title: A retrospective international study on primary extranodal marginal zone lymphoma of the lung (BALT lymphoma) on behalf of International Extranodal Lymphoma Study Group (IELSG).
abstract: Primary lymphoma of the lung is a rare entity. Clinical features, optimal treatment, role of surgery and outcomes are not well defined, and the follow-up is variable in published data. Clinical data of 205 patients who were confirmed to have bronchus mucosa-associated lymphoid tissue lymphoma from December 1986 to December 2011 in 17 different centres worldwide were evaluated. Fifty-five per cent of the patients were female. The median age at diagnosis was 62 (range 28-88) years. Only 9% had a history of exposure to toxic substances, while about 45% of the patients had a history of smoking. Ten per cent of the patients had autoimmune disease at presentation, and 19% patients had a reported preexisting lung disease. Treatment modalities included surgery alone in 63 patients (30%), radiotherapy in 3 (2%), antibiotics in 1 (1%) and systemic treatment in 128 (62%). Patients receiving a local approach, mainly surgical resection, experienced significantly improved progression-free survival (p = 0.003) versus those receiving a systemic treatment. There were no other significant differences among treatment modalities. The survival data confirm the indolent nature of the disease. Local therapy (surgery or radiotherapy) results in long-term disease-free survival for patients with localized disease. Systemic treatment, including alkylating-containing regimens, can be reserved to patients in relapse after incomplete surgical excision or for patients with advanced disease. Copyright © 2015 John Wiley & Sons, Ltd.
authors.affiliation: Haematoncology Division, European Institute of Oncology, Milan, Italy.
authors.forename: Simona
authors.initials: S
authors.lastname: Sammassimo
authors.affiliation: Pathology Division, European Institute of Oncology, Milan, Italy.
authors.forename: Giancarlo
authors.initials: G
authors.lastname: Pruneri
authors.affiliation: Haematoncology Division, European Institute of Oncology, Milan, Italy.
authors.forename: Giovanna
authors.initials: G
authors.lastname: Andreola
authors.affiliation: Haematoncology Division, European Institute of Oncology, Milan, Italy.
authors.forename: Juan
authors.initials: J
authors.lastname: Montoro
authors.affiliation: Haematoncology Division, European Institute of Oncology, Milan, Italy.
authors.forename: Sara
authors.initials: S
authors.lastname: Steffanoni
authors.affiliation: Mayo Clinic, Rochester, MN, USA.
authors.forename: Grzegorz S
authors.initials: GS
authors.lastname: Nowakowski
authors.affiliation: Epidemiology and Biostatistics Department, European Institute of Oncology, Milan, Italy.
authors.forename: Sara
authors.initials: S
authors.lastname: Gandini
authors.affiliation: Haematoncology Division, European Institute of Oncology, Milan, Italy.
authors.forename: Mara
authors.initials: M
authors.lastname: Negri
authors.affiliation: Mayo Clinic, Rochester, MN, USA.
authors.forename: Thomas M
authors.initials: TM
authors.lastname: Habermann
authors.affiliation: Medical University, Vienna, Austria.
authors.forename: Markus
authors.initials: M
authors.lastname: Raderer
authors.affiliation: Sun Yat-Sen University Cancer Center, Guangzhou, China.
authors.forename: Zhi-Ming
authors.initials: ZM
authors.lastname: Li
authors.affiliation: Policlinico Sant'Orsola, Bologna, Italy.
authors.forename: Pier Luigi
authors.initials: PL
authors.lastname: Zinzani
authors.affiliation: Pathologisches Institut der Universität, Würzburg, Germany.
authors.forename: Patrick
authors.initials: P
authors.lastname: Adam
authors.affiliation: IOSI, Bellinzona, Switzerland.
authors.forename: Emanuele
authors.initials: E
authors.lastname: Zucca
authors.affiliation: Haematoncology Division, European Institute of Oncology, Milan, Italy.
authors.forename: Giovanni
authors.initials: G
authors.lastname: Martinelli
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D018572:Disease-Free Survival; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D008175:Lung Neoplasms / Q000401:mortality* / Q000628:therapy*; D018442:Lymphoma, B-Cell, Marginal Zone / Q000401:mortality* / Q000628:therapy*; D008297:Male; D008875:Middle Aged; D012189:Retrospective Studies; D015996:Survival Rate
keywords: Achromobacter (Alcaligenes) xylosoxidans; BALT lymphoma; marginal zone lymphoma