Primary orbital extraskeletal osteosarcoma and review of literature

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.

Ectopic Fibrous Dysplasia Presenting As a Well-Circumscribed Orbital Mass

“…[11][12][13] Another entity is extraskeletal osteosarcoma, an aggressive malignant mesenchymal neoplasm that produces cartilaginous or osteoid material and arises from soft tissue without attachment to the periosteum or skeleton. 14 However, in this case, the authors speculate that this patient's tumor originated from an ectopic focus of osteoblasts derived from embryonic origin or differentiation of pluripotent orbital adipose stem cells. 15 In summary, this case highlights fibrous dysplasia as a possibility in the differential diagnosis of a well-circumscribed orbital mass and underscores the importance of considering a broad differential diagnosis when evaluating and treating patients with orbital tumors.…”

“…11–13 Another entity is extraskeletal osteosarcoma, an aggressive malignant mesenchymal neoplasm that produces cartilaginous or osteoid material and arises from soft tissue without attachment to the periosteum or skeleton. 14 However, in this case, the authors speculate that this patient’s tumor originated from an ectopic focus of osteoblasts derived from embryonic origin or differentiation of pluripotent orbital adipose stem cells. 15…”

Osteosarcoma in the Thyroid Gland

“…These tumors comprise 2–5% of all osteosarcomas and approximately 1% of soft-tissue sarcomas [1]. They have a composition similar to that of the bone or cartilage [2]. These types of osteosarcomas tend to occur in older patients, and they confer a worse prognosis than bone osteosarcomas [1].…”