Apical lung location of lymphomas is extremely rare and may pose diagnostic problems. Here we present a case of advanced primary Pancoast-like left apical lung lymphoma incidentally diagnosed in a 72 year old asymptomatic woman after a routine, prophylactic chest X-ray performed in June 2005. FNB was not diagnostic therefore an open lung biopsy was attempted. Histopathological examination of the excised specimen was consistent with B-cell low-grade marginal zone extranodal NHL (BALTL); CD20+, CD3-. Treatment combined of 9 cycles of chemotherapy followed by radiotherapy of the residual mass allowed to achieve a long-term complete remission.
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