A 78-year-old man was admitted because of rapidly worsening dyspnea and moderate hypoxemia. He had a past history of scrotal Paget disease treated by radiotherapy 1 year prior to admission. On examination, the jugular veins were distended, with a positive abdominal jugular reflux. Laboratory work-up demonstrated elevated D-dimer level at 14,067 ng/mL (normal ≤500 ng/mL) and lactate dehydrogenase level at 496 UI/L (normal <250). No evidence of pulmonary embolism or parenchymal abnormality was detected by pulmonary computed tomography (CT) angiogram. Pulmonary hypertension was confirmed by transthoracic echocardiography. A right heart catheterization showed precapillary pulmonary arterial hypertension (mean pulmonary arterial pressure of 32 mm Hg and pulmonary wedge pressure of 10 mm Hg). Due to the lack of explanatory abnormality on pulmonary CT, a ventilation/perfusion lung scan was ordered, showing multiple isolated subsegmental perfusion defects (Figure, A). Due to recent history of cancer, tumor pulmonary microembolism was suspected. During right heart catheterization, pulmonary capillary blood was sampled and analyzed for buffy coat smear and pathological examination. Both demonstrated free tumor cells (Figure, B). Abdominal CT further demonstrated multiple enlarged lymph nodes and hepatic and bone metastases. The pathological examination of an inguinal adenopathy eventually confirmed the suspected relapse of extramammary Paget disease. Chemotherapy (carboplatin-paclitaxel) in combination with anticoagulation allowed a quick improvement of dyspnea. At 4 months of follow-up, the patient was considered in complete remission.
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Published online: March 26, 2018
Conflicts of Interest: None.
Authorship: All authors had access to the data and contributed to the writing of the manuscript.
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