The patient was quality control admitted in September 2004 with a 2�\week history of gradually increasing shortness of breath, productive cough, fever and pleuritic chest pain, 12 months after imatinib was started. A chest x ray showed bilateral airspace opacities and a right pleural effusion (fig 11).). Investigations showed a white cell count of 2.5��109 cells/l with lymphopenia (0.2��109 cells/l). No infective agents were identified. The patient was HIV�\negative. Repeated antibiotic and antifungal treatment ultimately failed. Imatinib was stopped 10 days after admission without improvement in the clinical condition. However, after 10 days, there was gradual recovery of lymphopenia (fig 22). Figure 1Chest x ray (A) and computed tomography imaging (B) showing bilateral airspace opacities and right pleural effusion.

Figure 2Recovery of lymphocyte count. Imatinib mesylate treatment during hospital admission is indicated by black bar. The figure shows a marked improvement in the white cell count 10 days after discontinuation of imatinib. The patient’s condition continued to deteriorate and he died on the 43rd day of admission. Postmortem examination findings A consented limited, hospital postmortem examination of the heart and lungs was performed, which showed bilateral pleural effusions and partial or well�\defined pale grey/tan nodules of variable size showing central necrosis and focal cavitation (fig 33).). Lesions were present in all lobes of the right and left lungs, the largest measuring 14 cm. The heart was macroscopically normal. Figure 3Lung with multiple soft whitish nodules with focal necrosis.

Histological findings The lung showed no evidence of a metastatic GIST, and no CD117+ cell was present. However, the nodules showed large areas of necrosis, and viable tissue showed an angiocentric distribution of large CD20+ atypical lymphoid cells, and CD3+ small lymphocytes. These are the typical histological appearances of lymphomatoid granulomatosis (LYG), grade III (fig 44).). Atypical cells were shown to contain Epstein�CBarr virus (EBV; latent membrane protein and EBV�\encoded RNA positivity). Figure 4(A,B). Haematoxylin and eosin stains of lung lesions showing extensive necrosis containing large atypical cells with angiocentric distribution. (C,D). CD20 and latent membrane protein immunostaining showing positive large atypical cells. …

Review of the original small bowel lesion confirmed the diagnosis of malignant spindle cell�\type GIST. The tumour measured 11.5 cm. High mitotic count (>15 per high�\power field), foci of necrosis, haemorrhage and a strongly positive immunohistochemical Carfilzomib reaction with antibody CD117 were noted. Discussion We report on the first documented patient to be diagnosed with two rare malignant lesions of different origin, GIST and LYG. These two lesions occurred at a young age. LYG presented 2.