Primary extradural meningiomas: a report on nine cases and review of the CT-era literature

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Object. Primary meningiomas arising outside the intracranial compartment (primary extradural meningiomas [PEMs]) are rare tumors. To develop a better understanding of these tumors and to establish a comprehensive classification scheme for them, the authors analyzed a series of patients treated at the M. D. Anderson Cancer Center (MDACC) and reviewed all cases reported in the English-language literature since the inception of the use of computerized tomography (CT) scanning.

Methods. Clinical records, results of radiographic studies, and histological slides were reviewed for all cases of PEM at MDACC. Demographic features, symptoms, tumor location, histological grade, and patient outcome were assessed in all cases. A comprehensive literature search identified 168 PEMs in 142 patients reported during the CT era. These reports were also analyzed for common features. Tumors for both data sets were classified as purely extracalvarial (Type I), purely calvarial (Type II), and calvarial with extracalvarial extension (Type III). Type II and Type III tumors were further categorized as convexity (C) or skull base (B) lesions.

The incidence of PEMs at MDACC was 1.6%, which was consistent with the rate reported in the literature. In both data sets, the male/female ratio was nearly 1:1. The most common presenting symptom was a gradually expanding mass. The age of patients at diagnosis of PEM was bimodal, peaking during the second decade and during the fifth to seventh decades. In all MDACC cases and in 90% of those reported in the literature the PEMs were located in the head and neck. The majority of tumors originated in the skull (70%).

In the MDACC series and in the literature review, the majority (67% and 89%, respectively) of tumors were histologically benign. Although fewer PEMs were malignant or atypical (33% at MDACC and 11% in the literature), their incidence was higher than that observed for primary intracranial meningiomas. Distant metastasis was not a common feature reported for patients with PEMs (6% in the literature).

Outcome data were available in 96 of the cases culled from the CT-era literature. The combination of the MDACC data and the data obtained from the literature demonstrated that patients with benign Type IIB or Type IIIB lesions were more likely to experience recurrence than patients with benign Type IIC or Type IIIC tumors (26% compared with 0%, p < 0.05). The more aggressive atypical and malignant tumors were associated with a statistically significant higher death rate (29%) relative to benign tumors (4.8% death rate, p < 0.004).

Conclusions. Defining a tumor as a PEM is dependent on the tumor's relation to the dura mater and the extent and direction of its growth. Classification of PEMs as calvarial or extracalvarial and as convexity or skull base lesions correlates well with clinical outcome.

Article Information

Address reprint requests to: Frederick F. Lang, M.D., Department of Neurosurgery, M. D. Anderson Cancer Center, The University of Texas, 1515 Holcombe Boulevard, Box 064, Houston, Texas 77030–4095. email: flang@notes.mdacc.tmc.edu.

© AANS, except where prohibited by US copyright law.

Headings

Figures

  • View in gallery

    Case 3. Images documenting purely calvarial basal PEMs (Type IIB). A: A CT scan (bone window) revealing a left orbitofrontal meningioma with sclerotic bone. B: A CT scan demonstrating a left posterior medial sphenoid tumor (arrow) with radiographic features similar to the lesion shown in A, but without any anatomical connection. C and D: Axial (C) and coronal (D) T1-weighted MR images obtained after gadolinium injection demonstrating the lack of a soft-tissue component. In addition, the dura mater does not enhance, confirming the purely extradural nature of this meningioma.

  • View in gallery

    Case 5. A: Sagittal T1-weighted MR image revealing PEM of the nasal cavity and paranasal sinuses (arrow). The PEMs in this location were classified as calvarial—extracalvarial, basal (Type IIIB). B: Axial CT scans demonstrating erosion of the nasal bones.

  • View in gallery

    Case 4. Computerized tomography scans revealing a calvarial—extracalvarial convexity (Type IIIC) PEM. Left: Contrast-enhanced CT scan demonstrating soft-tissue and bone involvement without dural enhancement. Biopsy specimens obtained at surgery confirmed that the dura was not involved with the tumor. Right: A CT scan (bone window) demonstrating a sclerotic bone typical of PEMs arising in the calvaria.

  • View in gallery

    Bar graph showing the incidence of PEMs stratified by patient age. Data for the nine patients treated at MDACC were combined with those for the 142 patients described in the literature. Similar to PIMs, many of these tumors appear during the fifth, sixth, and seventh decades of life. However, a distinct incidence peak during the second decade of life differentiates the behavior of PEMs from that of PIMs.

  • View in gallery

    Schematic drawing showing the distribution of PEMs by anatomical site in 142 patients (168 tumors) reported in CT-era literature and in the nine patients (10 tumors) treated at MDACC.

  • View in gallery

    Case 2. Intra- and postoperative photographs and photomicrograph obtained in a patient who underwent orbitofrontal craniotomy and en bloc resection of a PEM. A: Gross specimen after en bloc resection of this purely calvarial meningioma. B: Histological specimen showing meningioma with sclerotic bone. H & E, original magnification × 100. C: Intraoperative view following resection. The histological analysis of the underlying dura was negative for tumor. Arrow identifies the site of dural biopsy.

  • View in gallery

    Outcome data obtained in patients treated at MDACC. All but three patients harbored benign tumors. All patients with benign tumors were treated with surgery alone, except the patient in Case 9, who received radiation therapy. The patient in Case 8 was diagnosed with an atypical tumor and was treated surgically. Two patients (Cases 1 and 6) had malignant tumors and both experienced metastases to a distant site. Both patients were treated aggressively with surgery, chemotherapy, and radiation therapy. Double marks in time line (/ /) indicate a break in time. GTR = gross-total resection; NED = no evidence of disease; STR = subtotal resection.

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