When a dermatopathologist encounters the ultra-rare: A case series of superficial soft tissue/cutaneous myxopapillary ependymomas Journal Article
| Authors: | Chatzopoulos, K.; Hytiroglou, P.; Charville, G. W.; Toland, A. M. S.; Martinez-Lage, M.; Cimino, P. J.; Rosenblum, M. K.; Linos, K. |
| Article Title: | When a dermatopathologist encounters the ultra-rare: A case series of superficial soft tissue/cutaneous myxopapillary ependymomas |
| Abstract: | Myxopapillary ependymoma (MPE) is an uncommon variant of ependymoma, almost exclusively seen in conus medullaris or filum terminale. MPE can be diagnostically challenging, especially when arising extra-axially. Here we report 5 cases of superficial soft tissue/cutaneous MPE, identified across three tertiary institutions. All patients were female and three of them (3/5, 60%) were children (median age 11 years, range 6–58 years). The tumors presented as slow-growing masses of the sacrococcygeal subcutaneous soft tissues, occasionally identified after minor trauma and clinically favored to be pilonidal sinuses. Imaging showed no neuraxis connection. Macroscopically, tumors were well-circumscribed, lobulated, and solid and microscopically they exhibited typical histopathology of MPE, at least focally. Two of the tumors (2/5, 40%) showed predominantly solid or trabecular architecture with greater cellular pleomorphism, scattered giant cells, and increased mitotic activity. All tumors (5/5, 100%) showed strong diffuse immunohistochemical expression of GFAP. One tumor clustered at the category “ependymoma, myxopapillary” by methylome analysis. Two patients (2/5, 40%) had local recurrence at 8 and 30 months after the initial surgery. No patients developed metastases during the follow-up period (median 60 months, range 6–116 months). Since a subset of extra-axial MPEs behaves more aggressively, timely and accurate diagnosis is of paramount importance. © 2023 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd. |
| Keywords: | adolescent; adult; child; middle aged; young adult; methylation; case report; pathology; spinal cord tumor; cutaneous; ependymoma; spinal cord neoplasms; cauda equina; superficial; humans; gfap; human; male; female; myxopapillary ependymoma |
| Journal Title: | Journal of Cutaneous Pathology |
| Volume: | 51 |
| Issue: | 1 |
| ISSN: | 0303-6987 |
| Publisher: | John Wiley & Sons |
| Date Published: | 2024-01-01 |
| Start Page: | 20 |
| End Page: | 29 |
| Language: | English |
| DOI: | 10.1111/cup.14475 |
| PUBMED: | 37317818 |
| PROVIDER: | scopus |
| PMCID: | PMC10721733 |
| DOI/URL: | |
| Notes: | The MSK Cancer Center Support Grant (P30 CA008748) is acknowledged in the PDF -- Corresponding author is MSK author: Konstantinos Linos -- Source: Scopus |
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