Browse

You are looking at 1 - 2 of 2 items for :

  • Craniofacial x
  • Refine by Access: all x
Clear All
Open access

Intraosseous meningioma, a rare presentation of a common brain tumor: illustrative case

Sherif Elwatidy, Abdulaziz Alanazi, Rahaf F. Alanazi, and Khulood K. Alraddadi

BACKGROUND

The subset of extradural meningiomas arising from bone is called primary intraosseous meningioma. The present article outlines the clinical presentation, investigation, surgical management, and clinical outcomes of a challenging case of intraosseous meningioma.

OBSERVATIONS

A 27-year-old male presented with new-onset seizure of 3 years’ duration and growing painless hard swelling involving the left frontoparietal bone, the medial aspect of the left orbit, the nasal bone, and the temporal bone. Brain computed tomography showed a diffuse intradiploic space osseous lesion with a ground-glass appearance. The patient underwent bifrontal craniotomy and tumor excision. The histopathological sections showed the presence of classic whorls of meningothelial cells around reactive bony tissue suggestive of intraosseous meningioma. Postoperatively, the patient was neurologically intact, and he was discharged in a stable condition. The outcome of this case was satisfactory.

LESSONS

The treatment of primary intraosseous meningioma is wide surgical excision together with the involved dura and any intradural tumor. The location of the tumor determines the approach and the amount of resection, keeping in mind preservation of function. The use of adjuvant therapy is reserved for cases of tumor recurrence or an atypical type.

Open access

Three decades of progress from surgery to medical therapy for isolated neuroaxis BRAF V600E–positive Langerhans cell histiocytosis management: illustrative case

Nallammai Muthiah, Kamil W. Nowicki, Jennifer L. Picarsic, Michael P. D’Angelo, Daniel F. Marker, Edward G. Andrews, Edward A. Monaco III, and Ajay Niranjan

BACKGROUND

“Langerhans cell histiocytosis” (LCH) is a term that encompasses single-system or multisystem disorders traditionally characterized by a proliferation of clonal CD1a+/CD207+ myeloid-derived histiocytes. In most cases of LCH, mitogen-activated protein kinase (MAPK) pathway somatic mutations lead to near universal upregulation of phosphorylated extracellular signal-regulated kinase expression. The clinical manifestations of LCH are numerous, but bone involvement is common. Intracranial lesions, especially as isolated manifestations, are rare.

OBSERVATIONS

The authors presented the case of a long-term survivor of exclusive intracranial LCH that manifested with isolated craniofacial bone and intraparenchymal central nervous system recurrences, which were managed with 3 decades of multimodal therapy. The patient was initially diagnosed with LCH at age 2 years, and the authors documented the manifestations of disease and treatment for 36 years. Most of the patient’s treatment course occurred before the discovery of BRAF V600E. Treatments initially consisted of chemotherapy, radiosurgery, and open resections for granulomatous LCH lesions. Into young adulthood, the patient had a minimal disease burden but still required additional radiosurgical procedures and open resections.

LESSONS

Surgical treatments alleviated the patient’s immediate symptoms and allowed for tumor burden control. However, surgical interventions did not cure the underlying, aggressive disease. In the current era, access to systemic MAPK inhibitor therapy for histiocytic lesions may offer improved outcomes.