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1 中華癌醫會誌 (J. Chinese Oncol. Soc.) 25(5), , 2009 Case Report journal homepage: Primary Intraosseous Meningioma Chung-Yu Wen, Yih-Yiing Wu 2, Jing-Shan Huang, Chih-Ju Chang * Department of Neurosurgery, Cathay General Hospital, Taipei, Taiwan 2 Department of Pathology, Cathay General Hospital, Taipei, Taiwan 病例報告 原發性骨內腦膜瘤 Abstract. Primary intraosseous meningioma is a subtype of meningioma that represents one of the uncommon manifestations of meningioma. These tumors of the skull are often confused preoperatively with a primary bone tumor or metastasis. We report a rare case in a 73-year-old female patient who was initially diagnosed as skull bone metastasis due to lymphoma with multiple lymph node enlargement. However the final histological examination revealed an intraosseous meningioma. We also discuss the findings on MRI, CT, histological features, and surgical treatments for this rare case of primary intraosseous meningioma. Keywords : meningioma 溫中瑜 劉昌熾 吳毅穎 2 黃金山 張志儒 * 國泰綜合醫院神經外科 2 國泰綜合醫院病理科 中文摘要原發性骨內腦膜瘤是一種非常少見的腦膜瘤亞型 這類腫瘤在術前的診斷常常與 原發性骨癌或轉移性病灶難以鑑別 我們提出一個病例是一名發生 73 歲女性的病人, 其身上有明顯突出的頭骨病灶及多處的淋巴結腫大, 起初懷疑是由惡性腫瘤的遠端轉移導致, 然而術後的病理報告卻顯示為原發性骨內腦膜瘤, 而淋巴結腫大的部分則為惡性淋巴瘤的表現 我們在此提出她的臨床症狀 放射線診斷 手術及病理組織的發現, 並做文獻回顧及探討 關鍵字 : 原發性骨內腦膜瘤 INTRODUCTION Meningioma is the most common benign intracranial neoplasm, comprising about 5~20% of brain tumors. It usually originates from clusters of arachnoid cap cells located in the external layer of the arachnoid membrane []. Most meningiomas are thought to be intradural lesions and located in the subdural space. However, there are extradural meningiomas, which constitute ~2% of all meningiomas and arise in locations other than the dura mater, such

2 380 C. Y. Wen et al./jcos 25(2009) as skin, paranasal sinuses, orbit, neck, salivary glands, and calvalria [2]. Primary intraosseous meningioma, which is often mistaken for primary bone tumor, represents about two thirds of extradural meningiomas [2]. These tumors mostly affect adults in the fourth decade, especially females. Most intraosseous meningiomas are either osteoblastic or mixed osteoblastic-osteolytic [3]. Here, we describe a case occurring in a 73-year-old female with leukemia. CASE REPORT A 78-year-old woman presented with headache, intermittent fever, general weakness for a month prior to admission. According to her statement, a left frontal protruding mass had been found 3 to 4 years before. In addition, multiple palpable masses over the neck, bilateral axillae and femoral regions appeared continuously following the left frontal mass. She had no other systemic disease or surgical history. Clinical examination revealed an 8 x 7 x 2 cm 3 dome-shaped mass situated over the left anterior aspect of the frontal bone (Figure ). On palpation, the lesion was non-tender, hard in consistency, and exhibited a smooth transition from normal skin convexity to the lesion. The overlying skin demonstrated no specific alterations in its consistency, color, texture, or thickness, and the patient denied any changes in sensation except general headache in the preceding month. All neurologic examinations were unremarkable. After consulting specialists from the infectious disease department about the multiple lymph node enlargements, metastasis or TB lymphadenopathy were suspected and biopsy was suggested. *Corresponding author: Chih-Ju Chang M.D. * 通訊作者 : 張志儒醫師 Tel: Fax: khaedohk@hotmail.com CT scan showed a bony mass with thickening over the left frontal region with multi-focal osteolytic lesions and rarefaction of the bone structures (Figure 2). Differential diagnosis included meningioma, duralbone metastatic lesion, osteomyelitis, and bone tumor. During the operation, the mass was approached after the scalp flap was elevated over bilateral coronal incisions. There was no attachment or invasion between dura and the bone lesion. Gross total removal was accomplished via craniectomy. The resulting bone defect was reconstructed by cranioplasty with titanium mesh. Together inguinal lymph node biopsy was done. All the lesions were sent for histopathologic examination. The surgical specimen of left frontal skull bone tumor (Figure 3) consisted of a piece of skull bone measuring 8.2 x 7.9 x 2.4 cm 3 in size. Grossly, it was gray and hard. At the center of the inner surface, there was a cluster of osteolytic defects. On cross section, a space-occupying, intraosseous, multiloculated and osteolytic tumorous lesion was seen. The tumor border was irregular and focally infiltrative, with tumor extension towards the section margin. Microscopically, it showed an intraosseous meningioma with high vascular density. Typical meningothelial and syncytial to transitional patterns were noted around the vessels, with scattered psammoma bodies. The tumor cells were benign and uniform, with occasional nuclear pseudoinclusions (Figure 4). Intravascular growth was also occasionally seen. However, the section of the inguinal lymph node showed a high-grade large cell malignant lymphoma, diffuse large B cell, immunoblastic type with plasmacytoid differentiation. An oncologist was consulted, and chemotherapy was suggested, but the patient refused it at the time. She had an uneventful recovery and was discharged without any post-operative complications or any signs or symptoms of recurrence of intraosseous meningioma. However, due to the lymphoma without any treatment, the patient expired after 2 months.

