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 Table of Contents  
CASE REPORT
Year : 2018  |  Volume : 8  |  Issue : 2  |  Page : 87-90

Primary extragnathic osteosarcoma with visual loss in an adolescent female


1 Department of Pathology, Faculty of Basic Clinical Sciences, Ahmadu Bello University and ABU Teaching Hospital, Zaria, Nigeria
2 Department of Maxillofacial Surgery, Neurosurgery Unit, Faculty of Clinical Sciences, Ahmadu Bello University and ABU Teaching Hospital, Zaria, Nigeria
3 Department of Surgery, Neurosurgery Unit, Faculty of Clinical Sciences, Ahmadu Bello University and ABU Teaching Hospital, Zaria, Nigeria

Date of Web Publication30-May-2019

Correspondence Address:
Prof. Modupeola O Samaila
Department of Pathology, Faculty of Basic Clinical Sciences, Ahmadu Bello University and ABU Teaching Hospital, Zaria
Nigeria
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ais.ais_2_19

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  Abstract 


The majority of primary osteosarcoma cases affect the metaphyseal plates of long bones, while approximately 5%–10% cases involve the craniofacial bones and are mainly located in the maxilla and mandible. Primary osteosarcoma of the cranial vault is uncommon, and it usually occurs as a metastatic focus from primary diseases elsewhere in older patients. An 18-year-old female presented to our hospital with a 6-month history of a left-sided skull mass involving the forehead and part of the left orbit and 3 months of left visual loss. Clinical examination revealed a 32 × 26 cm firm to soft mass involving the left frontal, parietal and temporal bones, and part of the left orbit. She had no other contributory complaints or significant medical history. A clinical diagnosis of orbital malignancy was made with differential of metastatic left-sided skull tumor. A computed tomography scan revealed a sclerotic lesion arising from the left parietal–temporal bones with sunburst appearances, while fine needle aspiration cytology of the mass showed loose clusters and singles of malignant spindle cells. Incisional biopsy of the mass was diagnosed as fibroblastic variant of osteosarcoma. Extragnathic primary osteosarcoma is uncommon and accounts for less than 1% of craniofacial osteosarcoma. In general, osteosarcoma has a bimodal age of occurrence in adolescents and adults >65 years, and the preferential sites of involvement are the femur, tibia, humerus, and gnathic bones in decreasing frequencies. Our patient is within the first age group; however, the site is unusual and is often associated with secondary osteosarcoma in adults >65 years.

Keywords: Adolescent, extragnathic, female, osteosarcoma, primary


How to cite this article:
Samaila MO, Abdullahi S, Aliyu HO, Fomete B, Jimoh A. Primary extragnathic osteosarcoma with visual loss in an adolescent female. Arch Int Surg 2018;8:87-90

How to cite this URL:
Samaila MO, Abdullahi S, Aliyu HO, Fomete B, Jimoh A. Primary extragnathic osteosarcoma with visual loss in an adolescent female. Arch Int Surg [serial online] 2018 [cited 2019 Sep 22];8:87-90. Available from: http://www.archintsurg.org/text.asp?2018/8/2/87/259457




  Introduction Top


Osteosarcoma is the most common malignant bone forming neoplasm with an estimated incidence of four to five cases per million people and accounts for less than 35% of all primary bone tumors.[1],[2],[3],[4] The majority of primary osteosarcoma cases affect the metaphyseal plates of long bones, while approximately 5%–10% cases involve the craniofacial bones and are mainly located in the maxilla and mandible (gnathic bones).[1],[4],[5],[6],[7] Primary osteosarcoma of the cranial vault is uncommon, and it usually occurs as a metastatic focus from primary diseases elsewhere in older patients.[3],[8] We present an 18-year-old female with primary osteosarcoma of the frontoparietal–temporal bones of the skull. This is the only extragnathic osteosarcoma case in a 20-year review in our pathology department.


  Case Presentation Top


An 18-year-old female presented to our hospital with a 6-month history of a left-sided skull mass involving the forehead and part of the left orbit. The mass had been progressively increasing in size and became associated with left visual loss 3 months prior to presentation. She had no other contributory complaints, antecedent trauma, or significant medical history. Clinical examination revealed a young ill-looking emaciated female, with a 32 × 26 cm firm to soft mass involving the left frontal, parietal, and temporal bones and part of the left orbit with complete left visual loss [Figure 1]. The mass extended backward and was 2 cm anterior to the left tragus. She had no obvious or palpable lymph nodes or masses elsewhere. A clinical diagnosis of orbital malignancy was made with differential of metastatic left-sided skull tumor from an unknown primary site. A fine needle aspiration cytology of the mass showed loose clusters and singles of malignant spindle cells, while computed tomography (CT) scan revealed a sclerotic lesion arising from the left parietal–temporal bones with sunburst periosteal reactions and the soft tissue components of the lesion extended intracranially in the left anterior and middle cranial fossa with encroachment of cerebral tissues and compression of the anterior horn of the ipsilateral ventricle. This was diagnosed as a locally invasive left cranial vault osteogenic sarcoma. An incisional biopsy of the mass was also sent for histopathological analysis and this revealed a necrotic, cellular, and hemorrhagic tumor dispersed in sheets of malignant spindle cells having pleomorphic hyperchromatic to vesicular nuclei with scanty cytoplasm and forming osteoid in a vascularized fibrocollagenized stroma [Figure 2]. There was no cartilage present in the lesion and it was diagnosed as fibroblastic variant of osteosarcoma. However, the patient was not managed in the hospital due to financial constraints following counseling on the disease progression and further management.
Figure 1: Fronto-temporal-parietal-orbital mass with eyeball extrusion

