Primary Pulmonary Angiosarcoma Found Incidentally in a Complicated Patient: A Rare Case Report

• Published in: The clinical respiratory journal Vol. 18; no. 8; pp. e13818 - n/a
• Main Authors: Basiri, Reza, Ziaei Moghaddam, Alireza, Rikhtegar, Arezoo, Jafarian, Amir Hossein
• Format: Journal Article
• Language: English
• Published: England John Wiley & Sons, Inc 01.08.2024; John Wiley and Sons Inc; Wiley
• Subjects: Adult; Biopsy; Bronchoscopy - methods; complicated patient; Fatal Outcome; Female; Hemangiosarcoma - complications; Hemangiosarcoma - diagnosis; Hemangiosarcoma - pathology; Humans; incidental finding of tumor; Incidental Findings; Indazoles; Lung Neoplasms - complications; Lung Neoplasms - diagnosis; Lung Neoplasms - pathology; Original; Ostomy; Paclitaxel - administration & dosage; Paclitaxel - therapeutic use; primary pulmonary angiosarcoma; Pyrimidines - therapeutic use; rare case report; Sulfonamides - therapeutic use; Tomography, X-Ray Computed - methods; incidental finding of tumor; rare case report; complicated patient; primary pulmonary angiosarcoma
• ISSN: 1752-6981; 1752-699X; 1752-699X
• DOI: 10.1111/crj.13818

ABSTRACT Introduction Primary pulmonary angiosarcoma (PPA) is a highly aggressive and rare malignancy originating from the endothelial cells of blood vessels in the lungs. PPA is an extremely rare subtype, with less than 30 cases reported to date. PPA is not only challenging to diagnose but also has a poor prognosis, often resulting in a high mortality rate within a year of diagnosis, regardless of the treatment approach. Method We present the case of a 33‐year‐old woman with no significant past medical history who presented with abdominal pain and was incidentally found to have a right hilar mass with pleural effusion and empyema. After undergoing surgery for a perforated gastric ulcer, her pulmonary lesions were further worked up. Despite an extensive diagnostic evaluation, including imaging, bronchoscopy, and thoracotomy, establishing a diagnosis was challenging. Ultimately, PPA was diagnosed on surgical lung biopsy, and the patient was started on pazopanib and paclitaxel chemotherapy but expired after 1 month due to multiple complications. Conclusion This case highlights the difficulty in diagnosing this rare tumor and its poor prognosis regardless of therapy. Greater awareness of PPA and more research are needed to improve early detection and treatment options for this deadly disease. This case report details the diagnostic complexity of primary pulmonary angiosarcoma (PPA), a rare and aggressive endothelial malignancy, found incidentally in a complicated 33‐year‐old woman, and serves to underscore the significant clinical challenges faced during the diagnostic process and the grim prognosis that accompanies this rare malignancy. Despite extensive investigative measures, the diagnosis remained elusive until postsurgical biopsy, following which the patient succumbed within a month.