Primary extracranial meningioma of the mandible: A report of 2 cases and a review of the literature Anne Cale Jones, DDS,a and Paul D. Freedman, DDS,b San Antonio, Tex, and Flushing, NY THE UNIVERSITY OF TEXAS HEALTH SCIENCE CENTER AND THE NEW YORK HOSPITAL MEDICAL CENTER OF QUEENS
Three cases of primary extracranial meningioma arising in the jaws have been reported in the English-language literature. The purpose of this report is to document 2 additional cases that arose in the mandible. Both cases occurred in women and appeared as radiolucent lesions. Microscopic examination revealed an unencapsulated tumor composed of uniform spindle-shaped cells arranged in whorls and interconnecting fascicles. Occasional nuclear pseudoinclusions and psammoma bodies were identified. The tumor cells demonstrated immunohistochemical reactivity for epithelial membrane antigen and vimentin. Both cases were treated by surgical excision. Based on previously published examples in the jaws, these neoplasms do not appear to be aggressive. (Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2001;91:338-41)
CASE 1 In April 1999, a 74-year-old woman with swelling of the right anterior mandible was referred to an oral and maxillofacial surgeon. A periapical radiograph from April 1993 demonstrated a 4.0 × 3.0-mm well-defined radiolucency in the edentulous area mesial to the mandibular right cuspid (Fig 1, A). The patient’s medical history included a hip replacement in 1990 and an angioplasty in 1996. The patient was being treated for hypertension with lisinopril (Prinivil). Clinical examination revealed buccal expansion in the edentulous area of the mandibular right-central and rightlateral incisors. The buccal expansion was asymptomatic, and the patient denied having associated anesthesia or paresthesia. Radiographic examination revealed a 1.5-cm round, well-delineated radiolucency. The radiolucency extended to and involved the mesial surface and the apex of the mandibular right cuspid (Fig 1, B). The radiographic features were thought to be consistent with a cyst of the mandible, and an excisional biopsy was performed.
CASE 2 A 41-year-old woman was referred to an oral and maxillofacial surgeon for an evaluation of a 4.0 × 2.0-cm radiolucent lesion of the right posterior mandible. The lesion involved the apices of the first and second molars and extended from the mesial surface of the first molar to the distal surface of the third molar (Fig 2). The treatment consisted of an excisional biopsy along with an extraction of the third molar. Nonsurgical endodontic therapy was performed on the first and second molars. The
aAssociate Professor, The University of Texas Health Science Center at San Antonio. bDirector, Section of Oral Pathology, The New York Hospital Medical Center of Queens, Flushing. Received for publication Aug 18, 2000; returned for revision Oct 9, 2000; accepted for publication Oct 24, 2000. Copyright © 2001 by Mosby, Inc. 1079-2104/2001/$35.00 + 0 7/14/112947 doi:10.1067/moe.2001.112947
338
tissue obtained from the bony defect had a gelatinous consistency and was thought to represent an odontogenic myxoma.
HISTOPATHOLOGIC FINDINGS The histopathologic features of both cases were remarkably similar, consisting of a well-delineated but unencapsulated tumor composed of uniform spindle-shaped cells arranged in whorls and interconnecting fascicles (Fig 3, A and B). Tumor cells demonstrated oval-shaped nuclei with pale “wavy” eosinophilic cytoplasm and were set in a background of fibrillar ground substance. Occasionally, nuclei exhibited pseudoinclusions. In other areas, haphazardly arranged epithelioid cells with uniform nuclei were noted. No mitotic figures were identified in either case. Numerous psammoma bodies were evident in case 1 (Fig 3, C).
IMMUNOHISTOCHEMICAL FINDINGS Using avidin-biotin immunostaining techniques on paraffinembedded sections, we evaluated the neoplasms for vimentin (monoclonal; Dako Corporation, Carpinteria, Calif), epithelial membrane antigen (EMA; monoclonal; Dako), S-100 protein (monoclonal; Chemicon International, Temecula, Calif), smooth muscle actin (monoclonal; Dako), carcinoembryonic antigen (monoclonal; Dako), and antimelanoma (HMB-45; monoclonal; Enzo Diagnostics, Inc, Farmingdale, NY). The immunohistochemical stains demonstrated strong cytoplasmic staining for vimentin and strong membrane staining for EMA (Fig 4). S100 protein staining was negative in the tumor cells but positive in small nerve trunks at the periphery of the neoplasms. All other immunohistochemical markers were negative. The appropriate positive and negative controls were used.
DISCUSSION The first case of extracranial meningioma involving the mandible was reported by Landini and Kitano in 1992.1 Their case concerned a 48-year-old woman who had a large, well-delineated radiolucent lesion involving the edentulous area of the left posterior mandible and the retromolar region. The lesion had been present for more
ORAL SURGERY ORAL MEDICINE ORAL PATHOLOGY Volume 91, Number 3
Jones and Freedman 339
A Fig 2. Panoramic radiograph demonstrated a 4.0 × 2.0-cm radiolucent lesion of the right mandible. The lesion involved apices of first and second molars and extended from the mesial surface of the first molar to the distal surface of the third molar. A radiolucent artifact is noted in the maxillary premolar/molar area, and an osteophytic artifact is noted along inferior border of mandible in the angle region.
