Primary Pulmonary Lymphoma Presenting with Superior Vena Cava Syndrome in a Young Female

Abstract

Primary Pulmonary Diffuse Large B Cell Lymphoma (PPDLBCL) is an extremely rare entity, which exhibits an aggressive behavior by compressing local blood vessels. It represents only 0.04% of all lymphoma cases and is extremely rare in young age. We present a case of a primary pulmonary lymphoma with superior vena cava syndrome (SVCS) in a young female. 27-year-old African American female presented with fever, cough, and facial puffiness for 2 weeks and unintentional weight loss. Chest examination showed decreased breath sounds and dullness on percussion on right side. Labs were normal except for mild leukocytosis, high lactate, and lactate dehydrogenase. Chest X-ray showed a large right side infiltrate with pleural effusion but chest CT showed 10 × 14 × 16 cm mass in the right lung without hilar and mediastinal lymphadenopathy. CT guided biopsy of the right lung mass was done and large B cell lymphoma was diagnosed. She received “involved field radiation” because of the bulky tumor size and superior vena cava involvement prior to R-CHOP to which she responded well. PPDLBCL should be considered as one of the differentials in a young patient with a large lung mass, which needs timely diagnosis and management.

<p>Primary Pulmonary Diffuse Large B Cell Lymphoma Mimicking Metastasis: A Case Report and Literature Review</p>

“…19 However, in another young female with PPDLBCL who presented with SVC syndrome, involved field radiotherapy was initiated to rapidly relieve symptoms, followed by R-CHOP chemotherapy. 2 As an aggressive subtype, PPDLBCL has a poorer prognosis compared with lung MALT lymphoma, intensive chemotherapy is essential especially for those who refused surgery just like our patient.…”

“…1 With regard to the aggressive subset termed primary pulmonary diffuse large B cell lymphoma (PPDLBCL), we have little information about its biological characteristics, optimized therapeutic protocols, and outcomes since less than 40 cases have been reported. [1][2][3][4][5][6][7][8][9][10][11][12][13][14][15][16][17][18][19] Here, we present an instructive PPDLBCL case mimicking lung metastasis in a heavily treated breast cancer patient. In view of the patient's nonspecific discomfort, isolated subpleural nodule, and definite history of metastatic breast cancer, it could easily have been misdiagnosed as lung metastasis.…”

Primary pulmonary diffuse large B-cell lymphoma with multiple ground-glass nodules as the primary manifestation

“…[ 8 ] Among them, DLBCL constitutes 1% of the cases of primary lymphoma of the lung, accounting for 0.4% of all the lymphomas. [ 9 ] The clinical features of PPL are not specific, and lung DLBCL can be seen in immunodeficient patients having a vascular collagen disease with or without potential pulmonary fibrosis, patients having AIDS, and patients taking cyclosporine after transplantation. Although the mechanisms of the increased incidence of lymphoproliferative diseases in immunodeficient patients are not fully understood, deterioration in immunoregulation, chronic antigenic stimulation, and tumor suppressor system dysregulation are thought to be the main reasons for it.…”

Pleural effusion as an initial manifestation in a patient with primary pulmonary monoclonal B-cell lymphocyte proliferative disease

“…Extrapulmonary diseases were also excluded; therefore, we concluded that primary pulmonary lymphoma (PPL) was likely. PPL is an extremely rare form of lymphoma with an incidence of 0.4% [2]; for the sake of making a diagnosis of PPL, extrapulmonary diseases must be ruled out [3]. Approximately half of PPL cases have no symptoms or lack specific respiratory manifestations, such as cough, dyspnea, chest pain and so on [4].…”