3 C. Y. Wen et al./jcos 25(2009) A B Figure. (A) From the vertex view, (B) Lateral view 8 x 7 x 2 cm 3 dome-shaped mass situated over the left anterior aspect of the frontal bone A B Figure 2. (A) Non-contrast enhanced CT, (B) Bone window CT scan study showed a bony mass with thickening over the left frontal region with multi-focal osteolytic lesions DISCUSSION Meningiomas represent about 5~20% of brain tumors, with an estimated incidence of 2. per 00,000 cases [4]. These tumors mostly affect adults, with a strong fourth-decade female with a 2: female-to male ratio []. Extradural meningiomas are very rare, accounting for less than 2% of all meningiomas [5]. Primary intraosseous meningioma is a subset of extradural meningiomas that arise in bone. The average reported age of patients presenting primary intraosseous meningioma was 50.5 years, with a slight female predominance of.65: [6,7]. The commonly reported craniofacial sites for primary intraosseous meningiomas are calvarial, sphenoid, and

4 382 C. Y. Wen et al./jcos 25(2009) A Figure 3. Grossly, it was gray and hard. At the center of the inner surface, there was a cluster of osteolytic defects without any dura attachment and invasion orbital bones and to a lesser extent the maxilla and mandible. Convexity area and the skull base are the two major locations for intraosseous meningioma [4]. B The etiology of intraosseous meningiomas has Figure 4. Histological features (A) Intraosseous not been clarified, but some hypotheses have been meningioma with high vascular density proposed. These lesions are usually observed near the (H&E, x40) (B) Typical meningothelial suture lines. Azar-Kia et al. suggested that arachnoid cells with a syncytial growth pattern was cap cells are trapped in the cranial sutures during birth noted around the vessels. The tumor and molding of the head [2]. Misplacement and en- cells were benign and uniform, with oc- trapment of meningothelial cells into suture or frac- casional nuclear pseudoinclusions (H&E, ture lines as a result of trauma has also been specu- x00) lated as the probable cause of calvarial meningioma [8]. In addition, arachnoidal cell presence in the sheaths of nerves and vessels exiting various skull fo- with extracalvarial extension [9]. The latter two are raminae, cerebral hypertension that displaces arach- further divided as convexity (C) or skull base (B) noid islets and a separate origin from an undifferenti- forms. The case presented here was a type IIC men- ated or multipotential mesenchymal cell are men- ingioma. According to the literature, 68% of the pri- tioned in recent reports [7]. mary extradural meningiomas involved the calvaria. Primary extradural meningiomas are classified Frontoparietal and orbital regions are the most com- by Lang and colleagues as type I: purely extracal- mon locations for intraosseous meningiomas, and to a varial, type II: purely calvarial, or type III: calvarial lesser extent the maxilla and mandible [9]. The tu-

5 C. Y. Wen et al./jcos 25(2009) mors are nearly uniformly solitary, although a small number of case reports have presented patients with two separate lesions [0]. Like many intracranial lesions, the clinical symptoms are dependent on tumor location, size, and involvement of the neighboring structures. The initial presentation is usually a firm, painless expansible mass with normal overlying skin, and may be detected incidentally []. There are not any neurologic symptoms or signs, unless the lesion extends through the inner table and compresses intracranial structures. Seizure, dizziness, headache, tinnitus, vague sensation, and cranial nerve deficits such as ophthalmoplegia, visual field defects or hearing loss have also been reported in the literature [2]. Histologically, the cells of intraosseous meningioma exhibit wide variety of histopathologic variants, which may range from epithelial to mesenchymal in appearance like their intracranial counterparts [4,7]. Microscopic pathology often reveals findings including psammoma bodies and eosinophilic tumor cells with indistinct borders grouped in clusters and whorls [0,3]. The nuclei are typically oval, and regular, and nuclear pseudoinclusions may be observed. In addition to meningotheliomatous meningioma, other histological types including microcystic, psammomatous, transitional, chordoid, atypical, malignant, and fibroblastic intraosseous meningiomas have been reported. Besides, calvarial meningiomas are more prone to develop malignant changes (%) compared with intracranial meningiomas (2%) []. In a review of the literature, hyperosteosis as bone remodeling and calvarial thickening at the site of origin of the meningioma and osteolytic lesion are frequent with these tumors. Hyperosteosis was the most common radiographic finding (59%) and osteolysis was reported in 35% and a mixed picture of both was reported in 6% of the cases []. Furthermore, it has been reported that meningiomas presenting with scalp swelling, osteolytic skull lesions, and extracranial soft-tissue masses are more aggressive or malignant in nature compared to others [4,5]. Although superimposed bone structures may limit the usefulness of conventional radiographs, skull radiographs can still detect abnormalities in 30 to 60% of cases of osteoblastic intraosseous meningioma, including hyperosteosis, irregular foci of calcification, and atypical vascular markings [6]. The tumor is usually hyperdense on a nonenhanced CT scan with a focally thickened lesion expanding the calvaria and enhances densely after contrast administration [6,7]. The osteolytic lesion may appear hypodense on plain radiographs and typically cause thinning, expansion, and interruption of the inner and outer cortical layers of the skull on CT scan [3]. MR imaging provides a better anatomic delineation in the evaluation of the soft tissue component and extradural extension of the lesion []. Meningiomas are typically hypointense compared with brain on T-weighted images, and hyperintense on T2-weighted images, while variable signal intensity has been reported. Prominent homogeneous enhancement after Gd administration is typical [8]. The differential diagnosis of intraosseous meningioma include osteoma, osteosarcoma, Paget s disease, meningioma en plaque and fibrous dysplasia. Furthermore, the osteolytic subtype must be differentiated from hemangioma, chondroma, chondrosarcoma, dermoid, epidermoid tumor, brown tumor, multiple myeloma, plasmacytoma, giant cell tumor, aneurismal bone cyst, eosinophilic granuloma, or metas- tatic cancer [9,20]. Although rare, intraosseous malignant meningioma should be excluded in an osteolytic skull lesion with an associated soft-tissue component []. In symptomatic primary intraosseous meningioma, total tumor removal with a wide surgical resection followed by cranial reconstruction is the preferred choice of treatment [,2]. If only subtotal resection is possible because of involvement of critical structures within the orbit, paranasal sinuses, or skull base, the residual tumor should be followed radiologically. Other adjuvant treatment modalities may

