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Case Report Open Access

Primary intracranial extraskeletal myxoid chondrosarcoma in a teenager with unusual frontal location and surgical complications: A case report and literature review

  • Authors:
    • Helu Wang
    • Fang Wang
    • Hongtao Zhang
  • View Affiliations / Copyright

    Affiliations: School of Clinical Medicine, Shandong Second Medical University, Weifang, Shandong 261000, P.R. China, Department of Pathology, The Affiliated Yantai Yuhuangding Hospital of Qingdao University, Yantai, Shandong 264000, P.R. China, Department of Neurosurgery, Yantai Yuhuangding Hospital, Yantai, Shandong 264000, P.R. China
    Copyright: © Wang et al. This is an open access article distributed under the terms of Creative Commons Attribution License.
  • Article Number: 84
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    Published online on: December 19, 2025
       https://doi.org/10.3892/ol.2025.15437
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Abstract

Primary intracranial extraskeletal myxoid chondrosarcoma (EMC) is rare and diagnostically challenging, with only sporadic pediatric and adolescent cases reported. The current study describes the case of a 17‑year‑old male presenting with an acute headache, nausea and emesis. Neuroimaging revealed a 7.2x5.2‑cm hemorrhagic mass in the right frontal lobe with involvement of the anterior skull base. A gross total resection was achieved. Histopathology confirmed EMC with high‑grade features, including loss of integrase interactor 1 (INI1) expression and a Ki‑67 labeling index of 80%. The postoperative course was complicated by bacterial meningitis, which resolved with antibiotics. Adjuvant radiotherapy was delivered to the tumor bed and involved the dura at 66 Gy in 33 fractions without major toxicity. At the 3‑month follow‑up, there was no evidence of recurrence. This presentation, namely frontal‑lobe localization in an adolescent and an aggressive immunophenotype with INI1 loss, is uncommon for intracranial EMC, which is more frequently described in middle‑aged patients and posterior fossa sites. Molecular subtyping, which can inform targeted therapy selection, was not performed due to financial constraints. The diagnosis relied on multimodal integration of imaging, histology and immunohistochemistry; key mimics were disfavored by negative staining for glial fibrillary acidic protein, epithelial membrane antigen, desmin and S‑100 protein. Given the pathological risk profile and the anticipated limited benefit of chemotherapy, systemic therapy was not pursued. The patient's long‑term prognosis remains uncertain, and imaging surveillance is planned at 3‑ to 6‑month intervals for 2 years and annually thereafter. This case highlights the importance of considering EMC in the differential diagnosis of young patients presenting with aggressive, hemorrhagic intracranial masses, particularly those with INI1 loss, and underscores the need for multimodal diagnostic approaches in such rare entities.
View Figures

Figure 1

Preoperative magnetic resonance
imaging. (A) Axial T1WI demonstrating a quasi-round, slightly
hypointense lesion in the right frontal lobe with ill-defined
margins. (B) Axial T1WI revealing heterogeneous intralesional
signal intensity. (C) Axial T2WI showing a slightly hyperintense
lesion obscuring the normal gray-white matter differentiation. (D)
Axial T2WI illustrating mild lobulation at the lesion margin. (E)
Axial contrast-enhanced T1WI demonstrating irregular annular and
nodular enhancement. (F) Coronal contrast-enhanced T1WI revealing a
central non-enhancing necrotic core with adjacent linear dural
enhancement. WI, weighted imaging.

Figure 2

Preoperative CT. (A) Axial
non-contrast CT showing a mixed-density mass in the right frontal
lobe (~5.0×5.2 cm). (B) The lesion has well-defined borders with
peripheral annular hyperdensity and internal punctate
calcifications. (C) Extensive surrounding parenchymal hypodensity,
consistent with vasogenic edema, resulting in a significant
leftward midline shift. CT, computed tomography.

Figure 3

Histopathology and
immunohistochemistry findings. Hematoxylin-eosin staining images at
(A) ×10 and (B) ×40 magnification demonstrating a predominantly
nodular growth pattern with variable cellular density. Hypocellular
regions contain abundant myxoid stroma with tumor cells arranged in
cord-like and reticular patterns. Hypercellular regions show
diffusely distributed cells, predominantly oval to short
spindle-shaped in morphology, with pronounced pleomorphism and
significant atypia. Mitotic figures are easily observed, with
prominent small red nucleoli. The cytoplasm is scant, with some
cells displaying vacuolation. Immunohistochemistry for cytokeratin
showing negative staining at (C) ×10 and (D) ×40 magnification.
Immunohistochemistry for S-100 showing negative staining at (E) ×10
and (F) ×40 magnification. Immunohistochemistry for desmin showing
negative staining at (G) ×10 and (H) ×40 magnification.
Immunohistochemistry for epithelial membrane antigen showing
negative staining at (I) ×10 and (J) ×40 magnification.
Immunohistochemistry for glial fibrillary acidic protein showing
negative staining at (K) ×10 and (L) ×40 magnification.
Immunohistochemistry for vimentin showing positive staining at (M)
×10 and (N) ×40 magnification. Arrows indicate representative tumor
areas of staining. All sections were obtained from the resected
intracranial mass, and are formalin-fixed paraffin-embedded
tissues, with hematoxylin counterstain. Scale bar, 100 µm.

