Primary Bone Tumors of the Skull: Spectrum of 125 Cases, with Review of Literature

Abstract

Aims Primary skull bone tumors, benign or malignant, are rare, and include a vast repertoire of lesions. These tumors are not reported systematically in the literature, with most studies being on individual entities or as single case reports.

Methods Primary bone tumors diagnosed over a period of 12 years were retrieved, histological diagnoses reviewed, and clinical parameters noted.

Results We identified 125 primary skull bone tumors. The mean age at diagnosis was 32 years (range: 2–65 years). Majority of patients were adults (82.4%); male preponderance was noted (72.8%). Malignant tumors were more frequent than benign tumors. Most common malignant tumor was chordoma (n = 37), while most common benign tumor was osteoma (n = 7). Tumors were most frequently located at the skull base, of which clivus was most common location. Chordomas accounted for majority of clival tumors, while chondrosarcoma predominated at other skull base locations. Benign tumors were extremely rare in skull base. Tumors of the vault bones were infrequent; with chondrosarcoma and osteoma being the most common malignant and benign tumors, respectively.

Conclusions This is the largest series of primary skull bone tumors from India. Documentation of such a series will aid in approaching differential diagnosis of skull tumors in a systematic manner.

Keywords

bone tumors - skull - skull base - chondrosarcoma - chordoma

• References

• 1 Gundewar S, Kothari DS, Mokal NJ, Ghalme A. Osteomas of the craniofacial region: A case series and review of literature. Indian J Plast Surg 2013; 46 (3) 479-485
• 2 Boffano P, Roccia F, Campisi P, Gallesio C. Review of 43 osteomas of the craniomaxillofacial region. J Oral Maxillofac Surg 2012; 70 (5) 1093-1095
• 3 Haddad FS, Haddad GF, Zaatari G. Cranial osteomas: their classification and management. Report on a giant osteoma and review of the literature. Surg Neurol 1997; 48 (2) 143-147
• 4 Ellison DW, Perry A, Rosenblum M, Asa S, Reid R, Louis DN. Tumors: non-neuroepithelial tumours and secondary effects. In: Lowe S, Louis DN, Ellison DW, eds. Greenfield's Neuropathology. London, United Kingdom: Edward Arnold; 2008: 2002-2182
• 5 Dugert E, Lagleyre S, Brouchet A, Deguine O, Cognard C, Bonneville F. Osteoid osteoma invading the posterior labyrinth of the petrous bone. AJNR Am J Neuroradiol 2010; 31 (9) 1764-1766
• 6 Inwards CY, Oliveira AM. Tumors of the osteoarticular system. In: Fletcher CDM, ed. Diagnostic Histopathology of Tumors. Philadelphia, PA: Saunders Elsevier; 2013: 1871-1932
• 7 Lin YC, Commins DL, Fedenko AN, Pinsky GS. A rare case of periosteal osteoblastoma located in the frontal cranial bone. Arch Pathol Lab Med 2005; 129 (6) 787-789
• 8 Moon KS, Jung S, Lee JH , et al. Benign osteoblastoma of the occipital bone: case report and literature review. Neuropathology 2006; 26 (2) 141-146
• 9 Hadley C, Gressot LV, Patel AJ , et al. Osteosarcoma of the cranial vault and skull base in pediatric patients. J Neurosurg Pediatr 2014; 13 (4) 380-387
• 10 Nora FE, Unni KK, Pritchard DJ, Dahlin DC. Osteosarcoma of extragnathic craniofacial bones. Mayo Clin Proc 1983; 58 (4) 268-272
• 11 Clark JL, Unni KK, Dahlin DC, Devine KD. Osteosarcoma of the jaw. Cancer 1983; 51 (12) 2311-2316
• 12 Kassir RR, Rassekh CH, Kinsella JB, Segas J, Carrau RL, Hokanson JA. Osteosarcoma of the head and neck: meta-analysis of nonrandomized studies. Laryngoscope 1997; 107 (1) 56-61
• 13 Haque F, Fazal ST, Ahmad SA, Abbas SZ, Naseem S. Primary osteosarcoma of the skull. Australas Radiol 2006; 50 (1) 63-65
