Lung Pathology: Chest Tumors |

Diarrhea as Initial Manifestation of Pulmonary Artery Intimal Sarcoma: A Case Report and Literature Review FREE TO VIEW

Xiaoling Xu, MD; Ruifeng Zhang, PhD; Kejing Ying, MD
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Department of Respiratory Medicine, Sir Run Run Shaw Hospital, Medicine School of Zhejiang University, Hangzhou, China

Copyright 2016, American College of Chest Physicians. All Rights Reserved.

Chest. 2016;149(4_S):A339. doi:10.1016/j.chest.2016.02.353
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SESSION TYPE: Case Report Poster

PRESENTED ON: Sunday, April 17, 2016 at 11:45 AM - 12:45 PM

INTRODUCTION: Pulmonary artery intimal sarcoma (PAIS) is a rare but aggressive malignant tumor, usually arises from the intimal layer of the pulmonary arteries, results in high mortality with a median survival time of approximately 13-18 months. Clinical diagnosis of PAIS is often delayed and difficult because of lack of characteristic symptoms.

CASE PRESENTATION: A 40-year-old female patient was referred to our hospital with complaints of chronic diarrhea and intermittent lower abdominal pain for more than 10 years, and acute exacerbation that had commenced two weeks ago. Transthoracic echocardiography demonstrated right ventricular dilation and severe tricuspid valve regurgitation with an estimated right ventricular systolic pressure of 103mmHg. Computed tomography pulmonary angiogram showed a large filling defect within the main pulmonary trunk and extending into the right and left pulmonary arteries. The patient was failure to respond to thrombolytic therapy and presented with progressive dyspnea and abdominal distension. An emergency pulmonary endarterectomy was performed and found a whitish-yellow mass obstructed the lumen of the pulmonary artery, which was pathologically proved to be PAIS. No other hypermetabolic lesions except surgery sites were noted under 18F-fluorodeoxyglucose uptake in positron emission computed tomography. After surgery, the patient’s clinical condition improved and the diarrhea disappeared. But the sarcoma relapsed as an obstructive mass in the right pulmonary artery, a right adrenal gland metastatic lesion and a mass in right hilum were detected during follow-up phrase. The patient suffered symptoms as chest pain, shortness of breath and fatigue, no obvious diarrhea. Her clinical condition continued to deteriorate and could not undergo palliative operation. The patient passed away 17 months after surgery.

DISCUSSION: The definitive diagnosis of PAIS is considered to be difficult and based on pathological examination, specimens are usually obtained from surgery or autopsy. The true incidence of this malignancy is unknown, and the natural course of this orphan disease remains unclear. Metastases of PAIS occurs commonly and are associated with poor prognosis. Patients with PAIS generally have a slowly progressive course over weeks to months, manifesting with a variety of cardiopulmonary complaints. Digestive complaints were rare in primary non-gastrointestinal stromal tumor soft-tissue sarcomas. We supposed that the patient's chronic diarrhea could be considered as paraneoplastic syndrome.

CONCLUSIONS: PAIS is a rare malignancy, clinical manifestation is atypical and easily misdiagnosed. Clinicians must be aware of the presentation and imaging characteristics of this disease.

Reference #1: Parish JM, Rosenow EC III, Swensen SJ, Crotty TB. Pulmonary artery sarcoma: clinical features. Chest 1996;110 (6):1480-1488.

Reference #2: Wong HH, Gounaris I, McCormack A, et al. Presentation and management of pulmonary artery sarcoma. Clin Sarcoma Res. 2015 Jan 21;5(1):3.

DISCLOSURE: The following authors have nothing to disclose: Xiaoling Xu, Ruifeng Zhang, Kejing Ying

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