uncommon disease with a poorly defined management. We reviewed and followed the cases of primary pulmonary lymphoma in our institution to gather an estimation of this entity in our population.

Design and methods

We reviewed the records of all patients with biopsy-proven lymphoma of the lung. The main diagnostic criterion for primary pulmonary lymphoma was the absence of extrapulmonary involvement.

Results

We identified 6 cases of primary pulmonary lymphoma among 33 patients with biopsy-proven lymphoma of the lung evaluated in our center in a 12-year period. A radiological abnormality in an asymptomatic patient was the most common clinical presentation. Four cases were low-grade and two cases high-grade non-Hodgkin PPL. Histopathologic analyses of lung specimens obtained by transbronchial biopsy were sufficient for a diagnosis in 5 of the 6 cases and avoided invasive surgical maneuvers. Most patients followed an indolent course, but with a tendency to relapse.

Conclusions

Although clinical management of this entity is undefined, we feel bronchoscopic study, which is less aggressive than surgery, may be an adequate procedure for a diagnosis. Mono-chemotherapy using alkylating agents and careful clinical observation may be the best therapeutic approach for these patients, since most of them have favorable outcomes, whatever the treatment modalities.