Figure 4

Histopathology and
immunohistochemistry findings. Immunohistochemistry images for
smooth muscle actin showing negative staining at (A) ×10 and (B)
×40 magnification. immunohistochemistry for INI-1 showing complete
loss of nuclear expression (negative) at (C) ×10 and (D) ×40
magnification. Immunohistochemistry for Ki-67 demonstrating a
markedly elevated proliferation index, reaching ~80% in the most
active regions, at (E) ×10 and ×40 (F) magnification.
Immunohistochemistry for synaptophysin showing negative staining at
(G) ×10 and (H) ×40 magnification. Arrows indicate representative
tumor areas of staining. All sections were obtained from the
resected intracranial mass, and are formalin-fixed
paraffin-embedded tissue, with hematoxylin counterstain. Scale bar,
100 µm.

Figure 5

Histopathology and
immunohistochemistry findings. Immunohistochemistry images for CD34
showing negative staining at (A) ×10 and (B) ×40 magnification.
Immunohistochemistry images for IDH-1 showing negative staining at
(C) ×10 and (D) ×40 magnification. Immunohistochemistry images for
NKX2.2 showing negative staining at (E) ×10 and (F) ×40
magnification. Immunohistochemistry images for p53 showing positive
nuclear expression (+, 60%) at (G) ×10 and (H) ×40 magnification.
Immunohistochemistry images for SOX-10 showing negative staining at
(I) ×10 and (J) ×40 magnification. Immunohistochemistry images for
STAT6 showing negative staining at (K) ×10 and (L) ×40
magnification. Arrows indicate representative tumor areas of
staining. All sections were obtained from the resected intracranial
mass and are formalin-fixed paraffin-embedded tissues, with
hematoxylin counterstain. Scale bar, 100 µm.

Figure 6

Follow-up magnetic resonance imaging
at 3 months postoperatively. (A) T1-weighted image showing an
irregularly shaped surgical cavity in the right frontal region with
hypointense signal; discontinuity of the right frontal bone is
noted, compatible with a postoperative change/bone defect. (B)
T2-weighted image showing a hyperintense signal within the surgical
cavity and patchy edema in the adjacent right frontal lobe
parenchyma. (C) Contrast-enhanced T1-weighted image showing
thickening and enhancement of the cavity margin and adjacent dura
mater, with focal nodular enhancement.
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Copy and paste a formatted citation
Spandidos Publications style
Wang H, Wang F and Zhang H: Primary intracranial extraskeletal myxoid chondrosarcoma in a teenager with unusual frontal location and surgical complications: A case report and literature review. Oncol Lett 31: 84, 2026.
APA
Wang, H., Wang, F., & Zhang, H. (2026). Primary intracranial extraskeletal myxoid chondrosarcoma in a teenager with unusual frontal location and surgical complications: A case report and literature review. Oncology Letters, 31, 84. https://doi.org/10.3892/ol.2025.15437
MLA
Wang, H., Wang, F., Zhang, H."Primary intracranial extraskeletal myxoid chondrosarcoma in a teenager with unusual frontal location and surgical complications: A case report and literature review". Oncology Letters 31.2 (2026): 84.
Chicago
Wang, H., Wang, F., Zhang, H."Primary intracranial extraskeletal myxoid chondrosarcoma in a teenager with unusual frontal location and surgical complications: A case report and literature review". Oncology Letters 31, no. 2 (2026): 84. https://doi.org/10.3892/ol.2025.15437
Copy and paste a formatted citation
x
Spandidos Publications style
Wang H, Wang F and Zhang H: Primary intracranial extraskeletal myxoid chondrosarcoma in a teenager with unusual frontal location and surgical complications: A case report and literature review. Oncol Lett 31: 84, 2026.
APA
Wang, H., Wang, F., & Zhang, H. (2026). Primary intracranial extraskeletal myxoid chondrosarcoma in a teenager with unusual frontal location and surgical complications: A case report and literature review. Oncology Letters, 31, 84. https://doi.org/10.3892/ol.2025.15437
MLA
Wang, H., Wang, F., Zhang, H."Primary intracranial extraskeletal myxoid chondrosarcoma in a teenager with unusual frontal location and surgical complications: A case report and literature review". Oncology Letters 31.2 (2026): 84.
Chicago
Wang, H., Wang, F., Zhang, H."Primary intracranial extraskeletal myxoid chondrosarcoma in a teenager with unusual frontal location and surgical complications: A case report and literature review". Oncology Letters 31, no. 2 (2026): 84. https://doi.org/10.3892/ol.2025.15437
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