Brain Tumor Res Treat.  2022 Apr;10(2):123-128. 10.14791/btrt.2022.0007.

Recurrent L3 Chordoma Presented as Intradural Extramedullary Mass With Distant Metastasis: A Case Report

• Jang SJ
• Han N
• Hong EK
• Gwak HS

• KMID: 2529445
• DOI: http://doi.org/10.14791/btrt.2022.0007

Abstract

Here, we report a rare case of L3 chordoma progressed to an intradural extramedullary (IDEM) mass and distant metastasis to the fascia lata. A 64-year old female patient presented to a local university hospital due to back pain and received excisional biopsy for a L3 destructive bony lesion. Local radiation therapy was initially administered, assuming a malignancy of unknown origin, but she developed cerebrospinal fluid leakage during adjuvant radiation therapy, which was managed by wound revision and lumbar drainage. As the destructive lesion progressed, she visited our hospital for a second opinion 3 months after the biopsy. After review of outside pathology, we diagnosed the lesion to be a chordoma, and performed a L3 corpectomy with cage and plate fixation. One and a half years later, positron emission tomography and computed tomography (PET-CT) revealed a right tensor fascia lata hypermetabolic lesion. Excisional biopsy confirmed a distant metastasis of the chordoma. One year later, she complained of L2 radiating pain. PET-CT and CT myelogram revealed an IDEM lesion. Surgical excision confirmed the transdural invasion of the chordoma. To our knowledge, this is the first report of an iatrogenic IDEM invasion and distant metastasis to the tensor of the fascia lata by a L3 chordoma.

Keyword

Chordoma; Bone neoplasms; Neoplasm metastasis; Neoplasm invasion; Cerebrospinal fluid leak

Figure

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  Fig. 1 Preoperative images suggesting L3 metastatic lesion. Spine MRI showed a low signal intensity on T1-weighted (A) and mixed signal intensity on T2-weighted with gadolinium enhancement suggesting metastatic lesion (B and C). However, virtual wholebody and axial PET-CT (D and E) showed no other hypermetabolic lesion except L3 vertebra.

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  Fig. 2 Serial postoperative images of the second salvage operation for of L3 chordoma of patients with severe osteoporosis. A and B: Postoperative spine MRI during the course of radiation therapy revealed cerebrospinal fluid leakage collected at subcutaneous space with residual tumor and bone cement injected. C and D: At our hospital, L3 corpectomy with cage and lateral plate fixation supported by pedicle screw and rod was performed. E: However, the cage was collapsed into L4 as soon as patient’s minimal ambulation. F: T12, L1-L5, and S1 percutaneous posterior fixation was added for her failed back syndrome.

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  Fig. 3 Photomicrographs of the initial, recurrent as intradural extramedullary and metastatic surgical specimens. A: Malignant tumor with epithelioid cell morphology and myxoid stroma, suggestive of chordoma (H&E, ×200). B: Chordoma, post-radiation therapy status (H&E, ×100). C: Metastatic mass to thigh (H&E, ×100). D and E: Cytokeratin immunohistochemical staining (D) and S-100 immunohistochemical staining (E) for the metastatic mass (×200). F: Recurrent intradural extramedullary mass with high grade feature (H&E, ×100).

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  Fig. 4 Images revealing metastasis of the chordoma. A: Positron emission tomography showed a hot uptake round lesion at right thigh. B: T1-weighted gadolinium enhanced MRI revealed an enhancing metastatic lesion abut to tensor fascia lata.

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  Fig. 5 Images implicated intradual extramedullary recurrence 1.5 years after the second operation. A: PET-CT taken showed intraspinal hotuptake lesion. B: CT-myelogram revealed a block of contrast at L2-L3 level suggesting intradural extramedullary mass.

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