Thoracic Chordoma Misdiagnosed as Primary Adenocarcinoma of the Mediastinum

Abstract

Chordoma is a rare malignant bone tumor originating from the embryonic notochord. Herein, we present a case of thoracic chordoma located at T3–T5 that was misdiagnosed as primary mediastinal adenocarcinoma. The patient underwent neoadjuvant chemoradiation and the disease showed little response. Due to vertebral body invasion, we performed en bloc mass removal and partial corpectomy (T4–5) in collaboration with orthopedic surgeons.

Unusual Presentation of Thoracic Chordoma with Spinal Epidural Hematoma: A Rare Case Report and PRISMA-Driven Systematic Review

“… 5 One-third of reported patients were asymptomatic and detected during routine chest examinations 20 Many preliminary reports described several presentations for thoracic chordoma 10 , 12 , 15 Among these reports, chordomas were initially misdiagnosed as different neoplasia, including hemangioma, benign neurinomas, 23 and adenocarcinomas. 13 Others reported chordomas metastasizing to lymph nodes, lungs, 18 bones, brain, and viscera. Nonetheless, this type of chordoma is more aggressive.…”

“…However, these features did not confirm chordomas, as some chordomas may mimic other neoplasia. 13 So, a biopsy should be examined under the microscope to ensure the specific pattern of physaliferous bubbly cells within the myxoid stroma, which indicates a chordoma lesion. A tissue sample should also be examined for a high Brachyury gene value manifesting in high concentrations.…”