Successful resection of a primary angiosarcoma of the azygos vein: A case report

Abstract

A 63‐year‐old woman was admitted to our department for the investigation of superior vena cava (SVC) syndrome. Computed tomography revealed an azygos tumor extending into the SVC. Video‐assisted thoracic surgery (VATS) was performed to remove the distal end of the azygos vein in the left lateral position, followed by complete resection of the entire tumor under median sternotomy in the supine position. The histological diagnosis was a primary angiosarcoma of the azygos vein. The patient was discharged without any complications and is now alive and tumor‐free 24 months after surgery. In addition, contrast‐enhanced computed tomography revealed no graft occlusion in the two reconstructed brachiocephalic veins. Thoracoscopic surgery in the lateral position is useful for safe and reliable complete resection of a tumor arising from the azygos vein.

Chest computed tomography demonstrated neoplastic lesion from the azygos vein to the superior vena cava. The radiological differential diagnosis suggested angiosarcoma or malignant lymphoma.

INTRODUCTION

A primary intrathoracic angiosarcoma is an extremely rare malignant tumor with a poor prognosis. ¹ Most angiosarcoma cases, when diagnosed, are advanced and unresectable; chemotherapy and/or radiotherapy are the mainstream treatments. ² , ³ , ⁴ , ⁵ Hence, early detection and complete tumor resection are essential for the long‐term survival of patients with an angiosarcoma.

ETHICS STATEMENT

The Teikyo University School of Medicine Ethics Committee approved the study (No. 17‐035) and waived the requirement for written informed consent.

CASE REPORT

A 63‐year‐old woman was referred to our hospital with a history of facial swelling and upper limb edema for 1 month. Chest computed tomography (CT) revealed a heterogeneous mass in the azygos vein which developed within the superior vena cava (SVC) (Figure 1 and Movie S1). The differential diagnosis was angiosarcoma or malignant lymphoma, and surgery was performed for diagnostic and therapeutic purposes. At first, the distal end of the azygos vein was cut off thoracoscopically in the lateral position (Figure 2a,b). After changing the patient to a supine position, full cardiopulmonary bypass was constructed under median sternotomy (Movie S2). A longitudinal incision of the anterior wall of the SVC revealed a tumor arising from the wall of the azygos vein which was developing into the SVC (Figure 2c). The tumor in the azygos vein was completely resected via the SVC. Of note, the aorta was not clamped, and the procedure was performed using the beating heart technique. The bilateral brachiocephalic veins were reconstructed using a 10‐mm synthetic graft (Figure 2d). The pathological diagnosis was angiosarcoma of the azygos vein. The tumor filled the azygos vein and protruded into the SVC macroscopically (Figure 3a), and microscopically consisted of irregularly sized and round to oval shaped tumor cells with atypical nuclei (Figure 3b). Immunohistochemistry demonstrated CD31 and factor VIII positivity (Figure 3c,d). Symptoms of SVC syndrome disappeared immediately after the surgery, and the patient was discharged without complications. The patient is alive 24 months after surgical treatment, and there was no evidence of tumor recurrence or graft occlusion at the standard 6‐month follow‐up.

FIGURE 1.

Chest CT images. Chest CT shows the tumor within the (a) SVC obstructing the blood flow (yellow arrow) and (b) azygos vein (yellow arrow). (c) Reconstructed three‐dimensional image of the tumor (yellow arrow). CT, computed tomography; SVC, superior vena cava.

FIGURE 2.

Surgical view of the tumor in the azygos vein. (a) Thoracoscopic view shows the tumor confined to the azygos vein (yellow arrow). (b) Excision of the azygos vein at the distal end using an automatic suture (yellow). Tumors in the azygos vein appear free from the surrounding tissues (blue). (c) A longitudinal incision of the anterior wall of the SVC reveals the tumor through median sternotomy (yellow arrow). The tumor is free from the SVC wall without the orifice of the azygos vein (blue). (d) Each bilateral BCV is vascularised using a 10‐mm synthetic graft (ringed Gore‐Tex) after complete tumor resection (yellow and blue arrows). BCV, brachiocephalic vein; RUL, right upper lobe; SVC, superior vena cava.

FIGURE 3.

Pathological examination. Hematoxylin and eosin staining demonstrated multiple anastomosing vascular structures lined by tumor cells (a) which consisted of irregularly sized and round to oval shaped tumor cells (b). Immunohistochemistry showed cytoplasmic expression of CD31 and factor VIII antigens (c, d).

DISCUSSION

A primary angiosarcoma of the thorax is extremely rare, and there have been no previous reports of its origin from the azygos vein. ¹ , ² , ³ , ⁴ , ⁵ In the present case, it was difficult to visualize the distal end of the azygos vein through a median sternotomy, even if thoracoscopy was used, because of the presence of the tumor itself. In rationale, VATS in the lateral position allows visualization more clearly with an easier and safer resection and is useful for the safe and reliable complete resection of a primary tumor arising from the azygos vein.

In conclusion, in this study, we report the successful resection of a primary angiosarcoma of the azygos vein, resulting in a good prognosis and long‐term patent revascularization. Therefore, our surgical strategy is worthy of consideration from the perspective of reliability and safety.

AUTHOR CONTRIBUTIONS

Study conceptualization: Yuichi Saito; data curation, Shinya Kohmaru, Masahiro Urata, and Tomohiro Imazuru; writing—original draft preparation, Yuichi Saito; writing—review and editing, Yukinori Sakao and Tomoki Shimokawa; visualization, Yuichi Saito and Koji Saito. All authors read and agreed to the published version of this manuscript.

CONFLICT OF INTEREST STATEMENT

The authors declare no conflicts of interest.

Supporting information

Movie S1. Coronal view of computed tomography. The tumor arising from the azygos tumor developing into the lumen of the SVC.

Movie S2. Surgery movie. After VATS was performed to resect the distal end of the azygos vein, the tumor was resected along with SVC under a median sternotomy.

Kohmaru S, Saito Y, Urata M, Imazuru T, Saito K, Shimokawa T, et al. Successful resection of a primary angiosarcoma of the azygos vein: A case report. Thorac Cancer. 2024;15(7):575–577. 10.1111/1759-7714.15188