Cervical intramedullary immature teratoma with metastatic recurrence in an adult

Abstract

Study design: Case report.

Objectives: We present for the first time an adult patient with cervical intramedullary immature teratoma with metastatic recurrence.

Setting: Peking university Shenzhen Hospital, Shenzhen, China.

Methods: A 30-year-old woman presented with rapidly progressive quadriplegia. Magnetic resonance imaging (MRI) revealed an intramedullary tumor occupying C1-C2 of the upper spinal cord. An urgent operation, consisting of decompression by laminectomy and tumor gross resection, was performed under a preoperative diagnosis of spinal glioma. The histological diagnosis was immature teratoma. The patient received local radiotherapy after gross total resection. The serum alpha fetoprotein (AFP) and human chorionic gonadotropin (HCG) levels were normal postoperatively, until these were evaluated on the 10th month with neurological deterioration. Metastatic recurrences were demonstrated on MRI with lesions located at the levels of C5-C6 and T11-12. Removal of the second tumors was performed and the pathological examination identified a malignant germ cell tumor (yolk sac tumor). The patient was then referred to chemotherapy and radiotherapy.

Results: No tumor regrowth was encountered and the patient remained stable for 6 months after adjuvant therapy.

Conclusion: Immature teratoma should be included in the differential diagnosis of holocord tumors in the adult with rapidly progressing symptoms and if found should be radically excised if possible. Adjuvant therapy should be the salvage therapy for this recurrent tumor.

Figure 1

Preoperative plain computed tomography scan shows a hypodense lesion located at C1–C2 levels without calcification (a). Preoperative axial T1-weighted MRI shows the bad-delineated mass of hypointense signal located exclusively in the cervical spinal cord (b). Sagittal MRI (c and d) depicts the fusiform-shaped and eccentrically located intramedullary tumor at the C1–C2 levels, appearing hypointense on T1-weighted and mixed signal intensity on T2-weighted images with adjacent spinal cord edema. The cystic lesion within the spinal cord distal to the mass is quite visible (see white dovetail arrow). (e and h) Immediately postoperative Gd-enhanced T1-weighted sagittal and axial MRI shows gross-total removal of the tumor (black arrowhead). Sagittal (f and g) and axial (i and j) on the 10th month follow-up MRI images with contrast reveal two metastatic extramedullary lesions at C4–C6 and T11–12, respectively, appearing highly enhanced with heterogeneous features. No changes are observed in the previous operation site (see white double arrow). (k) Postoperative T1-weighted sagittal MRI with contrast depicts near-total resection of the cranial and caudal tumors, respectively. Of note, numerous additional disseminated enhancing foci nodules are seen along the leptomeninges and dura, likely representing metastatic drop lesions (long white arrows).

Figure 2

Photomicrographs of the tumor (first operation) show immature neural tissue with the formation of neural tubes (a) and no immunoreactivity for AFP (b). Histopathological examination (second operation) reveals a typical yolk sac tumor with reticular network of medium-sized polygonal malignant cells, moderately pleomorphic hyperchromatic to vesicular nuclei, with many areas of myxoid degeneration. Intracellular hyaline bodies were present (c). Immunohistochemical images: tumor cells showing strong positivity for AFP (d). Scale bars=200 μm (a), 100 μm (b–d).

Figure 3

Clinical course and changes in the serum AFP and HCG levels. CSR, craniospinal radiation.