A typical radiographic presentation of osteosarcoma arising from skull and scapula: A rare case report

Vinay Kumar Reddy Kundoor; Rashmi Chelimala; Kotya Naik Maloth; Kesidi Sunitha; Moni Thakur

doi:10.4103/2321-3841.177058

CASE REPORT

Year : 2016 | Volume : 4 | Issue : 1 | Page : 14-17

A typical radiographic presentation of osteosarcoma arising from skull and scapula: A rare case report

Vinay Kumar Reddy Kundoor¹, Rashmi Chelimala¹, Kotya Naik Maloth¹, Kesidi Sunitha¹, Moni Thakur²
¹ Department of Oral Medicine and Radiology, Mamata Dental College and Hospital, Khammam, Telangana, India
² Department of Oral Pathology and Microbiology, Mamata Dental College and Hospital, Khammam, Telangana, India

Date of Web Publication

19-Feb-2016

Correspondence Address:
Kotya Naik Maloth
Department of Oral Medicine and Radiology, Mamata Dental College and Hospital, Giriprasad Nagar, Khammam - 507 002, Telangana
India

Source of Support: None, Conflict of Interest: None

Check

DOI: 10.4103/2321-3841.177058

Abstract

Osteosarcoma is an aggressive malignant tumor of the bone. It can occur in any bone, but long bones are affected more such as femur (42%), tibia (19%), and humerus (10%), compared to short bones such as skull, head, and neck region (<10%). The typical radiographic appearance of sunray pattern and Codman's triangle is highly suggestive of osteosarcoma. Early diagnosis and surgical treatment is the key to high survival rate. Here, we present a case with typical radiographic appearance of osteosarcoma arising from skull and scapula in a 25-year-old male patient.

Keywords: Codman′s triangle, osteogenic sarcoma, sunray appearance

How to cite this article:
Reddy Kundoor VK, Chelimala R, Maloth KN, Sunitha K, Thakur M. A typical radiographic presentation of osteosarcoma arising from skull and scapula: A rare case report. J Oral Maxillofac Radiol 2016;4:14-7

How to cite this URL:
Reddy Kundoor VK, Chelimala R, Maloth KN, Sunitha K, Thakur M. A typical radiographic presentation of osteosarcoma arising from skull and scapula: A rare case report. J Oral Maxillofac Radiol [serial online] 2016 [cited 2023 Mar 25];4:14-7. Available from: https://www.joomr.org/text.asp?2016/4/1/14/177058

Introduction

Osteosarcoma is a most common primary malignant aggressive tumor affecting long bones, and rarely affecting the short bones (ileum, clavicle, sacrum, scapula, and clavicle), skull, head, and neck region. The incidence is about 1%-2% of all skull tumors, ^[1],[2] and <10% in head and neck region. ^[3] The most common complications of osteosarcoma are bone pain, bone fracture, lung metastases, reduced red blood cells, increased alkaline phosphate levels, and loss of limb. ^[2] In the literature, only few cases are reported involving skull and short bones, and those of reported are frequently associated with osteosarcoma of the maxillomandibular complex. ^[4] Here, a rare case of osteosarcoma arising from skull and scapula is reported.

Case Report

A 25-year-old male patient reported to the department with a chief complaint of swelling on the right skull and left shoulder region with a 2-year history, which had rapidly enlarged within 4 months. Physical examination demonstrated a 10 cm × 14 cm firm to hard, nontender mass, fixed to bone in the right parietal-temporal area [Figure 1] and [Figure 2], and a similar mass measuring 8 cm × 6 cm on the left shoulder region with well-circumscribed macules 12 in number measuring approximately 1 cm × 1 cm [Figure 3]. Medical history was noncontributory. On laboratory investigations, alkaline phosphatase level was raised (329 IU/L). Radiographical examination with posterior-anterior skull view revealed well-defined radiopaque lesion involving right temporal and parietal bone with typical "sunray appearance" [Figure 4], a well-defined radiopaque lesion was also noted on scapula of the left shoulder and multiple radiopaque foci were seen in the lungs [Figure 5]. An axial view of skull computed tomography (CT) scan showed hyperdense lesion extending in the extra- and intra-cranial region with "sunray appearance" [Figure 6]. Lesion on the skull was surgically excised and sent for histopathology, which revealed small round cell proliferation with excessive fibrogenesis, myxoid area, and osteoid formation with occasional areas of chondroid. Neoplastic cells are scattered in groups, islands, and capillary structures. They were suggestive of small cell osteogenic sarcoma. Based on clinical, radiographical, and histopathological findings, a final diagnosis of small cell osteosarcoma was considered [Figure 7]. The patient was operated surgically and referred to higher center for chemotherapy, where high doses of intravenous (IV) methotrexate 12 g/m ² every week for 4 weeks and cisplatin IV 60 mg/m ² once in a 3 weeks for 3-month were administered, and the patient is under follow-up since 6-month [Figure 8].

Figure 1: Swelling present on the right side of head

Click here to view

Figure 2: Preoperative profile picture

Click here to view

Figure 3: Swelling present on the left shoulder with pigmentation

Click here to view

Figure 4: Posterior-anterior skull view showing radiopaque lesion with typical "sunray appearance"

Click here to view

Figure 5: Shoulder radiograph showing radiopaque lesion on scapula with metastasis of lung

Click here to view

Figure 6: Axial view of computed tomography scan showing radiopaque lesion in extra- and intra-cranial region with "sunray appearance"

Click here to view

Figure 7: Histopathology picture showing small round cell proliferation with fibrogenesis, myxoid, osteoid, and chondroid areas

Click here to view

Figure 8: Postoperative photograph after 1 month (a) Profile view (b) Lateral view

Click here to view

Discussion

Osteosarcoma is a common primary sarcoma of bone. It was first termed by French surgeon Alexis Boyer in 1805. ^[5] It can occur at any age, affecting mostly the younger children and about one-third occurs in older adults above 40 years of age. ^[1],[6] "Osteosarcoma is classified as a component of Ewing's sarcoma. Generally involving are long bones such as the femur, tibia, and humerus and very rarely arising are from the short bones such as ileum, clavicle, scapula, and sacrum." ^[7] The present case was seen in a 25-year-old male involving skull and scapula.

