Presentation
History of weight loss, back pain, and leg weakness. Now having difficulty ambulating and paresthesias in the feet and calves.
Patient Data
There are multiple, large homogeneously enhancing masses throughout the left hemithorax and left paraspinal soft tissues. There is focal extension of enhancing soft tissue through the left T6-T7 and T7-T8 neural foramen into the dorsal epidural space at these levels. There is resultant right lateral displacement of the thoracic spinal cord and subtle cord compression. However, there is no evidence of myelopathic edema within the thoracic spinal cord.
There is faint elevated STIR signal within the T7 vertebral body and the posterior aspect of the T6 vertebral body. The areas of elevated STIR signal also display reduced diffusivity.
Case Discussion
This is a case of a thoracic desmoplastic small round cell tumor.
The patient initially underwent a fine needle aspiration where pathology revealed fragments of atypical cells which were positive for desmin. There was also focal staining of synaptophysin.
The patient then underwent a decompressive thoracic laminectomy at T5-T6, T6-T7, and T7-T8, as well as debulking of the epidural tumor. Gross pathologic examination revealed a tan-white, rubbery tissue. Immunohistochemistry was positive for CK, desmin, WT1, and focally positive for synaptophysin. The histopathologic and immunohistochemical results were consistent with a desmoplastic small round cell tumor. The patient's post-operative course was uncomplicated.
Co-author:
Morgan Zhao, MD
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