Epithelioid angiosarcoma of the cervical spine: A case report

Abstract

Background: Epithelioid angiosarcoma (EA) is an aggressive, malignant endothelial-cell tumor of vascular or lymphatic origin. EA often arises from deep soft tissues such as pleura, breast, bone and gastrointestinal tract. It usually affects patients aged 60-70 years and is associated with high recurrence and metastasis rates with surgical resection as the primary treatment of choice. Overall survivals are generally poor, ranging from 6 to 16 months. More than 50% of patients died of disease within 2 to 3 years of diagnosis.

Case summary: We present a rare case of EA of the cervical spine causing a C6 pathological fracture complicated by severe kyphosis. The patient received C4-7 posterior laminectomy and C2/3/4/7/T1 transpedicular screw fixation, followed by anterior C5-6 corpectomy with allograft bone fusion and cervical plate fixation. Postoperative radiotherapy was administered without delay. However, the patient died of rapidly progressive acute respiratory distress syndrome 3 weeks after the second surgery.

Conclusion: EA with spinal involvement is extremely rare. Early detection and cord decompression may prevent neurological deterioration and preserve better quality of life.

Keywords: Case report; Cervical spine; Epithelioid angiosarcoma; Pathologic fracture; Spinal cord compression; Vertebral body.

Figure 1

Radiological changes in the cervical spine and lung. A: Computed tomography of the spine shows no obvious spinal fracture, kyphotic deformity, or alignment deformity; B: Cervical lateral radiograph shows C6 burst fracture with severe kyphosis; C: Computed tomography (CT) of the lung shows diffuse nodules in both lungs. The metastasis was suspected; D: Repeat CT of the lung 1 month later shows rapid progression of the tumor in both lung and pleura.

Figure 2

Pre-operative cervical spine magnetic resonance imaging, intra-operative C-arm radiograph, and post-operative chest radiograph. A: Spinal magnetic resonance images demonstrate the C6 pathologic fracture on T1-weighted images; B: With central necrosis and mixed some fluid and hematoma on T2-weighted images; C: Poor and heterogenous enhancement on T1-weighted images with gadolinium administration; D: The C-arm radiograph images show C2/3/4/7/T1 transpedicular screw and C5-6 corpectomy with tibia bone graft fusion and plate fixation in lateral view; E: The C-arm radiograph images show C2/3/4/7/T1 transpedicular screw and C5-6 corpectomy with tibia bone graft fusion and plate fixation in coronal view; F: Rapid progression of acute respiratory distress syndrome 3 weeks after the second surgery.

Figure 3

Histopathological examination of the tumor. Proliferation of cells exhibiting epithelioid morphology, prominent nucleoli and abundant eosinophilic cytoplasm. Tumor necrosis is present. A: Hematoxylin-eosin (HE), 40 ×; B: HE, 100 ×; C: High-power view demonstrates pleomorphic epithelioid cells, intermixed with acute inflammation. Mitotic cells are present (HE, 400 ×); D: Anastomotic vascular channels lined by epithelioid endothelial cells (HE, 200 ×).

Figure 4

Immunohistochemical staining of the tumor. Immunohistochemical staining shows diffuse nuclear staining of tumor cells expressing cytokeratin. A: AE1/AE3; B: CD31; C: CD34; D: ERG. All pictures are 40 × magnification.