Jonathan Irish, MD, FRCSC, Section Editor

Primary extradural meningioma presenting as a neck mass: Case report and review of the literature Rami Saade, MD,1 Amy Hessel, MD,1 Lawrence Ginsberg, MD,2 Greg Fuller, MD, PhD,3 Diana Bell, MD3* 1

Department of Head and Neck Surgery, The University of Texas MD Anderson Cancer Center, Houston, Texas, 2Department of Radiology, The University of Texas MD Anderson Cancer Center, Houston, Texas, 3Department of Pathology, The University of Texas MD Anderson Cancer Center, Houston, Texas.

Accepted 17 September 2014 Published online 16 June 2015 in Wiley Online Library ( DOI 10.1002/hed.23874

ABSTRACT: Background. Primary extradural meningiomas (PEMs) are rare, particularly those involving the paraspinal cervical area, so little is known about them. We identified a new case and compared it with 10 previous cases, addressed the diagnostic challenges, and highlighted the clinical and pathologic characteristics. Methods. We conducted a case report and literature review of PEM cases reported since 1976. Results. A 59-year-old man presented with right neck stiffness and discomfort that was present over the last several months, which is consistent with other cases. CT scan and MRI revealed a paraspinal cervical

INTRODUCTION Primary extradural meningiomas (PEMs) are rare tumors that occur in various sites of the body. Only 10 cases of PEMS in the neck have been published in the literature since 1976. We report a case of cervical paraspinal PEM in a patient recently evaluated and treated at our institution. We address the diagnostic challenges of these benign tumors and highlight their clinical and pathologic characteristics.

CASE REPORT Our case involved a 59-year-old man who presented with a complaint of right neck stiffness and discomfort that persisted for several months. He was otherwise asymptomatic. Two years before presentation, he had been diagnosed with prostate adenocarcinoma for which he underwent radical prostatectomy. The tumor recurred locally shortly thereafter and the patient received salvage radiotherapy. Interval history and surveillance findings of this primary tumor were unremarkable. The patient also reported a remote history of excised skin lesions, denied any trauma to the neck, and gave a noncontributory family history.

*Corresponding author: D. Bell, Department of Pathology, The University of Texas MD Anderson Cancer Center, 1515 Holcombe Blvd, Houston, TX 77030. E-mail: [email protected] Ehab Y. Hanna, MD, Editor, was recused from consideration of this manuscript.




mass. Fine-needle aspiration (FNA) and core biopsy revealed spindle cells with whorling consistent with meningioma. The patient underwent complete en bloc resection of the tumor. The PEM was histologically benign. Conclusion. Cervical PEMs should be considered in the differential diagnosis of a deep neck mass. These tumors are typically benign C 2014 Wiley Periodicals, Inc. Head Neck and are surgically resected. V 37: E92–E95, 2015

KEY WORDS: meningioma, extradural, primary, cervical, mass

On physical examination, no palpable neck mass was noted. Cranial nerve function was intact. The rest of the examination and the results from flexible endoscopy were unremarkable. Initial workup included a contrast-enhanced CT scan of the neck that revealed a 4 3 3.5 3 2.7-cm right paraspinal soft tissue mass of heterogeneous density and enhancement in areas of necrosis. The mass extended from the C1 to C3 levels, abutting the C2 transverse process and the vertebral artery medially. T1, T2, and gadolinium-weighted MRI results confirmed the absence of osseous erosion/destruction or extension into the canal/foramina (see Figure 1). The lesion exhibited a hypointense signal on T1, an isointense-to-hyperintense signal on T2, and strong heterogeneous enhancement with gadolinium. Clinical findings suggested a metastatic process. Other differential diagnoses included soft tissue sarcoma, neurofibroma, schwannoma, peripheral nerve sheath tumor, and desmoid tumor. CT-guided fine-needle aspiration (FNA) and core biopsy results revealed a spindle cell neoplasm with an immunohistochemical profile positive for epithelial membrane antigen (EMA) and negative for S100, melanoma cocktail, and glial fibrillary acid protein. This profile was consistent with meningioma. A multidisciplinary team (head and neck surgery, radiology, pathology, neurosurgery, and radiation oncology) was involved in the diagnosis, management, and care of the patient. A complete en bloc resection of the cervical paraspinal mass was performed with careful dissection and preservation of all surrounding critical neurovascular



FIGURE 1. Axial T1 precontrast (A) and postcontrast (B) images demonstrate a mass that is isointense (A, arrow) and enhances intensely (B, arrow) adjacent to the right side of C2. The lesion does not seem to involve the spine directly and with no apparent connection to the dura.

structures. Intraoperatively, the mass was distinct and had no connections to the dura. The specimen consisted of a well-encapsulated 6 3 3.5 3 3.5-cm bosselated multiloculated ovoid mass resected with a cuff of muscle (see Figure 2). The postoperative course was smooth and uneventful; the patient had minimal right shoulder weakness that was successfully addressed with physical therapy. The final pathology report described a world Health Organization (WHO) grade I meningioma with clear margins and no perineural or lymphovascular invasion. In the absence of adverse features, the patient did not require postoperative adjuvant radiotherapy. He will be followed up with regular clinical examinations and serial imaging.

