SESSION TITLE: Bronchology/Interventional Global Case Reports
SESSION TYPE: Global Case Report
PRESENTED ON: Tuesday, October 28, 2014 at 01:30 PM - 02:30 PM
INTRODUCTION: Malignant lymphoma may occur from autoimmune disorders such as chronic thyroiditis and Sjogren’s syndrome and a long-term inflammatory condition such as chronic tuberculous empyema. Peripheral T cell lymphoma (PTCL) is one of the subtypes of T cell lymphoma (TCL); it comprises a heterogeneous group of nodal and extranodal mature TCLs. Primary pulmonary lymphoma (PPL) is a distinct entity in which the lymphoma arises de novo in lung tissue. The majority of PPL are a MALT lymphoma (low-grade lymphoma of B-cell) and TCL is rare. We present unusual case of primary endobronchial PTCL not otherwise specified (NOS).
CASE PRESENTATION: A 81-year-old man was referred to our hospital because of cough and wheeze. He was a smoker and had a history of pulmonary tuberculosis at 17 years old. A chest X-ray showed hypolucency of the right middle lung field. CT images demonstrated chronic pyothorax with calcified foci in the right and a mass inside the broncus intermedius and no mediastinal adenopathy. Bronchoscopic examination identified an endobronchial tumor obstructing the bronchus intermedius. The biopsy specimen of the tumor revealed an infiltration composed predominantly of small atypical lymphocytes. Immunohistochemical analyses demonstrated that the proliferating cells were positive for CD3, CD4, and CD5 and negative for CD8 and CD20. The Ki-67 labeling index was low. Based on these findings, the diagnosis was confirmed as PTCL. Positron emission tomography (PET) with [18F] fluoro-2-deoxyglucose and CT revealed uptake at the endobronchial tumor, with a maximum standardized uptake value of 20.6. There was no evidence of abnormal uptake at the pyothorax, mediastinal lymph node, or other extrathoracic organs. He was treated with systemic chemotherapy consisted of pirarubicin, cyclophosphamide, vincristine, and prednisone and the endobronchial tumor diminished markedly.
DISCUSSION: PPL is a rare condition that comprises only 3-4% of extranodal non-Hodgkin lymphomas (NHL), less than 1% of all NHLs, and 0.5-1% of primary pulmonary malignancies. The majority of the reported cases have been of the B cell type, so-called mucosa-associated or bronchus-associated lymphoid tissue (MALT or BALT, respectively) lymphomas. TCL is rare. The current case was complicated by chronic pyothorax. Pyothorax-associated lymphoma (PAL) is a category in which a lymphoproliferative disorder develops in the pleural cavity after a long-standing history of pyothorax. In our case, PET/CT revealed a hypermetabolic mass at the endobronchial tumor, but there was no abnormal uptake at the pyothorax. Endoscopic examination showed a smooth-surfaced, round tumor with a stalk; these features do not correspond to an outer parietal invasion. We think that the lymphoma in the current case did not arise from the pyothorax, although the chronic inflammation could have contributed to the development of the disease. There is no consensus regarding treatment for primary pulmonary PTCL, and anthracycline-containing combination chemotherapies such as CHOP (cyclophosphamide, doxorubicin, vincristine, and prednisone) or EPOCH (etoposide added to CHOP) are usually administered.
CONCLUSIONS: We reported the rare manifestation of PTCL-NOS, which was confined to the bronchus. PTCL-NOS should be considered in the differential diagnosis of endobronchial tumor.
Reference #1: Cordier JF, Chailleux E, Lauque D, et al. Primary pulmonary lymphomas. A clinical study of 70 cases in nonimmunocompromised patients. Chest. 1993;103:201-208.
DISCLOSURE: The following authors have nothing to disclose: Yasuko Fuchimoto, Shinsaku Matsumoto, Michiko Asano, Naofumi Hara, Yosuke Miyamoto, Nobukazu Fujimoto, Kenichi Kitamura, Shinji Ozaki, Koji Taguchi, Takumi Kishimoto
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