Chordoma of the Thoracic Spine. Case Report.

Abstract

A 44-year-old woman presented with a thoracic chordoma with intrathoracic extension manifesting as complaints of lower extremity weakness, hypesthesia below the levels of T5-6, and sphincter incontinence. Almost total resection combined with anterior interbody fusion and stabilization was possible through a left transpleural transthoracic approach. She suffered recurrence after 2 years and was considered inoperable. Biopsy revealed a malignant chordoma with no sarcomatous differentiation. Chordoma is an uncommon malignant bone tumor originating from remnants of the embryonal notochord, occurring mostly along the axial skeleton, at the extremity of the vertebral spine, and is least common in the thoracic region. Differential diagnosis is problematic and biopsy is helpful particularly if considered inoperable. Thoracic chordomas of the malignant type manifest as cord or root compression. Classical malignant chordomas must be distinguished from chondroid, benign, or other types of chordomas, since the biological behavior and clinical features are distinct. However, the differential diagnosis cannot be based on histological examination, but long-term follow up is required. Most patients have extradural and intraspinal tissue extension at the time of diagnosis, which makes complete resection impossible. Aggressive surgery without violation of surgical borders is the best choice in the treatment of thoracic chordoma. Thoracic chordoma is a recurring neoplasm and is prone to dissemination and sarcomatous differentiation despite its slow-growing nature.

Giant Chordoma of the Upper Thoracic Spine with Mediastinal Involvement: A Surgical Challenge

“…The more accepted approach is aggressive surgery to resect the tumour as far as possible and prevent dissemination into the surrounding tissues [3,4]. Thoracic chordomas are more difficult to resect totally than analogous lesions affecting other sites since most patients like the present case have extradural and paraspinal tissue extension at the time of diagnosis.…”

“…A recent study described some cases of thoracic spine chordoma with mediastinal involvement from direct invasion to metastatic spread [2]; to our knowledge, the present case represents a rarity because of its dimension [3,4,5]. …”

Imaging of the Lumbar Spine Neoplasms

“…They have been described to be more common at the cranial and caudal ends of the spine, that is, clivus and sacrum, respectively, but may occur at any level, with 35% arising in the lumbosacral spine. [8][9][10][27][28][29][30] The basic cell type of a chordoma appears soap bubbly under light microscopy (physaliphorous), thus dictating its imaging properties. It appears as a lytic lesion on radiography and CT scanning.…”

Huge myxoid chondrosarcoma expanded into the thoracic cavity with spinal involvement

“…En bloc resection is therefore the treatment of choice [2,3,[13][14][15]. Regional constraints and the dimension of some spine tumor can make impossible to perform en bloc resection [16][17][18][19][20].…”