The exact etiology and pathogenesis of osteosarcoma are unknown, but according to Hudson et al., ^[8] the risk factors associated with osteosarcoma are rapid bone growth during the adolescence, environmental factors such as radiation, and genetic predisposition in bone dysplasias such as Paget's disease and fibrous dysplasia.

Males are affected more commonly than females with a ratio of 1.4:1. ^[2],[5] The majority of craniofacial lesions involve the zygomatic bone. ^[9] The most common presenting signs and symptoms are swelling, pain, or both, if the patient is asymptomatic, it is diagnosed accidentally or following a traumatic event. ^[10] In the present case, the patient reported with a painless swelling.

Hayden and Hoang ^[11] classified osteosarcoma into different subtypes,

Conventional osteoblastic (70%),
Chondroblastic and fibroblastic (10%),
Rare subtypes of anaplastic, telangiectatic, giant-cell rich, and small cell (<10%).

The fibroblastic subtype has good response to chemotherapy than chondroblastic subtype. The case reported here is a small cell subtype of osteosarcoma, which accounts <10%.

The plain radiography plays a major role in the initial diagnosis of bone tumors. ^[12] The typical or classic radiographic presentation of osteosarcoma is "sunray appearance" of the affected bone. ^[5] The extent of the tumor in both bone and soft tissue is best appreciated in higher imaging modalities such as CT scan and magnetic resonance imaging (MRI). ^[5] In the present case, MRI and positron emission tomography CT could not be done due to financial status of the patient, and he was more concerned about lesion on the skull due to esthetics and was not willing for any treatment for lesion on the scapula.

The treatment modalities depend on the severity and extent of the lesion manifested in the bone. Neoadjuvant chemotherapy, surgery, and postoperative chemotherapy are mainstay of the treatment. Large lesions can be treated with surgical excision, including large margins of normal bone to prevent recurrence. According to Kaste, ^[13] osteosarcoma metastasis to lungs has a recurrence rate of approximately 30%. The present case was treated surgically and referred to higher centers for chemotherapy, where combination of drugs such as methotrexate and cisplatin were administered.

Conclusion

Although osteosarcoma is a common aggressive malignant tumor of bone, its occurrence is rare in short bones and head and neck region. The clinician should be aware of the clinical and typical radiographic appearance of the lesion that may help in early diagnosis and prompt treatment to prevent the mortality, as it has a poor prognosis.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/ their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

References

1.	Bose B. Primary osteogenic sarcoma of the skull. Surg Neurol 2002;58:234-9.
2.	Sofiene B, Asma B, Adnene B, Imed BS, Jalel K, Lassaad A, et al. A case report of osteosarcoma of the skull. Open J Mod Neurosurg 2014;4:105-9.
3.	Yamamoto A, Sakamoto J, Muramatsu T, Hashimoto S, Shibahara T, Shimono M, et al. Osteosarcoma of maxilla with unusual image findings in child. Bull Tokyo Dent Coll 2011;52:201-7.
4.	Kanazawa R, Yoshida D, Takahashi H, Matsumoto K, Teramoto A. Osteosarcoma arising from the skull - Case report. Neurol Med Chir (Tokyo) 2003;43:88-91.
5.	Khorate MM, Goel S, Singh MP, Ahmed J. Osteosarcoma of mandible: A case report and review of literature. J Cancer Sci Ther 2010;2:122-5.
6.	Chennupati SK, Norris R, Dunham B, Kazahaya K. Osteosarcoma of the skull base: Case report and review of literature. Int J Pediatr Otorhinolaryngol 2008;72:115-9.
7.	Findik G, Günay E, Agaçkiran Y, Aydogdu K, Aydin E, Kaya S. Small cell osteosarcoma of rib: Diagnosis and treatment of the rare case. Tuberk Toraks 2012;60:172-5.
8.	Hudson M, Jaffe MR, Jaffe N, Ayala A, Raymond AK, Carrasco H, et al. Pediatric osteosarcoma: Therapeutic strategies, results, and prognostic factors derived from a 10-year experience. J Clin Oncol 1990;8:1988-97.
9.	Chang CS, Bergeron L, Liao CC, Liao HT, Chang CN, Chen PK, et al. Craniofacial reconstruction of primary osteogenic sarcoma of the skull. J Plast Reconstr Aesthet Surg 2010;63:1265-8.
10.	Guillon MA, Mary PM, Brugière L, Marec-Bérard P, Pacquement HD, Schmitt C, et al. Clinical characteristics and prognosis of osteosarcoma in young children: A retrospective series of 15 cases. BMC Cancer 2011;11:407.
11.	Hayden JB, Hoang BH. Osteosarcoma: Basic science and clinical implications. Orthop Clin North Am 2006;37:1-7.
12.	Gebhardt MC, Springfield D, Neff JR. Sarcomas of bone. In: Abeloff MD, Armitage JO, Lichter AS, Niederhuber JE, Kastan MB, McKenna WG, eds. Clinical Oncology. 4 ^th ed. Philadelphia, Pa.: Elsevier; 2008: 2471-2572.
13.	Kaste SC. Imaging pediatric bone sarcomas. Radiol Clin North Am 2011;49:749-65, vi-vii.