CASE REPORT Gen Thorac Cardiovasc Surg (2011) 59:371–375 DOI 10.1007/s11748-010-0671-2 Pulmonary mucosa-associated lymphoid tissue lymphoma with a pulmonary arteriovenous fistula Kazuhiko Shien, MD ⋅ Motohiro Yamashita, MD Mikio Okazaki, MD ⋅ Hiroshi Suehisa, MD Shigeki Sawada, MD ⋅ Akira Kurita, MD Abstract We report an extremely rare case of pulmonary mucosa-associated lymphoid tissue (MALT) lymphoma with a pulmonary arteriovenous fistula (PAVF). A 60-year-old woman with vulvar carcinoma was admitted to our hospital for further examination of an abnormal shadow on chest computed tomography (CT). She showed hypoxemia in the arterial blood gas analysis. 18 F-Fluorodeoxyglucose positron emission tomography (FDG-PET) showed consolidations in the left lower lobe and soft-tissue density lesions in the anterior mediasti- num. Each lesion showed heterogeneous FDG uptake. Although needle biopsy of these lesions was performed, a pathological diagnosis was not obtained. For the eval- uation of hypoxemia, chest contrast-enhanced CT was performed, and a PAVF in the consolidation of the left lower lobe was revealed. For diagnostic and therapeutic purposes, we performed left lower lobectomy under video-assisted thoracoscopic surgery. In the surgical specimen the PAVF measured 3 cm, and histopathologi- cal examination revealed pulmonary MALT lymphoma adjacent to the PAVF. Key words Pulmonary arteriovenous fistula ⋅ Mucosa-associated lymphoid tissue lymphoma ⋅ Positron emission tomography Received: 7 April 2010 / Accepted: 22 June 2010 © The Japanese Association for Thoracic Surgery 2011 Introduction Pulmonary arteriovenous fistula (PAVF), a rare vascu- lar abnormality, is an abnormal connection between the pulmonary artery and vein. It is seen in 2–3 per 100 000 population. 1 On the other hand, pulmonary malignant lymphomas comprise <0.5% of all pulmonary tumors, and most pulmonary lymphomas are composed of mucosa-associated lymphoid tissue (MALT) lym- phoma. 2 We report an extremely rare case of a PAVF adjacent to a pulmonary MALT lymphoma. Case report A 60-year-old woman with vulvar carcinoma was admit- ted to our hospital for further examination of an abnor- mal shadow on chest CT. At the time of admission to our hospital, she was asymptomatic, but her saturation of pulse oximetry oxygen (SpO 2 ) was 91% at room air. Hematological examinations, including tumor markers such as carcinoembryonic antigen (CEA), cytokeratin fragment (CYFRA), and pro-gastrin-releasing peptide (ProGRP), were within their normal ranges. Arterial blood gas analysis showed hypoxemia, with the arterial oxygen pressure (PaO 2 ) 52 mmHg at room air. Chest radiography showed an unclear shadow in the right lung hilum and left lower lung field (Fig. 1). 18 F-Fluorodeoxyglucose positron emission tomography (FDG-PET) revealed consolidations in the left lower lobe (Fig. 2A), and soft tissue density lesions in the anterior mediastinum (Fig. 2B). Each lesion showed het- erogeneous FDG uptake (Fig. 2C). The standardized uptake values (SUVs) for the pulmonary lesions were 6.04 and for the mediastinal lesion 9.93. Although needle K. Shien (*) ⋅ M. Yamashita ⋅ M. Okazaki ⋅ H. Suehisa ⋅ S. Sawada Department of Thoracic Surgery, National Hospital Organization Shikoku Cancer Center, 160 Minami-Umemoto, Matsuyama 791-0280, Japan Tel. +81-89-999-1111; Fax +81-89-999-1186 e-mail: kshien-ths@umin.ac.jp A. Kurita Department of Surgery, National Hospital Organization Shikoku Cancer Center, Matsuyama, Japan