Neurosurg. Rev. 13 (1990) 147-154

Spinal metastases o f intracranial ependymomas. Four case reports and review o f the literature Ralf-Ingo Ernestus and Ortwin Wilcke Department of Neurosurgery, University of Cologne, Cologne, West Germany

Abstract

2 Results

Intracranial ependymomas tend to spread along on the liquor pathways and thus to seed subarachnoid metastatic implants. According to autopsy data, spinal seeding can be expected in 25% of cases subsequent to surgery of the primary tumor.

A review of 125 patients with intracranial ependymomas (subependymoma excluded) treated at the Department of Neurosurgery, University Clinics of Cologne, West Germany, from 1951 to 1988 revealed spinal seeding in four cases (Table I). They represent 3.3% of all 125 patients 5.0% of the 80 patients who survived surgery of the primary tumor for more than 30 days.

Analysis of four of our own cases (out of 125 primary intracranial ependymomas) together with those described in the literature suggests clinical evidence of seeding in 75% of patients. 47 of 75 metastases originated from malignant infratentorial ependymomas. Malignant ependymomas metastasize earlier than benign ones. They are characterized by disseminated seeding of tumor implants along the entire spinal subarachnoid space. The median survival time after diagnosis of seeding was 6 months. 80% of all patients died within the first 12 months following diagnosis. The necessity of prophylactic spinal radiation therapy in the course of the initial treatment of intracranial ependymomas has not yet been proven.

Keywords: Ependymomas, gliomas, spinal metastases.

1 Introduction Due to their close contact with the ventricular system, neuroepithelial tumors, especially intracranial ependymomas subsequent to medulloblastomas tend to seed in liquor pathways and thus to generate spinal implantation of tumor cells. These metastases occur predominantly in the subarachnoid space and only exceptionally in the medulla of the spinal cord [17]. Evidence of tumor cells in the cerebrospinal fluid does not necessarily indicate spinal seeding [34]. 9

1990 by Walter de Gruyter & Co. Berlin - New York

Autopsy of the entire spinal cord was performed routinely in medulloblastoma but not in intracranial ependymoma patients. All patients with spinal metastases were younger than 30 years at the time of the initial treatment. Two primary ependymomas were found in supratentorial, two in infratentorial locations. According to the grading of malignancy proposed by the W H O in 1976 [40], the original tumor was classified as Grade 3 in three cases. In the fourth case, there was a local recurrence of a primary ependymoma Grade 2 which had already increasing malignancy before diagnosis of multiple spinal implants. The tumor dose of an initial postoperative radiation therapy, which never included the spinal cord, was under 40 Gy in all cases. In our series all metastases appeared within the first five postoperative years and were clinically manifest. Nevertheless, only one patient with a metastatic implant in the region of the cauda equina could be treated curatively by a combination of surgery, irradiation, and chemotherapy.

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7.3% 92.7%

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72.7% 27.3%

(n = 36 + 4) (n = 15)

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Neurosurg. Rev. 13 (1990)

Ernestus & Wilcke, Spinal metastases of intracranial ependymomas

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Table III. Frequency of spinal seeding Author

Sagerman et al. J~nisch et al. Tarlov, Davidoff Kim, Fayos Svien et al. Fokes, Earle Houtteville Renaudin et al. Salazar et al. Onoyama et al. Pierre-Kahn et al. Bloom Chin et al. de Tribolet et al. Marks, Adler Wentworth, Birdsell Boudreau Glanzrnann et al. Hahn et ak Phillips et al. Shuman et al. Garrett, Simpson Namer et al. Kricheff et al. Bouchard Barone, Elvidge Sheline Coulon, Till

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all cases with initial postoperative radiation therapy all cases with post-mortem examination only children only supratentorial ependymomas 3 papillomas of the choroid plexus included

its grade of malignancy (Figure 1). Thus, infratentorial ependymomas [3, 6, 7, 12, 22, 28, 29, 33, 36, 39], which are usually in contact with the ventricular system, and malignant tumors [3, 6, 8, 10, 11, 12, 30] seed most frequently. 47 of 75 metastases originated from a malignant infratentorial ependymoma.

of 31 symptomatic spinal metastases [8, 10, I6, 19, 23, 26, 30, 32, 37] reveals a median interval between the operation of the primary neoplasm and the diagnosis of seeding of seven months for malignant ependymomas (range from 2 to 89 months) in contrast to 25 months for tumors which appear to be benign (Range from 12 to 108 months).

In general, ependymomas containing histologic features of increased biologic activity metastasize earlier than benign ones (Figure 2). Thus, analysis

Localization of spinal metastatic deposits could be identified in 46 cases [7, 8, 10, 13, 15, 17, 19, 20, 21, 23, 25, 26, 32, 35, 36, 37]. Despite an

Neurosurg. Rev. 13 (1990)

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increased frequency in the thoracic region (35%) a preference for certain spinal segments could not be established (Figure 3). Ependymomas do tend to multiple seeding of tumor implants: In eight cases metastases were disseminated along the whole spinal subarachnoid space. Prognosis of spinal metastasis from intracranial ependymoma has been poor (Figure 4). Surgery was practicable in fewer than 25% of cases. We analysed the survival times and rates of 25 patients who had received various initial treatment [8, 10,

Figure 1. Primary localization and grade of malignancy in 75 intracranial ependymomas with spinal seeding. 71 cases from the literature [3, 7, 8, 10, 12, 14, 16, 19, 23, 26, 28, 29, 30, 32, 34, 35, 36, 37, 38]. 4 own cases.

16, 25, 26, 30, 32, 37]: The median survival time after diagnosis of seeding was six months. 80% of all patients died within the first 12 months following diagnosis. Only one patient - Case 1 in our series - survived longer than five years.

4 Conclusions

1. According to autopsy data, frequency of spinal seeding from intracranial ependymoma is about 25%. Neurosurg. Rev. 13 (1990)

Ernestus & Wilcke, Spinal metastases of intracranial ependymomas

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27 cases from the literature [8, 10, 16, 19, 23, 26, 30, 32, 37]. 4 own cases.

2. According to reports in the literature, nearly

The intervals between initial treatment and diagnosis of seeding suggest that these check-ups should take place once a year for benign and twice a year for malignant ependymomas, Many authors favor a prophylactic irradiation of the entire central nervous system axis in the course of the initial treatment of intracranial ependymal tumors. However, it has not yet been proven that craniospinal radiation therapy has an effect on frequency of seeding and thus on prognosis. To prove this, a complete documentation and compilation of the individual follow-ups should be carried out.

75% of all metastases gain clinical significance. 3. 80% of all patients with spinal metastases die over the first 12 months subsequent to diagnosis.

Spinal seeding of intracranial ependymoma is associated above all with primary infratentorial site and more highly malignant neoplasms. Thus, in these cases periodical examinations of the spinal

cord should be carried out, if possible using nuclear magnetic resonance tomography (NMRT). Neurosurg. Rev. 13 (1990)

152

Ernestus & Wilcke, Spinal metastases of intracranial ependymomas

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Figure 3. Localization of spinal metastases. 42 cases from the literature [7, 8, 10, 13, t 5, 17, 19, 20,

21, 23, 25, 26, 32, 35, 36, 37]. 4 own cases.

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Neurosurg. Rev. t3 (1990)

Ernestus & Wilcke, Spinal metastases of intracranial ependymomas 100

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n=21

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Neurosurg. Rev. 13 (1990)