Myxopapillary ependymoma with anaplastic features

Abstract

A case of myxopapillary ependymoma with anaplastic features in 15-year-old boy is reported. The tumor was located in the intradural space extending to the 12th thoracic to 2nd lumbar vertebral level. It was excised with the accompanying spinal arch of the T12 to L2 vertebra. At operation, the tumor was not attached to the surrounding soft and bony tissues. The tumor, measuring 49 x 19 x 15 mm, was brownish-yellow in color and involved the conus medullaris and filum terminale. Histologically, the tumor was composed of biphasic features of a hypercellular papillary growth area and a hypocellular myxoid area. In the papillary growth area, ependymal rosettes and perivascular pseudorosettes were observed. These findings were consistent with those of a myxopapillary ependymoma, although multiple foci of punctate necrosis within the tumor and proliferation of endothelial cells showing glomeruloid structures were observed. Many mitotic figures were also observed. In addition, the Ki-67 labeling index of tumor cells was 10.1%. These findings are unusual for myxopapillary ependymoma, and therefore, it appeared that the diagnosis of myxopapillary ependymoma with anaplastic features was appropriate.

Clinicopathologic features of anaplastic myxopapillary ependymomas

“…Anaplasia within MPEs has been described as exceptional. Only five have been reported in the literature to our knowledge, most occurring within the pediatric setting with ages ranging from 7 months to 15 years and one occurring in an adult at 38 years of age . Two cases were intradural, two were subcutaneous sacrococcygeal lesions and one was anaplastic at the time of metastasis to an inguinal lymph node .…”

“…Only five have been reported in the literature to our knowledge, most occurring within the pediatric setting with ages ranging from 7 months to 15 years and one occurring in an adult at 38 years of age . Two cases were intradural, two were subcutaneous sacrococcygeal lesions and one was anaplastic at the time of metastasis to an inguinal lymph node . Malignant features included punctate necrosis, microvascular proliferation (MVP), increased Ki‐67 LI (80%, 70%, 40% and 10% respectively) and increased mitotic activity .…”

“…Two cases were intradural, two were subcutaneous sacrococcygeal lesions and one was anaplastic at the time of metastasis to an inguinal lymph node . Malignant features included punctate necrosis, microvascular proliferation (MVP), increased Ki‐67 LI (80%, 70%, 40% and 10% respectively) and increased mitotic activity . One anaplastic sacrococcygeal MPE occurred in the setting of Schinzel–Giedion syndrome with anaplastic ependymoblastic areas .…”

Anaplastic myxopapillary ependymoma in an infant: Case report and literature review

“…Up to this time, there has only been one prior report of these findings in a myxopapillary ependymoma (7). That prior case occurred in a 15-year-old boy and extended from T12 to L2.…”

Primary Glial Tumor of the Retina With Features of Myxopapillary Ependymoma

“…24,29,30 Anaplastic transformation is uncommon. 1 In MEs, conformation of perivascular pseudorosettes varies from case to case and from area to area of the same lesion. Some pseudorosettes are composed of flat or cuboidal cells perpendicularly arranged around a hyalinazed vessel and others, similar to those seen here consist of perivascular elongated cells with fibrillary cytoplasm and peripheral nucleus.…”