6 384 C. Y. Wen et al./jcos 25(2009) be used, for example, chemotherapy and bisphosphonate therapy. The recurrence rates for all locations are estimated to be between 0% and 23%. However, the overall recurrence rate and prognosis for intraosseous extracranial tumors is unknown due to both the small number of cases and degree of follow-up [22]. The majority of recurrences are believed to be due to residual tumor left in the operative bed due to fear of causing severe functional deficits when complete resection is attempted [23,24]. According to the literature, rate of recurrence is higher when calvarial and basal bone structures are involved [24]. CONCLUSIONS In conclusion, intraosseous meningioma is the most common type of extradural meningioma. This rare lesion can cause proptosis and other neurological symptoms depending on their location and mass effect. CT and particularly MRI are helpful in making the diagnosis and evaluating the soft tissue components and extent of extradural extension. Most of these tumors are benign and cause hyperostosis. However, the osteolytic subtype of intraosseous meningiomas are more likely to be malignant than the osteoblastic subtype. The treatment is resection followed by cranioplasty and it is potentially curative or may require adjuvant therapy, if complete resection is not possible. Intraosseous meningioma should be considered in the differential diagnosis when a patient presents with osteoblastic or osteolytic skull lesions. REFERENCES. El Mahou S, Popa L, Constantin A, et al. Multiple intraosseous meningiomas. Clin Rheumatol 25: , Marwah N, Gupta S, Marwah S, et al. Primary intraosseous meningioma. Indian J Pathol Microbiol 5: 5-52, Agrawal V, Ludwig N, Agrawal A, et al. Intraosseous intracranial meningioma. AJNR Am J Neuroradiol 28: 34-35, Yener U, Bayrakli F, Vardereli E, et al. Intradiploic meningioma mimicking calvarial metastasis: case report. Turk Neurosurg 9: , Lell M, Tudor C, Aigner T, et al. CT and MR imaging features. AJNR Am J Neuroradiol 28: 29-3, Politi M, Romeike BF, Papanagiotou P, et al. Intraosseous hemangioma of the skull with dural tail sign: radiologic features with pathologic correlation. AJNR Am J Neuroradiol 26: , McGuire TP, Palme CE, Perez-Ordonez B, et al. Primary intraosseous meningioma of the calvaria: analysis of the literature and case report. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 04: e34-4, Weber J, Spring A, Czarnetzki A. [Parasagittal meningioma in a skull dated years before present from southwestern Germany]. Dtsch Med Wochenschr 27: , Lang FF, Macdonald OK, Fuller GN, et al. Primary extradural meningiomas: a report on nine cases and review of the literature from the era of computerized tomography scanning. J Neurosurg 93: , Elder JB, Atkinson R, Zee CS, et al. Primary intraosseous meningioma. Neurosurg Focus 23: E3, Tokgoz N, Oner YA, Kaymaz M, et al. Primary intraosseous meningioma: CT and MRI appearance. AJNR Am J Neuroradiol 26: , Yamazaki T, Tsukada A, Uemura K, et al. Intraosseous meningioma of the posterior fossa-- Case report. Neurol Med Chir (Tokyo) 4: 49-53, Cirak B, Guven MB, Ugras S, et al. Frontoorbitonasal intradiploic meningioma in a child. Pediatr Neurosurg 32: 48-5, Muthukumar N. Primary calvarial meningiomas. Br J Neurosurg : , 997.

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