Aggressive rhabdoid meningioma with osseous, papillary and chordoma-like appearance

Pinar Karabagli1, Hakan Karabagli, Guler Yavas

  • 1Department of Pathology, Faculty of Medicine, Selcuk University, Konya, Turkey.

Insights

Rhabdoid meningioma (RM), a rare WHO grade III tumor, presents aggressive behavior with frequent recurrences and poor outcomes. This case highlights the challenges in managing this aggressive brain tumor despite multiple resections.

Area of Science:

  • Neuro-oncology
  • Pathology
  • Neurosurgery

Background:

  • Meningiomas are common primary intracranial tumors, typically benign but can be malignant (WHO grade II/III).
  • Rhabdoid meningioma (RM) is a rare, aggressive WHO grade III variant with a poor prognosis.
  • Clinical management of RM is challenging due to its aggressive nature and limited treatment options.

Observation:

  • A case of recurrent aggressive left occipital parasagittal rhabdoid meningioma is presented.
  • The patient underwent four tumor resections over five years, initially declining radiation therapy.
  • Histopathology revealed rhabdoid features with metaplastic, papillary, and chordoid differentiation.

Findings:

  • The patient experienced local recurrences, invasion, and ultimately died of progressive disease six months after the fourth surgery.
  • Rhabdoid meningioma demonstrates aggressive behavior, including local recurrence, leptomeningeal dissemination, and metastasis.
  • Despite aggressive surgical management, the prognosis for rhabdoid meningioma remains poor.

Implications:

  • This case underscores the aggressive clinical course and poor prognosis associated with rhabdoid meningiomas.
  • Further research is needed to determine the efficacy of adjuvant therapies for this rare malignant meningioma subtype.
  • Understanding the clinical presentation, radiological features, and pathological findings is crucial for managing rhabdoid meningioma.