B Fig 1. A, Periapical radiograph from April 1993 demonstrated a 4.0 × 3.0-mm well-defined radiolucency involving the edentulous area mesial to the mandibular right cuspid. B, Periapical radiograph from April 1999 revealed a 1.5-cm round, well-delineated radiolucency in the edentulous area of mandibular right-central and right-lateral incisors. The radiolucency extended to and involved the mesial surface and the apex of the mandibular right cuspid.
than 1 year, and the patient denied having associated anesthesia or paresthesia. The treatment entailed resectioning the affected area and reconstruction with a bone graft. No recurrence was noted at a 2-year follow-up examination. Two cases of extracranial meningioma of the maxilla have been described in the English-language literature.2,3 On surgical exploration, neither case exhibited continuity with the cranial cavity. The first case, reported in 1987 by Simpson and Sneddon,2 involved a 63-year-old woman who had a 4.5 × 2.7 × 2.7-cm expansile lesion of the left posterior edentulous maxillary alveolus. The lesion had been present for many years, and radiographic examination demonstrated a mixed radiolucent-radiopaque mass thought to be consistent with a fibro-osseous lesion. The mass was treated through an excisional biopsy. The second case, reported in 1999 by Reddi et al,3 involved a 26-year-old woman with a 3-cm ill-defined mixed radiolucent-radiopaque mass of the right canine fossa. The
lesion had been present for several years. No provisional diagnosis was provided, but the lesion was treated by resectioning and was reconstructed with a bone graft. No recurrence was noted during a 2-year follow-up period. A summary of the clinical and radiographic characteristics of all cases of primary extracranial meningioma of the jaws is presented in Table I. The 3 previously reported cases of primary extracranial meningioma of the jaws occurred in women, as did both cases reported here. The patients’ ages ranged from 26 to 74 years, with a mean of 50.4 years. No distinct site of predilection was noted. Two of the cases occurred in the posterior mandible—1 on the left side and 1 on the right side—whereas a third case involved the right anterior mandible. The 2 maxillary cases involved the right canine fossa and the left posterior alveolus, respectively. Both maxillary cases appeared as mixed radiolucent-radiopaque lesions, whereas the 3 mandibular cases were entirely radiolucent. It is worth noting that the 2 extracranial meningiomas that arose in the maxilla demonstrated a mixed radiolucent-radiopaque pattern on radiographic examination, whereas the 3 mandibular cases did not. This opaque component cannot be explained entirely by the presence of calcification or psammoma-body formation because one of our cases (case 1) exhibited florid psammomabody deposition even though the lesion was entirely radiolucent. Therefore, it appears that a primary extracranial meningioma involving the jaws might appear as either a radiolucent or a mixed radiolucent-radiopaque lesion. Three of the 5 cases of extracranial meningioma occurred in an edentulous area of the jaws. This might
340 Jones and Freedman
ORAL SURGERY ORAL MEDICINE ORAL PATHOLOGY March 2001
A Fig 4. Immunohistochemical stains demonstrated strong membrane staining for EMA (case 1: EMA, original magnification ×400).
Table I. Clinical and radiographic characteristics of primary extracranial meningioma of the jaws (n = 5) Age (y)
B
C Fig 3. A, Uniform spindle-shaped cells arranged in whorls and interconnecting fascicles (case 2: hematoxylin-eosin stain, original magnification ×100). B, Uniform spindleshaped cells arranged in whorls (case 2: hematoxylin-eosin stain, original magnification ×400). C, Numerous psammoma bodies were evident (case 1: hematoxylin-eosin stain, original magnification ×400).
be a significant finding that serves to explain, in part, the pathogenesis of these neoplasms when they arise in the jaws. Landini and Kitano1 postulated that nerve injury during the extraction of teeth, in addition to chronic apical inflammation caused by dental caries, might be responsible for the stimulation and proliferation of ectopic arachnoid tissue, and thus for the development of extracranial meningioma.
