Characteristics and outcomes of primary pleural angiosarcoma

Abstract

Primary pleural angiosarcoma (PPA) is an extremely rare malignancy for which there is no consensus on treatment. The clinical course of PPA is usually quickly fatal, regardless of the treatment used.We summarized and evaluated a relatively large population of published PPA cases to assess prognostic factors, diagnostic approaches, treatment methods and clinical outcomes. Using the CNKI, Embase, and PubMed databases, literature published in English and Chinese from 1988 through 2020 was searched using the terms “primary pleural angiosarcoma,” “pleural angiosarcoma,” and “pleuropulmonary angiosarcoma.”A total of 43 patients with PPA were identified in retrospective case series and case reports. The median age at diagnosis was 64 years (range 24–87 years), and the median overall survival was 4 months (range 0.1–180 months). Approximately 80% of patients died from PPA within 10 months of diagnosis, and the 2-year survival rate was approximately 4.4%. In univariate analyses, the presence of pleural effusion and hemothorax were significant predictors of decreased survival, with hazard ratios (HRs) of 2.7 (P = .04) and 3.3 (P = .006), respectively. Sixteen patients received no therapy, and their prognosis was worse than patients who did receive therapy (P = .019). Radiation therapy improved survival more than no radiation therapy (P = .007). Patients appeared to derive clinical benefit from chemotherapy (P = .048). However, tumor resection did not seem to provide a survival benefit (P = .051). In multivariate analysis, tumor resection, and radiation were independent, statistically significant, positive predictors of better survival, with HRs of 0.3 (P = .017) and 0.1 (P = .006), respectively. The presence of hemothorax was an independent predictor of worse prognosis (P = .006).Primary angiosarcoma of the pleura is a rare, poorly understood malignancy with a poor prognosis; hence, the clinical spectrum of PPA is not completely defined. By multivariate analysis, this retrospective study showed a survival benefit of tumor resection or radiation therapy, and the presence of hemothorax was a significant prognostic factor for poor outcomes.

Primary Pleural Epithelioid Angiosarcoma with Lung and Bone Metastases: A Case Report

“…Primary pleural EAS is an extremely rare malignancy with atypical clinical symptoms and CT findings, resulting in a high rate of misdiagnosis. Wang et al [ 5 ] summarized 43 cases of primary pleural angiosarcoma with a mean age of 64 years (24–87 years) which indicated a higher incidence in males. The most frequent manifestations were pleural mass (44%) and pleural invasion (70%).…”

“…Currently, there is no standard therapy. Pleural effusion and hemothorax were significant predictors of decreased survival [ 5 ]. The mean survival after the diagnosis was less than 1 year [ 14 , 15 ].…”

Angiosarcoma of breast and chest wall complicated with tuberculous empyema: A case report

“…At present, complete surgical resection is the first and optimal method for the treatment of angiosarcomas. A study 22 reported that patients who undergo surgical resection survive for at least 15 years. Angiosarcomas are invasive and prone to metastasis.…”

Hypothesis: HEG1 and claudin‐4 staining will allow a diagnosis of epithelioid and biphasic mesothelioma versus non‐small‐cell lung carcinoma with only two stains in most cases

“…Some thyroid carcinomas also can be both HEG1 and claudin‐4‐positive 3,9 . One potential positive HEG1/negative claudin‐4 confounder is angiosarcoma, but these tumours are extremely rare in the serosal membranes; a recent review of the literature from 1988 to 2020 found only 43 primary pleural angiosarcomas, 20 and most angiosarcomas are not epithelioid 21 . For comparison there are approximately 3000 mesotheliomas every year in the United States alone and 34 000 worldwide 22 …”