Open Journal of Modern Neurosurgery, 2014, 4, 105-109
Published Online July 2014 in SciRes.
How to cite this paper: Sofiene, B., et al. (2014) A Case Report of Osteosarcoma of the Skull. Open Journal of Modern Neu-
rosurgery, 4, 105-109.
A Case Report of Osteosarcoma of the Skull
Bouali Sofiene*, Bouhoula Asma, Boubaker Adnene, Ben Said Imed, Kallel Jalel,
Aouij Lassaad, Jemel Hafedh
Department of Neurosurgery, Faculty of Medicine, University of Tunis El Manar, National Institute of
Neurology Mongi Ben Hmida, Tunis, Tunisia
Email: *sofienebouali@h
Received 6 April 2014; revised 6 May 2014; accepted 14 May 2014
Copyright © 2014 by authors and Scientific Research Publishing Inc.
This work is licensed under the Creative Commons Attribution International License (CC BY).
http://creativecommo by/4.0/
Background: Osteosarcoma is the most common primary malignancy and aggressive neoplasm of
bone composed of spindle cells producing osteoid. The incidence of primary osteogenic sarcomas
of the skull is about 1% to 2% of all skull tumors. Methods: We present an extraordinary case of
osteosarcoma arising in the postero-temporal region of the skull. The clinical features, diagnosis
and management are discussed. Results: A 34-year-old woman was operated in our department
for mass, fixed to bone in the left postero-temporal area. Histopathology confirmed the diagnosis
of osteogenic sarcoma. Conclusion: We review the literature of reported cases of primary osteo-
genic sarcomas of the skull to discuss the common clinical presentation, evaluation methods, and
recommended treatment plans.
Osteosarcoma, Primary Tumor, Skull
1. Introduction
Oste os arco ma (OS) of the skull is an extremely rare tumour particularly as a primary tumor. Osteosarcomas are
typically long bone tumors and rarely affect the skull particularly after radiation or chemotherapy for other ne-
oplasms. The incidence of primary osteogenic sarcomas of the skull is about 1% to 2% of all skull tumors [1] [2].
Reports are few and most combine skull lesions with the much more frequent Osteosarcoma of the maxillo-man-
dibular complex [3].
We report a rare case of Osteosarcoma arising in parieto-temporal bone.
2. Case Report
A 34-year-old woman presented with a 4-year history of a postero-temporal bump which had rapidly enlarged in
*Corresponding a uthor.
B. Sofiene et al.
5 months prior to admission. Physical examination demonstrated a 5 × 7 cm rubbery hard, non tender mass, fix-
ed to bone in the left postero-temporal area. The remainder of physical and neurological evaluations was normal,
as were all routine laboratory studies.
Both brain computerized tomography (CT) CT-scan and Magnetic resonance imaging (MRI) show ed an e xtra
and intra axial lesion which had three components: fleshy, cystic and ossified (Figure 1 and Figure 2).
The patient underwent surgery with a complete resection of the lesion associated with dural plasty and cranial
reconstruction (Fi gure 3 a nd Figure 4). Postoperative CT scan showed gross total removal (Figure 4).
Histopathology exam confirm the diagnosis of osteoginec sarcoma (Figure 5). Postoperative radiation t he rapy
is ongoing.
Figure 1. Axial CT scan showing a left postero-temporal lesion
made 4 years ag o .
Figure 2. axial CT scan (C and D) without contrast, showed irregular calcification and low attenuation
areas (bone window setting). MRI showed a large mass arising from the postero-temporal aspect of the
skull on the left side. There was a calcified hemorrhagic part of the mass with marked vascularity of
the tumor (E, F and G).
B. Sofiene et al.
Figure 3. Pre-operative (a) and per-operative (b, c and d) pic-
tures showing the cutaneous aspect and the different time of
Figure 4. Pos t o p er ative axial ct scan (H and I).
