Birt-Hogg-Dubé syndrome (BHDS) is a rare autosomal dominant condition characterized by pulmonary cysts, skin lesions, recurrent spontaneous pneumothorax and renal carcinoma (1). We present a case of a woman with a remarkable family history found to have genetically proven BHDS.
Extranodal marginal zone lymphoma (MZL) of the lung is a rare pulmonary lymphoid malignancy and World Health Organization (WHO) classifies Mucosa associated lymphoid tissue (MALT) lymphoma as a subtype of B-cell MZL.
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