Chordoma of the thoracic spine in an 89-year-old

Abstract

Study design Case report and literature review. Objective Report the highly unusual presentation of thoracic spine chordoma in an 89-year-old and review existing literature as it impacts treatment in the elderly. Summary of background data Chordomas are infrequent tumors of the spine that commonly present during midadulthood at the spheno-occipital or sacrococcygeal junctions. The mobile spine is affected in 10-15% of cases but chordomas are extremely rare in the thoracic spine. Chordoma rarely enters the differential diagnosis of spinal tumors in elderly patients, for whom metastases and multiple myeloma are by far the most common. Methods A case report is detailed of an 89-year-old male presenting with incapacitating pain and early signs of thoracic myelopathy. A lytic, expanding lesion of the T10 vertebral body with epidural spinal cord compression was identified. In the absence of evidence of other primary tumor, a CT-guided needle biopsy revealed chordoma. A literature review of reported thoracic spine chordomas was also performed. Results An intralesional posterolateral resection and reconstruction was performed with good results and no recurrence at 13 months' follow-up. 30 reports of thoracic spine chordomas were identified in the literature since 1902. Mean age of presentation at 35.7 years is earlier than for most chordomas. The oldest previously reported patient was 68-year-old. Neurological impairment at presentation is rare with the usual presenting symptom being pain.Response to radiation and chemotherapy is limited. 5-year survival rates range from 50 to 60%. Conclusion Chordoma is a highly uncommon epidural neoplasm of the thoracic spine and is vanishingly rare in elderly patients. This report documents the oldest reported patient with thoracic spine chordoma at 89-year-old. Their locally aggressive behavior typically prompts consideration of aggressive surgical resection. When tailored to the individual patient, such procedures are feasible without excessive morbidity even in elderly patients.

Clamshell thoracotomy for en bloc resection of a 3-level thoracic chordoma: technical note and operative video

“…Thoracic chordomas are very uncommon and represent an estimated 1% of all chordomas. 4 Although these tumors tend to arise at a much earlier age than other chordomas, our 20-year-old patient was still well below the mean age of presentation (37.5 years). 2 Early diagnosis of thoracic chordomas is generally difficult because of lack of specific symptoms.…”

“…5 In 2012, Fontes and O'Toole reviewed the literature and found only 22 reported cases of thoracic chordomas. 4 Moreover, chordomas usually appear after age 40 years and are rare in young adults and pediatric patients. 1 When chordomas do occur in pediatric patients, they occur almost exclusively along the clivus.…”

Surgical treatment of giant chordoma in the thoracic spine combining thoracoscopic and posterior spinal surgery

“…[1,2] The tumor grows slowly, recurs locally and may metastasize later, thus, it can be difficult to diagnose and may result in devastating consequences if mismanaged. [7,8] It is a relatively rare tumor, and involvement of the thoracic spine is exceedingly rare. [1] Clinically, the tumor may be asymptomatic but usually presents with symptoms related to compression or involvement of adjacent structures and organs, including the trachea, esophagus, pleura cavity and spinal cord.…”

Lumbar spine chordoma

“…Chordoma occurs in the sacrococcygeal spine in about 50-60% of cases. and in the base of the skull in about 15-40% of cases ( 2 , 3 ). It is a slow-growing tumor, which makes it difficult to diagnose and follow up after treatment ( 4 ).…”