Spinal Cord Lesion

Clinical History

This 45-year-old patient presented with a 2-month history of right upper extremity pain. MRI demonstrated the presence of a well-circumscribed partially cystic C8-T1 intramedullary mass.

Question

Which of the following diagnoses is most appropriate based on images seen in Figures #1 and #2?

A. Meningioma

B. Schwannoma

C. Metastatic Carcinoma

D. Ependymoma

Answer- D. Ependymoma

Figure 1

Hematoxylin and eosin stained sections demonstrate the presence of a glial neoplasm with scattered variably sized cystic and/or lumen type spaces lined by cuboidal cells, as well as areas where lesional cells are arranged around blood vessels and show radially oriented cytoplasmic processes. Only a rare mitotic figure is seen.

Positive immunostaining of cytoplasmic processes for GFAP is present.

Figure 2

Immunostains for EMA and D2-40 (podoplanin) show dot-like to ring-like areas of cytoplasmic staining in many of the lesional cells. These represent intracytoplasmic microlumen type structures.

The morphologic and immunophenotypic features are consistent with histopathologic classification of this neoplasm as an ependymoma (answer D).

Reference(s)/Additional Reading

• Ellison DW, Aldape KD, Capper D, Fouladi M, Gilbert MR, Gilbertson RJ, Hawkins C, Merchant TE, Pajtler K, Venneti S, Louis DN. cIMPACT-NOW update 7: advancing the molecular classification of ependymal tumors. Brain Pathol. 2020 Sep;30(5):863-866. doi: 10.1111/bpa.12866. Epub 2020 Jun 23. PMID: 32502305; PMCID: PMC8018155.
• Kresbach C, Neyazi S, Schüller U. Updates in the classification of ependymal neoplasms: The 2021 WHO Classification and beyond. Brain Pathol. 2022 Jul;32(4):e13068. doi: 10.1111/bpa.13068. Epub2022 Mar 21. PMID: 35307892; PMCID: PMC9245931.