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Primary Pulmonary Lymphoma: An Extremely Rare Disease FREE TO VIEW

Sujith Cherian, MD; Subhraleena Das, MD; Shakuntala Mauzo, MD; Hayas Haseer Koya, MD; Dona Varghese, MD; Rahat Hussain, MD
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University of Texas Health Science Center at Houston, Houston, TX

Chest. 2013;144(4_MeetingAbstracts):605A. doi:10.1378/chest.1705149
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SESSION TITLE: Cancer Case Report Posters I

SESSION TYPE: Affiliate Case Report Poster

PRESENTED ON: Tuesday, October 29, 2013 at 01:30 PM - 02:30 PM

INTRODUCTION: Primary pulmonary lymphoma (PPL) is an extremely rare disease, defined as clonal lymphoid proliferation affecting one or more lungs in a patient with no extra pulmonary involvement at the time of diagnosis or 3 months after.

CASE PRESENTATION: A 36-year-old Hispanic nonsmoking male was referred to our institution by his primary care physician with complaints of persistent cough and chest x-ray showing right hilar mass. A chest CT scan performed confirmed the presence of the right hilar mass and revealed multiple small pulmonary nodules bilaterally (Fig 1). Review of systems was significant for weight loss of 10 pounds in the preceding 3 months. Physical examination was significant for the presence of inspiratory crackles in the right lower chest. Laboratory data did not reveal any significant abnormalities. CT guided biopsy done revealed a clonal lymphoid neoplasm invading bronchiolar epithelium with immunohistochemical evidence suggestive of a Mucosa associated lymphoid tissue (MALT) type PPL . Staging scan with position emission tomography (PET) scan revealed increased uptake in the right lung mass and other pulmonary nodules. Bone marrow biopsy was negative and diagnosis of PPL (Stage IV- B) was made. He received 6 cycles of chemotherapy with R- CHOP (rituxan, cyclophosphamide, vincristine, adriamycin and prednisone) and localized radiotherapy. Two years later, he has improved remarkably with no residual sequelae .(Fig 2)

DISCUSSION: PPL is an extremely rare neoplasm, comprising < 1% of all non-Hodgkin’s lymphoma (NHL) and 0.5 % of all primary pulmonary malignancies. Current definition of PPL involves 1) Low grade B-cell PPL- the most common 2) High grade PPL and 3) lymphomatoid granulomatosis. Low grade B-cell PPL forms almost 85-90 % of all reported cases, out of which almost all cases correspond to MALT-type NHL. Age of onset is usually around 50-60 years, although rarely cases of younger age of onset have been reported. Nearly half of reported cases of MALT-type NHL have been asymptomatic at presentation, while in the others, the presentation has been associated with non specific respiratory symptoms. Radiologically, localized or bilateral alveolar infiltrates have been the most described findings, although pleural effusion, reticulonodular opacities have been reported rarely. Prognosis is generally good, with treatment options ranging from surgical resection in localized cases to chemotherapy in more diffuse involvement.

CONCLUSIONS: Our case serves as an apt reminder of a rare pulmonary neoplasm with a favorable prognosis, thus reiterating the need for tissue diagnosis when the battle seems lost!!

Reference #1: Cadranel J, Wislez M, Antoine M. Primary pulmonary lymphoma. Eur Respir J. 2002 Sep;20(3):750-62. Review

DISCLOSURE: The following authors have nothing to disclose: Sujith Cherian, Subhraleena Das, Shakuntala Mauzo, Hayas Haseer Koya, Dona Varghese, Rahat Hussain

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