Mean Range Location
Sex 50.4 26-74
Mandible Left posterior edentulous area1 Right anterior edentulous area Right posterior area Maxilla Left posterior edentulous area2 Right canine fossa area3
Female Male
5 0
Appearance Radiolucent Radiolucent Radiolucent Radiolucent-radiopaque Radiolucent-radiopaque
It is thought that primary extracranial meningioma arises from either displaced embryonic rests of arachnoid cells or from multipotential mesenchymal cells,4-7 whereas secondary extracranial meningioma arises as a direct extension of an intracranial mass.4,8 Neither of the cases reported in this article demonstrated extension from an intracranial tumor of the central neuraxis; both are thus examples of primary extracranial meningiomas. Meningiomas that arise as central tumors in the jaws demonstrate histopathologic features that are similar to their intracranial counterparts. Because a detailed description of the various histopathologic patterns seen in meningiomas has been presented elsewhere,9 our emphasis will be on the general histologic diagnosis of meningioma. These neoplasms are typically well-delineated, unencapsulated tumors composed of uniform spindle-shaped cells arranged in whorls, rosettes, and interconnecting fascicles. The tumor cells demonstrate oval-shaped nuclei, with or without pseudoinclusions, and a pale wavy eosinophilic cytoplasm. The tumor cells are often set in a fibrillar ground substance. Haphazardly arranged epithe-
ORAL SURGERY ORAL MEDICINE ORAL PATHOLOGY Volume 91, Number 3
Jones and Freedman 341
lioid cells with uniform nuclei might be present. Mitotic figures and atypia are rare, and laminated acellular psammoma bodies might be present. Immunohistochemical characteristics are useful in supporting the diagnosis. The cytoplasm of tumor cells is usually immunoreactive for vimentin, whereas the cytoplasmic membrane stains for EMA. S-100 protein might be demonstrated in the nucleus or cytoplasm of tumor cells, and occasional keratin immunoreactivity might be observed.1,3,6 Several benign and malignant spindle-cell neoplasms might demonstrate cytoplasmic membrane reactivity with EMA and must be included in the differential diagnosis of the 2 cases presented in this report. Lesions that might exhibit EMA reactivity include perineurioma, malignant nerve sheath tumor, synovial sarcoma, epithelioid sarcoma, leiomyosarcoma, malignant fibrous histiocytoma, and rhabdomyosarcoma. As neither case described in this report demonstrated cytologic atypia or mitotic activity, the malignant spindlecell neoplasms listed above are unlikely. Perineurioma is also unlikely because this tumor often resembles a fibrous histiocytoma or neurofibroma. It also does not demonstrate the whorls, rosettes, pseudoinclusions, and psammoma bodies described in our 2 cases. Although primary extracranial meningioma arising in the jaws is uncommon, surgical excision appears to be the treatment of choice. Two of the previously reported cases were treated through resectioning followed by reconstructing with a bone graft,1,3 whereas a third case was treated through an excisional biopsy.2 Both of the cases described in this report were treated with an exci-
sional biopsy. Given the lack of recurrence in the 3 previously published cases,1-3 we conclude that these neoplasms appear to be nonaggressive. However, close long-term follow-up appears to be prudent. We thank Dr Dennis Barbieri for his permission to use case 1 and Dr Thomas Cerbone for his permission to use case 2. REFERENCES 1. Landini G, Kitano M. Meningioma of the mandible. Cancer 1992;69:2917-20. 2. Simpson MT, Sneddon KJ. Extracranial meningioma of the oral cavity. Br J Oral Maxillofac Surg 1987;25:520-5. 3. Reddi SP, Strauss SI, Strauss JE, Blanchaert RH Jr. Anterior maxillary lesion. J Oral Maxillofac Surg 1999;57:1234-8. 4. Michel RG, Woodard BH. Extracranial meningioma. Ann Otol Rhinol Laryngol 1979;88(3 Pt 1):407-12. 5. Shuangshoti S, Panyathanya R. Ectopic meningiomas. Arch Otolaryngol 1973;98:102-5. 6. Kershisnik M, Callender DL, Batsakis JG. Pathology consultation. Extracranial, extraspinal meningiomas of the head and neck. Ann Otol Rhinol Laryngol 1993;102:967-70. 7. Batsakis JG. Pathology consultation. Extracranial meningiomas. Ann Otol Rhinol Laryngol 1984;93(3 Pt 1):282-3. 8. Gagnon NB, Lavigne F, Mohr G, Guerard MJ, Bouvier G. Extracranial and intracranial meningiomas. J Otolaryngol 1986; 15:380-4. 9. Perzin KH, Pushparaj N. Nonepithelial tumors of the nasal cavity, paranasal sinuses, and nasopharynx. A clinicopathologic study. XIII: Meningiomas. Cancer 1984;54:1860-9. Reprint requests: Anne Cale Jones, DDS The University of Texas Health Science Center at San Antonio Department of Pathology 7703 Floyd Curl Dr San Antonio, TX 78284-7750
[email protected]
To receive the tables of contents by e-mail, sign up through our Web site at: http://www.mosby.com/tripleo Choose e-mail notification. Simply type your e-mail address in the box and click the Subscribe button. Alternatively, you may send an e-mail message to
[email protected]. Leave the subject line blank and type the following as the body of your message: subscribetripleo_toc You will receive an e-mail to confirm that you have been added to the mailing list. Note that the table of contents e-mails will be sent out when a new issue is posted to the Web site.