DISCUSSION PEMs represent only 1.6% of all meningiomas.1,2 These tumors, which have also been called ectopic,3,4 extracranial,5 extraneuraxial,6 and extradural,7 constitute the rare group of primary meningiomas that arise outside the cen-

tral nervous system with no dural connections. The exact origin of PEMs is unknown. They may derive from undifferentiated mesenchymal arachnoidal cells trapped during embryogenesis8,9 or from cellular dedifferentiation.10 Several PEM sites have been described11: calvarium, scalp, ear/temporal bone, skull base, sinuses, pharynx, orbit, face, neck, lung/mediastinum, adrenal glands, abdomen, paraspinal, and extremities. The paraspinal cervical area accounts for up to 6% of all PEMs. Only 10 of these cases have been reported in the literature from 1976 to the present---the CT era2,11 (Table 1). The case we have described is, to our knowledge, the 11th reported case of a PEM presenting as a neck mass (excluding tonsil, parapharyngeal, pterygoid, parotid, mandible, and shoulder cases). PEMs have been diagnosed in slightly more women than men and seem to have a bimodal age distribution (peaking in the second and fifth decades of life).2,11 They typically manifest as painless, firm, and slowgrowing masses. Depending on their size and exact

FIGURE 2. Gross appearance of a well-encapsulated 6-cm bosselated, multiloculated ovoid mass resected with a cuff of muscle.





TABLE 1. Published cases of cervical/paraspinal primary extradural meningiomas. Author/year


Age, y


Symptoms at presentation



Sartor et al 1977 Gonzales–Campora et al 1988 Gonzales–Campora et al 1988 Galm et al 2006 Takeuchi et al 2006 Yamada et al 2007 Frank et al 2008 Sanei et al 2008 Santiago et al 2009 Qutub et al 2010


29 29 51 51 50 22 45 16 42 37

C1-C4 paraspinal level Carotid sheath area Carotid sheath area Posterior triangle C1-C4 paraspinal level C1-C5 paraspinal level C5-C7 paraspinal level Neck C2-C3 paraspinal level Posterolateral neck

Neck pain Mass Mass Enlarging neck mass Hand numbness Hand weakness, gait disturbance Pain Mass Paraparesis Mass

Complete tumor resection Complete tumor resection Complete tumor resection None Complete tumor resection Incomplete tumor resection Incomplete tumor resection Complete tumor resection Complete tumor resection N/A

Benign Benign Benign Benign Benign Benign Benign Benign Benign Benign

Abbreviation: N/A, not available.

location, a variety of symptoms and neurologic deficits can ensue. Optimal radiologic evaluation includes both contrastenhanced CT and MRI with the following characteristic signal intensities: hypointense on T1, hyperintense on T2, and homogenous enhancement with gadolinium contrast. The differential diagnosis of these tumors is dictated mainly by their location. Soft tissue sarcomas and neurogenic tumors may be considered at first. FNA or tissue biopsy of these tumors reveal pathognomonic spindled or polygonal cells, which, in some areas, form fascicles or tend to stream in small whorls, or syncytial appearance (see Figure 3). Psammoma bodies are present, but mitoses and necrosis are uncommon. Ancillary studies reveal immunoreactivity for vimentin, EMA, and progesterone receptor but no reaction to S100. More than 90% of PEMs are benign (WHO type I). Like all 10 PEM cases previously reported in the literature, our patient’s tumor was histologically benign (WHO I). In 2000, Lang et al2 assessed PEMs and, on the basis of the vast propensity of these cases occurring in the calvarium and skull base, proposed a more practical classification system (Table 2) that could also predict the rate of recurrence. In this classification system, cervical paraspinal PEMs are type I tumors and are expected to have a low recurrence rate adopted in most case reports that have followed. Management of PEMS often requires a multidisciplinary team approach. Gross tumor resection with or without postoperative radiotherapy offers the best chance of cure. Residual disease, positive margins, recurrence, and atypical and malignant histologic features are all consensus indications for adjuvant radiotherapy. Chemotherapy

TABLE 2. Lang et al2 2000, primary extradural meningiomas classifications. FIGURE 3. Classical microscopic findings of meningioma. Low power (A) and higher power (B) hematoxylin-eosin stain showing pathognomonic spindled or polygonal cells, which in some areas form lobules or fascicles or tend to stream in small whorls (B inset: close-up on syncytial pattern). Meningiomas show immunoreactivity with anti-epithelial membrane antigen (EMA) antibodies (C).




