J Korean Soc Radiol.  2019 May;80(3):568-573. 10.3348/jksr.2019.80.3.568.

Extraskeletal Ewing's Sarcoma of the Thoracic Nerve Root: A Case Report

Affiliations
  • 1Department of Radiology, Inje University College of Medicine, Sanggye Paik Hospital, Seoul, Korea. merita@paik.ac.kr
  • 2Department of Neurosurgery, Inje University College of Medicine, Sanggye Paik Hospital, Seoul, Korea.
  • 3Department of Pathology, Inje University College of Medicine, Sanggye Paik Hospital, Seoul, Korea.

Abstract

Extraskeletal Ewing's sarcoma (EES) is a rare malignant soft tissue tumor which is morphologically indistinguishable from skeletal ES. EES usually occurs in young adults and children and there has been only one case reported in a patient aged over 70 years old. We report a case of an EES arising from the first thoracic spinal nerve root in a 73-year-old female, which was misdiagnosed as benign nerve sheath tumor in preoperative imaging evaluation.


MeSH Terms

Aged
Child
Female
Humans
Sarcoma, Ewing*
Soft Tissue Neoplasms
Spinal Nerve Roots
Thoracic Nerves*
Young Adult

Figure

  • Fig. 1 Extraskeletal Ewing's Sarcoma in a 73-year-old female. A. Thoracic spine CT with contrast enhancement shows a dumbell-shaped mass in the T1–T2 left neural foramen extending inside the central canal and outside the neural foramen, with mild homogeneous enhancement (arrows). Mild widening of the neural foramen is noted. B. On MRI with contrast enhancement, the lesion shows intermediate signal intensity on the axial T1WI and slightly high signal intensity on the T2WI. Homogeneous enhancement of the lesion is seen on the FS-T1WI after administration of Gd-CE. The FS-T2WI shows the tumor extending along the first branch of left T2 nerve root (arrowheads). Gd-CE = gadolinium contrast-enhanced, FS = fat saturation, T1WI = T1-weighted image, T2WI = T2-weighted image C. Intraoperative microscopic photograph. The tumor is located in the intradural portion of T1–T2 (asterisk). The T1 nerve root is seen on the left side (arrowhead). D. Left photomicrograph of the specimen shows alternating (highly cellular and paucicellular) cellularity with dense collagen bands. The right high-power view shows highly overlapping blue round cells with oval nuclei and scanty cytoplasm. Neuroectodermal differentiation such as rosette formation was not observed (H&E stain × 10; left, H&E stain × 400; right). Immunohistochemical staining result reveals weak positivity for vimentin, CD56, and strongly membranous staining for CD99 (H&E stain × 400). H&E = hematoxylin and eosin


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