Clinical Analysis of 50 Cases of Primary Pulmonary Lymphoma: A Retrospective Study and Literature Review

Abstract

Objective: This study aimed at to raise the awareness understanding of primary pulmonary lymphoma (PPL) by analyzing the clinical manifestation, imaging, pathology, diagnosis, treatment, and prognostic features of 50 cases of PPL. Methods: The study of 50 individuals with PPL diagnosed at the First affiliated hospital of Nanchang university between January 2009 and December 2019 was performed. Results: Overall, 27 males and 23 females were enrolled, with an average age of 57.6 ± 15.6 years. The primary symptoms included, cough (n = 37), expectoration (n = 25), sputum with blood (n = 12), and chest pain (n = 12). Two individuals had Hodgkin's lymphoma and 48 patients had non-Hodgkin's lymphoma (NHL). We divided the NHL cases into mucosa-associated lymphoid tissue lymphoma (MALT) (n = 21), diffuse large B-cell lymphoma (n = 12), small lymphocytic lymphoma (n = 2), mantle B-cell lymphoma (n = 2), follicular lymphoma (n = 1), B-cell lymphoma without further classification (n = 8), and T-cell lymphoma (n = 2). The imaging findings revealed that unilateral lung involvement was more common among the patients. The longest follow-up duration up to December 2019 was 123 months with 40 surviving patients. The 5-year overall survival and progression-free survival were 46.7% and 44.4%, respectively. Age was an independent predictive factor for the 5-year survival (hazard ratio, 8.900; P = .038), (P < .05). Conclusion: PPL is a uncommon disease with atypical clinical manifestations and is often misdiagnosed. Immunohistochemistry is currently the standard used in pathologic evaluation of PPL. MALT prognosis is better in contrast with other kinds of PPL. Surgery or radiotherapy can be considered in patients with limited lesions, and chemotherapy is the first treatment option for diffuse lesions. Age of ≥ 60 years was reported as an independent adverse predictive factor.

Keywords: clinical characteristic; literature review; pathological characteristic; primary pulmonary lymphoma; retrospective study.

Figure 1.

Representative CT images of the patient lungs. (A) In the middle and upper lobe of the right lung, there is a patch under the anterior pleura, which shows the air bearing sign of the bronchus. (B) Diffuse nodules and mass shadows in both lungs. (C) Soft tissue mass shadow is seen in the middle and lower lobe of the right lung, measuring approximately 4.5 × 6.1 cm. (E) The density of the right upper lobe of the lung was increased. (F) The space-occupying the lesion in the lower left lung, measuring approximately 6 × 5 cm in size, accompanied by pleura stretch.

Figure 2.

Primary pulmonary HL. HE staining shows the lymph nodes exhibit a relatively preserved nodal architecture with diffuse infiltration of small- or medium-sized lymphocytes with mild nuclear irregularity. Small aggregated foci or clusters of a few giant cells with irregularly lobulated, highly convoluted Reed-Sternberg or Hodgkin's cell-like nuclei are scattered throughout the expanded paracortex (A) (40 × ). Immunohistochemical staining shows positive for CD3 (B), CD15 (weak positive) (C), CD30 (D), and CD79a (E), negative for CD20 (F) (40 × ).

Figure 3.

Primary pulmonary MALT. HE staining shows a dense lymphoid proliferation with relatively well circumscribed border (bottom) extending into adjacent lung parenchyma in a lymphangitic pattern of spread (A) (40 × ). Immunohistochemical staining shows diffuse positive for CD20 (B) and CD79a (C) (40 × ), negative for CD3 (D), EBER (E), and low Ki-67 (<10%) (F) (40 × ).

Figure 4.

Primary pulmonary DLBCL. HE staining shows confluents sheets of large atypical lymphoid cells, at least 2 times larger than a “mature” lymphocyte, with coarse nuclear chromatin, prominent nucleoli, and a rim of amphophilic to slightly eosinophilic cytoplasm (A) (40 × ). Immunohistochemical staining shows diffuse positive for CD20 (B), CD79a (C) and high Ki-67 (60%-90%) (D), negative for CD3 (E) and EBER (F). (40 × ).

Figure 5.

Primary pulmonary small B-cell lymphomas. HE staining shows SLL consists of a monotonous population of small lymphocytes with round nucleus and coarsely granular chromatin (A) (40 × ), immunohistochemical staining shows positive for CD20 (B) and CD5 (C), negative for cyclinD1 (D) (40 × ). FL is composed of centrocytes and only a few centroblasts, <5/HPF. Lymphoid tissue hyperplasia, follicles are formed in different sizes, tumor boundary is not clear and infiltrating growth to lung tissue (E) (40 × ). Immunohistochemical staining shows positive for CD20 (F) and Bcl-2 (G) and CD23 (follicular dendritic reticulum was positive) (H) (40 × ). MCL consists of small- to medium-sized lymphoid elements with irregular nuclear contours, somewhat dispersed chromatin, nuclei are round to ovoid and moderately hyperchromatic (I) (40 × ). Immunohistochemical staining shows positive for CD20 (J) and CD5 (K) and CyclinD1 (L) (40 × ).

Figure 6.

Primary pulmonary lymphoma of peripheral T-cell. HE staining shows the tumor was necrotic in the center, with dense peripheral cells, medium-sized cells, irregular nuclei and homogeneous chromatin (A) (40 × ). Immunohistochemical staining shows positive for CD3 (B) and CD4 (C), Ki-67 (65% + ) (D), negative for CD8 (E) and CD20 (F) (40 × ).

Figure 7.

Overall survival (OS) curve and progression-free survival (PFS) curve.