• 14 Ashkan K, Pollock J, D'Arrigo C, Kitchen ND. Intracranial osteosarcomas: report of four cases and review of the literature. J Neurooncol 1998; 40 (1) 87-96
• 15 Salvati M, Ciappetta P, Raco A. Osteosarcomas of the skull. Clinical remarks on 19 cases. Cancer 1993; 71 (7) 2210-2216
• 16 Cianfriglia F, Pompili A, Occhipinti E. Intracranial malignant cartilaginous tumours. Report of two cases and review of literature. Acta Neurochir (Wien) 1978; 45 (1–2) 163-175
• 17 Bloch OG, Jian BJ, Yang I , et al. A systematic review of intracranial chondrosarcoma and survival. J Clin Neurosci 2009; 16 (12) 1547-1551
• 18 Hogendoorn PCW, Bovee JVMG, Nielsen GP. Chondrosarcoma (grades I–III), including primary and secondary variants and periosteal chondrosarcoma. In: Fletcher CDM, Bridge JA, Hogendoorn PCW, Mertens F, eds. WHO Classification of Tumors of Soft Tissue and Bone. Lyon, France: IARC; 2013: 264-268
• 19 Hakan T, Vardar Aker F. Chondromyxoid fibroma of frontal bone: a case report and review of the literature. Turk Neurosurg 2008; 18 (3) 249-253
• 20 Xu H, Qin Z, Shi Z. Chondromyxoid fibroma in the sella turcica region. J Clin Neurosci 2011; 18 (10) 1419-1421
• 21 Miyamoto E, Kuriyama T, Iwamoto M, Tsuji N, Shizuki H. Cranial chondromyxoid fibroma. Case report. J Neurosurg 1981; 55 (6) 1001-1003
• 22 Khalatbari MR, Hamidi M, Moharamzad Y. Chondromyxoid fibroma of the anterior skull base invading the orbit in a pediatric patient: case report and review of the literature. Neuropediatrics 2012; 43 (3) 140-145
• 23 Stacchiotti S, Sommer J ; Chordoma Global Consensus Group. Building a global consensus approach to chordoma: a position paper from the medical and patient community. Lancet Oncol 2015; 16 (2) e71-e83
• 24 Jian BJ, Bloch OG, Yang I, Han SJ, Aranda D, Parsa AT. A comprehensive analysis of intracranial chordoma and survival: a systematic review. Br J Neurosurg 2011; 25 (4) 446-453
• 25 Cho HY, Lee M, Takei H, Dancer J, Ro JY, Zhai QJ. Immunohistochemical comparison of chordoma with chondrosarcoma, myxopapillary ependymoma, and chordoid meningioma. Appl Immunohistochem Mol Morphol 2009; 17 (2) 131-138
• 26 Arai M, Nobusawa S, Ikota H, Takemura S, Nakazato Y. Frequent IDH1/2 mutations in intracranial chondrosarcoma: a possible diagnostic clue for its differentiation from chordoma. Brain Tumor Pathol 2012; 29 (4) 201-206
• 27 Yadav R, Sharma MC, Malgulwar PB , et al. Prognostic value of MIB-1, p53, epidermal growth factor receptor, and INI1 in childhood chordomas. Neuro-oncol 2014; 16 (3) 372-381
• 28 Van Gompel JJ, Janus JR. Chordoma and chondrosarcoma. Otolaryngol Clin North Am 2015; 48 (3) 501-514
• 29 Wittekind C, Compton C, Quirke P , et al. A uniform residual tumor (R) classification: integration of the R classification and the circumferential margin status. Cancer 2009; 115 (15) 3483-3488
• 30 Stacchiotti S, Longhi A, Ferraresi V , et al. Phase II study of imatinib in advanced chordoma. J Clin Oncol 2012; 30 (9) 914-920
• 31 Mobley BC, Roulston D, Shah GV, Bijwaard KE, McKeever PE. Peripheral primitive neuroectodermal tumor/Ewing's sarcoma of the craniospinal vault: case reports and review. Hum Pathol 2006; 37 (7) 845-853
• 32 Nambirajan A, Suri V, Sharma MC , et al. A 7-year-old girl with recurrent episodes of abdominal pain, seizures, and loss of vision: Primary diffuse leptomeningeal primitive neuroectodermal tumor masquerading as chronic meningitis. Neurol India 2015; 63 (5) 736-742
• 33 Furuno Y, Nishimura S, Kamiyama H , et al. Intracranial peripheral-type primitive neuroectodermal tumor. Neurol Med Chir (Tokyo) 2008; 48 (2) 72-76