Hemoptysis in a Patient with Multifocal Primary Pulmonary Angiosarcoma

Abstract

Primary pulmonary angiosarcoma (PPA) is a rare tumour arising from arterial or venous pulmonary vessels of various size. It is characterized by aggressive course and poor prognosis. The early diagnosis is difficult due to diverse clinical and radiological manifestations. We present a case report of 70 year-old man, active cigarette-smoker, with a 2-month history of non-massive hemoptysis. The thorax CT revealed several solid pulmonary nodules surrounded by areas of ground glass opacity. As bronchoscopy failed to deliver adequate tissue samples, video assisted thoracic surgery (VATS) with pleura and lung biopsy was necessary. Histopathological findings were consistent with pulmonary angiosarcoma. Since no extrapulmonary lesions were demonstrated, the final diagnosis of primary pulmonary angiosarcoma was made. The patient died three months after the onset of symptoms. Our case report highlights that differential diagnosis in patients with hemoptysis and pulmonary nodules should include primary pulmonary sarcoma. Case presentationA 70-year-old man was referred to our department from one of municipal hospitals in January 2016 with a 2-month history of non-massive hemoptysis (daily volume of expectorated blood was approximately 15 ml). The patient also reported weight loss of 3 kg during the last month, general weakness and mild recurrent epistaxis. His past medical history included arterial hypertension and chronic sinusitis, both diagnosed approximately 10 years ago. He had also undergone endovascular stent grafting of abdominal aorta aneurysm as well as transurethral resection of the prostate due to benign prostatic hyperplasia. He was an active cigarette-smoker with a history of 52 pack-years of tobacco smoking. During the hospitalisation in the municipal hospital two pulmonary nodules in the left lower lobe were found in chest radiograph. These findings were confirmed in thorax CT scan which also showed ground glass areas surrounding the nodules (halo sign).On admission the patient's general condition was satisfactory. His vital signs were within normal range: arterial blood pressure was 120/80 mm Hg, heart rate 80 beats per minute, transcutaneous arterial oxygen saturation 93% while breathing room air. Dullness to percussion and diminished breath sounds over the left lower lung field was noted on physical examination. Except these findings the physical examination did not reveal any relevant abnormalities.The results of laboratory studies showed normocytic anaemia (hemoglobin level 90.5 g/L), an elevated peripheral white blood cell count (WBC)

Radiologic findings of primary pulmonary angiosarcoma

“…[ 3 – 5 ] The most common clinical symptom of primary pulmonary angiosarcoma is hemoptysis, which has been described in previous studies. [ 6 , 7 ] But the initial symptom of our report is cough. Most patients with primary pulmonary angiosarcoma have poor prognosis after surgery excision.…”

Primary pulmonary epithelioid angiosarcoma with thyroid tumor history: A case report and literature review

“…Pulmonary angiosarcomas are invariably (>90%) metastatic tumors from primary malignancies of the skin, bone, liver, breast or heart ( 8 ). Primary pulmonary angiosarcoma (PPA) is a rare tumor arising from arterial or venous pulmonary vessels of various sizes ( 9 ), and only ~30 cases of PPA have been reported to date ( 5 , 7 ). PPAs are more common in males and the majority of patients are aged 40 years or above ( 7 , 10 ).…”

Spontaneous Regression of Epithelioid Angiosarcoma in a Young Woman

“…Angiosarcoma is a rare soft-tissue neoplasm that accounts for 1-2% of all sarcomas, and which occurs most commonly in the skin and subcutaneous tissues. Angiosarcoma arising in the gastrointestinal tract or lung is very rare ( 2 - 4 ). The treatment modalities include surgical resection of the primary tumor and radiotherapy.…”