Type I Type II Type III

Purely extracalvarial Purely calvarial (C) convexity form (B) skull base form Calvarial with extracalvarial extension (C) convexity form (B) skull base form


has a modest role in the palliative setting12,13 as do several emerging molecular targeted therapies.14–18

CONCLUSION Cervical paraspinal PEMs are so rare that they may pose a diagnostic challenge for clinicians and pathologists. These tumors should be considered in the differential diagnosis of a deep neck mass. Recognizing this entity requires a critical radiographic review and judicious histologic examination. PEMs in the neck have historically been benign and complete surgical resection typically achieves cure.

REFERENCES 1. Friedman CD, Costantino PD, Teitelbaum B, Berktold RE, Sisson GA Sr. Primary extracranial meningiomas of the head and neck. Laryngoscope 1990;100:41–48. 2. Lang FF, Macdonald OK, Fuller GN, DeMonte F. Primary extradural meningiomas: a report on nine cases and review of the literature from the era of computerized tomography scanning. J Neurosurg 2000;93:940–950. 3. Bitoh S, Obashi J, Kobayashi Y. Primary ectopic extracalvarial meningioma. Surg Neurol 1986;25:591–594. 4. Sadasivan B. Ectopic meningiomas. In: Schmidek HH, editor. Meningiomas and their surgical management. Philadelphia, PA: WB Saunders; 1991. pp 493–495. 5. Farr HW, Gray GF Jr, Vrana M, Panio M. Extracranial meningioma. J Surg Oncol 1973;5:411–420.


6. Shuangshoti S. Primary meningiomas outside the central nervous system. In: Al-Mefty O, editor. Meningiomas. New York, NY: Raven Press; 1991. pp 107–128. 7. Rosencrantz M, Stattin S. Extradural meningiomas. Report of two cases. Acta Radiol Diagn (Stockh) 1972;12:419–427. 8. Lopez DA, Silvers DN, Helwig EB. Cutaneous meningiomas – a clinicopathologic study. Cancer 1974;34:728–744. 9. Inagaki K, Otsuka F, Matsui T, Ogura T, Makino H. Effect of etidronate on intraosseous meningioma. Endocr J 2004;51:389–390. 10. Cirak B, Guven MB, Ugras S, Kutluhan A, Unal O. Fronto-orbitonasal intradiploic meningioma in a child. Pediatr Neurosurg 2000;32:48–51. 11. Mattox A, Hughes B, Oleson J, Reardon D, McLendon R, Adamson C. Treatment recommendations for primary extradural meningiomas. Cancer 2011;117:24–38. 12. Newton HB. Hydroxyurea chemotherapy in the treatment of meningiomas. Neurosurg Focus 2007;23:E11. 13. Chamberlain MC, Tsao–Wei DD, Groshen S. Salvage chemotherapy with CPT-11 for recurrent meningioma. J Neurooncol 2006;78:271–276. 14. Wen PY, Yung WK, Lamborn KR, et al. Phase I/II study of imatinib mesylate for recurrent malignant gliomas: North American Brain Tumor Consortium Study 99-08. Clin Cancer Res 2006;12:4899–4907. 15. Norden AD, Raizer JJ, Abrey LE, et al. Phase II trials of erlotinib or gefitinib in patients with recurrent meningioma. J Neurooncol 2010;96:211–217. 16. Raizer JJ, Grimm SA, Rademaker A, et al. Phase II trial of PTK787/ZK 222584 in recurrent or progressive radiation and surgery refractory meningiomas. J Neurooncol 2014;117:93–101. 17. Kaley T, et al. Phase II trial of sunitinib (SU011248) in patients with recurrent or inoperable meningioma. Neuro Oncology 2008. 18. Chamberlain MC, Glantz MJ, Fadul CE. Recurrent meningioma: salvage therapy with long-acting somatostatin analogue. Neurology 2007;69:969– 973.




Primary extradural meningioma presenting as a neck mass: Case report and review of the literature.

Primary extradural meningiomas (PEMs) are rare, particularly those involving the paraspinal cervical area, so little is known about them. We identifie...
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