Lymphomatoid granulomatosis in a patient previously diagnosed with a gastrointestinal stromal tumour and treated with imatinib

A 39-year-old man with a history of recurrent malignant gastrointestinal stromal tumour (GIST) of the small bowel, treated with imatinib, presented with a 2-week history of shortness of breath, fluctuating fever, pancytopenia and bilateral airspace opacities. He was treated for pneumonia, but his condition deteriorated and he died 6 weeks after admission. We present the subsequent postmortem examination findings.

CLINICAL HISTORY

This is the case of a 39-year-old man, with a medical history of malignant gastrointestinal stromal tumour (GIST) of the small bowel, resected 2.5 years before this episode. Local recurrence and liver metastases occurred 18 months from initial diagnosis. Imatinib mesylate 400 mg once daily (Glivec; Novartis, Basel, Switzerland) was started, with a good clinical response (including a reduction in tumour size). Family history included a sister diagnosed with cancer and mother with a brain tumour (unfortunately, further information could not be obtained).

The patient was 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 1). 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 2).