Long-term follow-up after en bloc resection and reconstruction of a solitary paraganglioma metastasis in the first lumbar vertebral body: a case report

Abstract

Introduction: Paragangliomas are rare tumors that originate from the autonomic nervous system-associated paraganglia. They metastasize infrequently. Malignancy can only be demonstrated by the presence of chromaffin tissue at sites where it usually is not present, such as bone, lung or liver, or local recurrence after total resection of a primary mass. Paragangliomas within the central nervous system are usually intradural near the conus medullaris. The metastatic spread of a retroperitoneal paraganglioma to a vertebral body is extremely rare, and there are only a few cases reported in the literature.

Case presentation: We report the case of a 16-year-old Caucasian girl who had undergone resection of a retroperitoneal paraganglioma that measured 15 × 11.5 × 9.5 cm. After further staging, a solitary metastatic paraganglioma was detected in the first lumbar vertebral body. After initial chemotherapy, marginal en bloc resection and reconstruction were performed followed by radiotherapy. Histologic examination of the specimen revealed that the tumor cells did not show any response to preoperative chemotherapy, which is in line with a few other reports in the literature. Ten years after operative treatment, the patient is free of complaints, very satisfied with the result and without signs of local recurrence or distant metastases.

Conclusion: We recommend en bloc spondylectomy and local radiotherapy in the treatment of solitary spinal metastatic paragangliomas.

Figure 1

Magnetic resonance image showing the metastatic lesion within the vertebral body with destruction of the posterior cortex, encroachment of the spinal canal and invasion of the left pedicle.

Figure 2

En bloc resected vertebral body with the affected left pedicle left en bloc.

Figure 3

Postoperative anteroposterior and lateral plane radiograph showing reconstruction with modular tumor cage and a pedicle-screw instrumentation.

Figure 4

Horizontal cut through the resected vertebral body. Complete destruction of the posterior cortical lamellae with intact pseudocapsule. Metastatic lesion in zones 4 to 9 and layer B (intraosseous superficial), according to Boriani et al [23].

Figure 5

Anteroposterior and lateral plane radiograph at 10-year follow-up showing no signs of lysis or cage dislocation.