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A 34-year-old woman with a massive pulmonary malignant hemangiopericytoma and coagulopathy as a paraneoplastic syndrome is reported. Although coagulopathy may appear as a paraneoplastic syndrome and cause treatment to be more complicated and difficult, it can also be a useful marker to monitor the results of operation and tumor recurrence. This unusual case shows that primary pulmonary malignant hemangiopericytoma deserves aggressive surgical intervention with complete resection even under the circumstances of coagulation abnormality.
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