Primary Spinal Germ Cell Tumors: A Case Analysis and Review of Treatment Paradigms

Abstract

Objective. Primary intramedullary spinal germ cell tumors are exceedingly rare. As such, there are no established treatment paradigms. We describe our management for spinal germ cell tumors and a review of the literature. Clinical Presentation. We describe the case of a 45-year-old man with progressive lower extremity weakness and sensory deficits. He was found to have enhancing intramedullary mass lesions in the thoracic spinal cord, and pathology was consistent with an intramedullary germ cell tumor. A video presentation of the case and surgical approach is provided. Conclusion. As spinal cord germinomas are highly sensitive to radiation and chemotherapy, a patient can be spared radical surgery. Diverse treatment approaches exist across institutions. We advocate biopsy followed by local radiation, with or without adjuvant chemotherapy, as the optimal treatment for these tumors. Histological findings have prognostic value if syncytiotrophoblastic giant cells (STGCs) are found, which are associated with a higher rate of recurrence. The recurrence rate in STGC-positive spinal germinomas is 33% (2/6), whereas it is only 8% in STGC-negative tumors (2/24). We advocate limited volume radiotherapy combined with systemic chemotherapy in patients with high risk of recurrence. To reduce endocrine and neurocognitive side effects, cranio-spinal radiation should be used as a last resort in patients with recurrence.

Primary Extramedullary, Extradural Cervical Spine Seminoma

“…Primary spinal germinomas have also been described although they typically arise from the medulla of the spinal cord itself. In a literature review by Loya et al, 13 30 primary intradural spinal germinomas were reported, only 13% of which were purely extramedullary. All four of the cases of primary extramedullary spinal germinomas were intradural, requiring posterior laminectomy and durotomy to access the lesions, and careful and extensive dissection of the tumor from the nerve roots with variable clinical success (Table 1 ).…”

Delayed diagnosis of intramedullary spinal cord germinoma

“…Primary intramedullary germinoma is rare and usually involves the lower thoracic part of the spinal cord, as in the present patient. 2 A GCT looks similar to an astrocytoma in MRI, which can be hypointense or isointense in T1-weighted images, hyperintense in T2-weighted images, and moderately enhanced in gadolinium-enhanced images. The initial imaging typically reveals a mass, 2 but in rare cases it can only show spinal cord atrophy, which is an early sign of intramedullary germinoma.…”

“…2 A GCT looks similar to an astrocytoma in MRI, which can be hypointense or isointense in T1-weighted images, hyperintense in T2-weighted images, and moderately enhanced in gadolinium-enhanced images. The initial imaging typically reveals a mass, 2 but in rare cases it can only show spinal cord atrophy, which is an early sign of intramedullary germinoma. 3 These features make a radiological diagnosis difficult, and hence biopsy or resection with pathohistological examination is necessary.…”

“…An intracranial germinoma is a radiosensitive tumor whose cure rate is more than 90% with radiotherapy alone, 5 which is also the case for intramedullary germinoma. 2 The response to treatment is highly favorable, but an early diagnosis of germinoma is critical for a good prognosis. It is particularly important to note that early imaging findings might not be apparent, as was the case in the present patient.…”

Successful treatment of mixed yolk sac tumor and mature teratoma in the spinal cord: case report

“…Primary cluding 27 cases of pure germinoma (71%), 5 cases of germinoma with syncytiotrophoblastic giant cells (STGCs) (13%), 3 cases of mixed germ cell tumor (8%), 2 cases of yolk sac tumor (5%), and 1 case of embryonal carcinoma (3%). 1,2,5,7,9,11,12,16,[19][20][21] The proportion of NGGCTs among primary spinal germ cell tumors appears slightly lower than that among intracranial germ cell tumors. 15 Among these 38 cases, 25 (66%) involved Japanese patients and 19 (50%) involved male patients.…”