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Figure 2: Cellular tumour composed of malignant spindle cells and forming osteoid. H&E stain, Mag ×40

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  Discussion Top


Extragnathic primary osteosarcoma is uncommon and accounts for less than 1% of craniofacial osteosarcoma.[5] The preferential sites of involvement for primary osteosarcoma in decreasing frequencies are the distal third of femur, proximal third of tibia, thus making the knee area the most frequently affected anatomical location, and the upper third of the humerus around the shoulder girdle, the least common site involving metaphyseal bones.[1],[9] Gnathic bones, mainly the mandible and maxilla and pelvic bones, account for 8% of all primary osteosarcoma.[3],[4],[6],[7],[8],[10] This extragnathic case involving the cranial vault and comprising the frontal, parietal, and temporal bones is the only diagnosed case in our pathology department over a 20-year period where 31 gnathic osteosarcoma cases involving either the mandible or maxilla were recorded in the same period. Craniofacial osteosarcoma involving the temporal–parietal and temporal–sphenoid regions have also been documented in literature.[5],[11]

In general, osteosarcoma has a bimodal age of occurrence in adolescents and adults >65 years.[9] Our patient is within the adolescent age group; however, the extragnathic site is unusual and is often associated with secondary osteosarcoma in adults >65 years usually as a sequel of Paget's disease, trauma, irradiation, bone lesions such as fibrous dysplasia, and retinoblastoma.[8],[9],[12],[13] Our patient had no history of trauma, radiation, or bone disease. The mean age of occurrence of primary craniofacial osteosarcoma in a study covering a 28-year period was 19.7 years with no gender predilection.[14] This index case's age is comparable although the mean age for the departmental craniofacial (gnathic) cases was 13 years.

The definitive diagnosis of osteosarcoma is achieved with radiological and biopsy (needle aspiration and tissue) examinations as done for this adolescent female. The correct interpretation of radiographic finding requires expertise in recognizing and differentiating the various changes in bone mineralization, destruction, and periosteal bone formation to aid proper patient care.[11],[15] Sunburst periosteal reaction as seen in CT is not necessarily diagnostic but suggestive of a malignant bone neoplasm because inflammation of the bone as seen in osteomyelitis may also elicit the same sunburst response.[10] No other lesions or lung metastasis was detected from CT scan. Tissue biopsy of the lesion is mandatory, and the identification of neoplastic osteoid tissue formation is confirmatory of osteosarcoma. This tissue histopathological examination also differentiated osteosarcoma from other malignant tumors such as chondroblastoma, fibrosarcoma, and malignant fibrous histiocytoma. Orbital tumor was the clinical diagnosis in this case possibly due to visual loss; however, skull osteosarcoma may be associated with exophthalmia and visual loss as seen in this patient.[11],[16] The common possible orbital lesions include retinoblastoma, rhabdomyosarcoma, sebaceous carcinoma, and squamous cell carcinoma, all of which lack neoplastic osteoid formation.

Head and neck osteosarcoma cases are less aggressive and have better prognosis with surgery alone with localized unifocal tumors. However, extragnathic cases are more aggressive high-grade tumors with bad prognostic outcome of less than 1-year survival after diagnosis.[11],[17] High-grade osteosarcoma is characterized by rapid growth as seen in this case. Other high-grade histologic variants include osteoblastic, chondroblastic, small cell, telangiectatic, pagetoid, extraskeletal, and postradiation types.

The preferred treatment of osteosarcoma is a combination of surgery, chemotherapy, and radiotherapy, although the management of head and neck cases has not been standardized.[18] However, this patient did not benefit from any treatment in our hospital due to financial constraints. Surgery with complete resection is advocated for localized tumors but would not have been helpful in this case due to tumor extent intracranially and involvement of cerebral tissue. Extragnathic osteosarcoma should be considered in the differential diagnosis of tumors of the face involving the orbits and craniofacial bones in adolescents.

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 Top

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Jasnau S, Mever U, Potraz J, Jundt G, Kevric M, Joos UK, et al.; Cooperative Osteosarcoma Study Group. Craniofacila osteosarcoma Experience of the cooperative German-Austrian Swiss osteosarcoma study group. Oral Oncol 2008;44:286-94.  Back to cited text no. 14
    
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Cavalcanti MGP, Ruprecht A, Yang J. Radiological findings in an unusual osteosarcoma in the maxilla. Dentomaxillofac Radiol 2000;29:180-4.  Back to cited text no. 15
    
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Byme J, Fears TR, Whitney C, Parry DM. Survival after retinoblasyoma: Long term consequences and family historyof cancer. Med Pediatr Oncol 1995;24:160-5.  Back to cited text no. 16
    
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Fernandes GL, Natal MRC, Cruz CLP da, Nascif RL, Tsuno NSG, Tsuno MY. Primary osteosarcoma of the cranial vault. Radiol Bras 2017;50:263-5.  Back to cited text no. 17
    
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Ferguson WS, Goorin AM. Current treatment of osteosarcoma. Cancer Invest 2001;19:292-315.  Back to cited text no. 18
    


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