Extramedullary Intradural Primary Spinal Angiosarcoma: A Case Study

Abstract

Angiosarcoma is a rare soft tissue sarcoma, namely when it presents as a primary intradural extramedullary spinal neoplasm, with only one case of non-vertebral origin reported in the literature. We present the case of a 51-year-old woman with neurological symptoms of paraparesis and constipation who underwent a magnetic resonance imaging (MRI) that revealed a well-demarcated, predominantly homogeneous, intensely enhancing intradural extramedullary lesion in T2-weighted images. Histology, immunohistochemistry, and genetics of the lesion showed an angiosarcoma. The patient received adjuvant radiation therapy (RT) and is currently stable, showing no signs of recurrence, in the third year of follow-up. Our work demonstrates a rare case of extramedullary intradural primary spinal angiosarcoma, highlighting the challenges associated with its diagnosis. The multidisciplinary approach, including surgery and adjuvant RT, has been shown to effectively control the disease. Although the prognosis is generally poor, the presented case shows apparent success.

Keywords: neuro radiology; neurosurgery; primary angiosarcoma; primary intradural extramedullary spinal tumor; radiation therapy.

Figure 1. MRI showing an intracanal extramedullary lesion with compression of the cauda equina

A) Sagittal T2-weighted images showed a hyperintense intradural extramedullary lesion at the level of L2-L3 with well-defined borders. B) Sagittal T1-weighted images revealed a hyperintense lesion relative to the spinal cord. C) Strong enhancement of the lesion on post-contrast axial T1-weighed imaging.

Figure 2. Microscope images of the resected angiosarcoma

A) Hematoxylin-eosin staining of the resected angiosarcoma; B) Showing diffuse ERG staining; C) Strong endothelial expression of CD31 ERG: Erythroblast transformation-specific Related Gene

Figure 3. Post-operative lumbosacral spine MRI

Lumbosacral MRI is showing signs of a laminectomy of L2 and L3; the presence of a left center-lateral cystic-appearing collection centered on the posterior soft tissues, measuring approximately 40 mm in cranio-caudal diameter, hyperintense on axial T2 (A) and sagittal STIR (B), hypointense on sagittal T1, without signal enhancement after gadolinium (C), probably corresponding to a seroma; and foci of signal enhancement after gadolinium in close relation with the roots of the cauda equina, possibly due to previous injury/surgical intervention (C). STIR: Sagittal short tau inversion recovery

Figure 4. Post-operative PET/CT scan

Lateral (A) and coronal (B) views of the maximal intensity projection (MIP) display, axial (C) and sagittal plane (D) of fused tomographic renditions showing high uptake in the posterior planes of the spinous apophysis of L2 and in the body of L3, probably related to iatrogenic alterations. Tumor persistence could not be excluded. No other irregular foci were observed.

Figure 5. Last surveillance MRI of the spine

Sagittal post-contrast T1-weighted (A) and axial T1-weighted (B) MRI showing signs of a previous laminectomy of L2 and L3. No notable foci of enhancement were visible.