Extraskeletal osteosarcoma in right neck subcutaneous tissue: A case report of an extremely rare tumour

Abstract

Extraskeletal osteosarcoma (ESOS) is a rare soft-tissue sarcoma that is treated with surgical resection, chemotherapy and radiotherapy; however, as it is now considered to be radiation resistance, it is associated with conflicting management principles and poor outcomes. A multimodality approach is currently used to treat ESOS, which entails the incorporation of multidrug chemotherapy and/or radiotherapy coupled with surgery to obtain the best outcome; however, there are many factors that influence the treatment effects and clinical outcomes of ESOS. In the present study, a case of an 81-year-old man who suffered from primary ESOS in the subcutaneous tissue of the right-hand side of the neck was reported. The patient was treated several times with partial resection and once with radiotherapy, and was still living following 3 years of follow-up. Thus, the present case report demonstrated that surgical resection and postoperative radiotherapy regimens may be favourable in the short term with a disease-free survival of ~15 months; however, patients are prone to relapse.

A very rare mass mimicking paraganglioma in the parapharyngeal area: extraskeletal osteosarcoma

“…Extraskeletal osteosarcoma is a rare malignant soft tissue sarcoma with a histological similarity to primary bone osteosarcoma, but not attached to bone or periosteum. 1 , 2 , 3 , 4 , 5 , 6 , 7 , 8 , 9 Extraskeletal osteosarcoma is very rare. The incidence of ESOS is so low that the annual incidence in a study conducted in Norway; it was expressed as 0.2 per million.…”

“…If it can be accepted today, it is a wide surgical resection. 1 , 2 , 3 , 4 , 5 , 6 , 7 , 8 , 9 However, recently, multi-approach treatment modalities are recommended. However, the efficacy and use of chemotherapy and radiotherapy in these approaches are not completely clear.…”

“…Extraskeletal osteosarcoma (ESOS) is a rare malignant soft tissue sarcoma with a histological similarity to primary bone osteosarcoma but not attached to bone or periosteum. 1 , 2 , 3 , 4 , 5 , 6 , 7 , 8 , 9 It accounts for 1% of all soft tissue sarcomas and about 4% of osteogenic osteosarcomas. 1 , 2 , 6 , 8 Since first described by Wilson in 1941, 10 only several major series have been published.…”

“…3 , 8 Although tumor of advanced age group, the average median age group is 60. 1 , 2 , 3 , 4 , 5 , 6 , 7 , 8 , 9 In the literature, studies are emphasizing male dominance equally or female dominance. 2 , 3 , 5 , 8 Extraskeletal osteosarcoma is a very rare subtype that makes up 4%–5% of all osteosarcomas.…”

“… 2 , 3 , 5 , 8 Extraskeletal osteosarcoma is a very rare subtype that makes up 4%–5% of all osteosarcomas. 1 , 2 , 3 , 4 , 5 , 6 , 7 , 8 , 9 The lower extremity, shoulder girdle, upper extremity, and retroperitoneum, especially the thigh and hip, are the most common places for ESOS; 1 , 2 , 3 , 4 , 5 , 6 , 7 , 8 , 9 these tumors are rarely seen in the head and neck region. 1 , 2 , 3 , 5 , 6 , 7 Only 6%–10% of patients present with tumors in the head and neck area; in such cases, the mandible and maxilla are the dominant places.…”