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Requests for reprints should be addressed to Julien De Greef, MD, Department of Internal Medicine, Saint-Luc University Hospital, Avenue Hippocrate 10, Brussels 1200, Belgium.
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.
Figure(A) Ventilation/perfusion scan of the lungs showing multiple bilateral isolated perfusion
defects at the subsegmental level. (B) Tumoral cells shown by buffy coat smear examination of pulmonary capillar blood
sampled during right heart catheterization.
