Abstract: Case Reports |


Alexis H. Meredith, MD*; Timothy Williamson, MD
Author and Funding Information

University of Kansas, Kansas City, KS


Chest. 2005;128(4_MeetingAbstracts):459S-a-460S. doi:10.1378/chest.128.4_MeetingAbstracts.459S-a
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INTRODUCTION:  Disseminated microvascular pulmonary tumor cell embolism is a known cause of pulmonary hypertension, however it is fairly uncommon. Nevertheless, in an older patient without a clear cause of pulmonary hypertension, it should be included in the differential diagnosis.

CASE PRESENTATION:  A previously healthy sixty-one year old male presented with a two month history of generalized fatigue and low grade fevers. While undergoing evaluation of his fatigue and fevers, he had an echocardiogram performed that showed elevated pulmonary artery pressures. Right heart catheterization was performed and demonstrated pulmonary artery pressures of 72/30 mmHg with a normal pulmonary artery occlusion pressure. Further workup revealed a positive anti-nuclear antibody, symptoms consistent with Raynaud’s disease, and a Factor VII deficiency. CT of the chest was done and was clear. Other workup including ventilation perfusion scan, pulmonary function testing, exercise and overnight oximetry, C-ANCA, P-ANCA, rheumatoid factor, and human immunodeficiency virus testing was all unremarkable. The patient’s pulmonary hypertension was attributed to an underlying connective tissue disorder, and he was started on corticosteroids. After a remarkable improvement of two World Health Organization (WHO) functional classes he was discharged home. Two weeks later he returned with complaint of increasing shortness of breath, fevers and night sweats, and cough productive of blood tinged sputum. Admission radiography showed bilateral cavitary pulmonary infiltrates. PPD was negative. Sputum culture and AFB were also negative. The patient was treated empirically with broad spectrum antibiotics with improvement in his symptoms and was again discharged home. The patient returned two weeks later with left sided chest pain and worsening dyspnea. CT chest was performed on admission and showed no change in the patient’s cavitary lesions. Flolan was initiated based on the patient’s severe pulmonary arterial hypertension and his WHO functional class IV symptoms. The patient initially improved on Flolan, but despite aggressive titration, he suffered progressive cardio-pulmonary collapse and died. Post mortem examination was performed and revealed a small pancreatic mass that was found by microscopy to be poorly differentiated pancreatic adenocarcinoma. In addition to the pancreatic mass, there were parenchymal masses seen in the liver and lung, and widespread angiolymphatic dissemination to multiple organs. Sections of the lung showed that the cavitary lesions were areas of infarction and necrosis with tumor emboli occluding many pulmonary vessels.

DISCUSSIONS:  When evaluating possible causes of pulmonary hypertension, the differential diagnosis is quite large. In this patient several things confounded the ultimate diagnosis. The patient’s positive anti-nuclear antibody in addition to his Raynaud’s symptoms led to a mistaken diagnosis of a connective tissue disease. In addition, with the cavitary lesions on chest x-ray and no history of malignancy, the patient’s constitutional symptoms were wrongly attributed to either connective tissue disease or infectious causes. Also, with the patient’s severe pulmonary hypertension and rapidly deteriorating course, invasive procedures such as lung biopsy and bronchoscopy were deemed too risky to perform. In retrospect, both the constitutional symptoms and abnormal lung findings on radiography were all attributable to the patient’s underlying pancreatic malignancy. If tumor cell embolism had been suspected, a pulmonary capillary wedge aspirate could have been used for diagnostic purposes..

CONCLUSION:  Although rare, tumor cell emboli should be included in the differential diagnosis of cryptic pulmonary hypertension.

DISCLOSURE:  Alexis Meredith, None.

Tuesday, November 1, 2005

4:15 PM - 5:45 PM




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