Introduction: Ependymomas arise from ependymal lining of the intracranial ventricles and spinal cord central canal. While tumor dissemination within cerebrospinal-fluid occurs in 10% of cases, extraneural metastasis is rare. Previously reported sites include lymph nodes (LNs), scalp, bone, lungs, pleura, mediastinum, and liver.
Methods: We present a supratentorial ependymoma that metastasized to bilateral cervical LNs. A comprehensive literature search was performed using PubMed, Web of Science, and Scopus from 1950-2018 to find cases involving cervical LNs. Non-English publications were excluded.
Results: A 17-year-old female initially presented to an outside institution with headaches and vomiting. MRI brain revealed a large cystic lesion with nodularity in the left frontal lobe adjacent to the lateral ventricle. After gross total resection, pathology showed anaplastic ependymoma (WHO Grade III) with a high MIB-1 index. She subsequently received adjuvant radiotherapy at 59.4 Gy, and over the next three years, 25 contrast-enhancing intracranial lesions were treated with gamma knife radiosurgery. Five years after initial diagnosis, she presented to our institution with painless bilateral neck masses. MRI neck showed several enlarged, contrast-enhancing right level II and left level Va cervical LNs with corresponding hypermetabolic activity on PET/CT. Core needle biopsy revealed papillary ependymoma (WHO Grade II) with a Ki-67 of 5-10%.
Of the 14 cases found in our literature search, seven cases presented solely with cervical LN involvement, and one case had a concurrent mediastinal lesion. Four cases presented with scalp metastases within the craniotomy scar before cervical LN involvement; the remaining two cases presented with simultaneous scalp and cervical LN metastases.
Conclusions: Extracranial metastasis should be considered in the differential diagnosis of neck masses in patients with a history of intracranial ependymoma. Furthermore, given that our literature review showed that scalp involvement often accompanies cervical lymph node metastases, the scalp near the craniotomy scar should be carefully examined for metastatic nodules.
Patient Care: Even though extracranial metastasis of ependymoma is rare, it should be considered in the differential diagnosis of cervical lymphadenopathy in patients with a history of intracranial ependymoma. Our literature review further showed that scalp metastasis often accompanies cervical lymph node involvement; as such, the scalp surrounding the craniotomy scar should be carefully examined for metastatic nodules. Given the rarity of extracranial metastasis of ependymoma, there is no accepted treatment regimen, and the mechanism by which metastasis occurs is unknown. Our future research will define the natural history of this condition, discuss potential mechanisms, and review treatment strategies.
Learning Objectives: After reading this poster, participants should be able to:
1. Consider extracranial metastasis of ependymoma in the differential diagnosis of patients presenting with painless neck masses and a previous history of intracranial ependymoma.
2. Discuss characteristics of cases of intracranial ependymoma metastasizing to cervical lymph nodes.
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