Figure 5. Sections were subjected to Hemotoxylin & Eosin
staining, showing the presence of pleomorphic spindeloid to po-
lygonal cells with numerous atypical mitoses. Separating the neo-
plastic cells are seams of a bright pink matrix which is typical of
osteoid and typical of an osteosarcoma.
B. Sofiene et al.
3. Discussion
Osteosarcoma is the most common primary malignancy of bone and 5% to 6% arise in the craniofacial bones [1]
[4]. The etiology and precise pathogenesis of this disease remain unknown [1]. Osteogenic sarcoma is largely a
disease of the young but about one-third occurs in patients over 40 years of age [2] [5]. They are more common
in male than female and the vast majority of craniofacial Osteosarcomas are located in the zygomatic bone [6]
[7]. They are an aggressive neoplasm composed of spindle cells producing osteoid [8]. Clinical features are lo-
calized pain that frequently beg ins after injury.
Common presenting symptoms were swelling, pain, or both and the most important finding in physical ex-
amination is a soft tissue mass, which is fr equently large and tender to palpation. Radiological investigations in-
clude CT-scan and MRI. The general radiographic features are not specific but may be osteolytic, osteoblastic or
mixed [1 ]. The spicular pattern of calcification is usually present and no radiographic finding is pathognomonic
[1] [2]. The final diagnosis of Osteosarcoma was only established by the histological studies [8].
It can be classified into three main subtype s : the chondroblastic, osteoblastic, a nd fibroblastic types [7] [8].
The recommended treatment has not yet been established, but radical surgery for extirpation including a large
margin of normal bone is the most significant factor contributing to a good outcome. Adjuvant therapy, such as
irradiation or chemotherapy can be performed if radical resection cannot be achieved. The role of chemotherapy
is less clear for cranial tumors [6 ] [7].
The 10 years survival following resection of large tumors was 69% if the resection was complete but was only
13% if excision was incomplete [1] [2].
4. Conclusion
Because of the rarity of this type of tumor and a very poor prognosis, treatment of Osteosarcomas has not yet
benefited from a rigorous evaluation and needs more studies to research the role of chemo and radiotherapy.
Conflicts of Interest
All authors declare no conflict of interest.
[1] Benson, J.E., Goske, M., Han, J.S., et al. (1984) Primary Osteogenic Sarcoma of the Calvaria. American Journal of
Neuroradiology, 5, 810-813.
[2] Bose, B. (2002) Primary Osteogenic Sarcoma of the Skull. Surgical Neurology, 58, 234-240.
[3] Kanazawa, R., Yoshida, D., Takahashi, H., et al. (2003) Osteosarcoma Arising from the Skulle Case Report. Neurolo-
gia Medico-Chirurgica, 43, 88-91.
[4] Sturgis, E.M. and Potter, B.O. (2003) Sarcomas of the Head and Neck Region. Current Opinion in Oncology, 15, 239-
[5] Chennupati, S.K., Norris, R., Dunham, B. and Kazahaya, K. (2008) Osteosarcoma of the Skull Base: Case Report and
Review of Literature. International Journal of Pediatric Otorhinolaryngology, 72, 115-119.
[6] Chang, C.S. (2010) Craniofacial Reconstruction of Primary Osteogenic Sarcoma of the Skull. Journal of Plastic, Re-
constructive & Aesthetic Surgery, 63, 1265-1268.
[7] Saeter, G. (2005) Minimum Clinical Recommendations for Diagnosis, Treatment and Follow-Up of Osteosarcoma. An-
nals of Oncology, 16, 71-72.
[8] Fukunaga, M. (2005) Low-Grade Central Osteosarcoma of the Skull. Pathology-Research and Practice, 201, 131-135.
[9] Maurizio, S. (1994) Radiation-Induced Osteosarcomas of the Skull: Report of Two Cases and Review of the Literature.
Clinical Neurology and Neurosurgery, 96, 226-229.
B. Sofiene et al.
CT: Computed Tomography
MRI: Magnetic Resonance Imaging
OS: Osteos arco ma