Primary orbital extraskeletal osteosarcoma and review of literature
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CASE REPORT
Open Access
Primary orbital extraskeletal osteosarcoma and review of literature Jingwen Hui1,2,3,4, Yun Zhao1,2,3,4, Lei Zhang1,2,3,4, Jinyong Lin1,2,3,4 and Hong Zhao1,2,3,4*
Abstract Background: Extraskeletal osteosarcoma is a malignant tumour composed of an osteoid and/or cartilaginous matrix; it arises in soft tissues without connection to the skeleton, and to our knowledge, this type of tumour is extremely rare. Case presentation: The present study reports a 57-year-old man with primary orbital extraskeletal osteosarcoma who presented with a history of painful swelling in the left orbit that had occurred for 11 months. Imaging of the orbit showed an atypical, well-defined heterogeneous mass attached to the posterior globe of the left orbit. The patient underwent an anterior orbitotomy and complete excision of the tumour. The mass was originated from neither the globe nor the bony orbital wall but from the soft tissue. Histopathology demonstrated an extraskeletal osteosarcoma. After 13 months of follow-up, there was apparent recurrence of the tumour. The medical history showed no complaints of previous trauma or radiotherapy. Conclusions: ESOS is a highly malignant tumour. Immunosuppression, trauma and adjuvant radiotherapy are possible predisposing factors in the development of this tumour. Prompt recognition and thorough treatment are essential for preventing orbital lesions and presence of metastasis from other organs. Keywords: Orbit, Osteosarcoma, Extraskeletal osteosarcoma
Background Extraskeletal osteosarcoma (ESOS) is an extremely rare tumour that is not usually located in the orbit. It is defined as a malignant mesenchymal neoplasm that produces an osteoid and/or cartilaginous matrix and arises in soft tissues without connection to the skeleton. It is rare and comprises fewer than 5% of all osteosarcomas. Only 4 previous cases of orbital ESOS have been reported. Extraskeletal osteosarcoma primarily affects patients over the age of 50 years and has a poor prognosis. Our patient is the oldest and the fourth case ever detected with primary orbital ESOS without a history of previous radiotherapy or trauma. In this report, we * Correspondence: [email protected] 1 Tianjin Eye Hospital, Tianjin Eye Institute, NO.4 Gansu Road, Heping District, Tianjin 300020, China 2 Tianjin Key Lab of Ophthalmology and Visual Science, Tianjin, China Full list of author information is available at the end of the article
describe the clinical, radiologic, and pathologic records of a rare case of primary orbital extraskeletal osteosarcoma.
Case presentation A 57-year-old male presented with painful swelling in the left orbit that had occurred for 11 months. The medical history showed no chronic systemic disease, sinus infection, mucocele, previous trauma, previous eye surgery or irradiation. We performed a complete ophthalmic examination. Best-corrected visual acuity was 20/25 OD and 20/70 OS. Examination of the left eye revealed a 3 mm proptosis and maximal restriction of extraocular movements in all gazes.
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