Dabas Surendra K, Menon Nandini N, Ranjan Reetesh, Gurung Bikas, Tiwari Sukirti, Bassan Bharat Bhushan, Shukla Himanshu, Pasricha Sunil, Sinha Ajit, Kapoor Rahul, Verma Vinay Kumar, Verma Devesh, Arora Saurabh, Sharma Ashwani, Mukharjee Sourabh, Singal Rishu, Fernandes Trishala Bhadauria
Department of Surgical oncology, BLK- MAX Super specialty hospital, Pusa road, Rajendra Place, Delhi, 110005 India.
Pathology Department, Rajiv Gandhi Cancer Institute & Research Centre, New Delhi, 110085 India.
Indian J Otolaryngol Head Neck Surg. 2024 Feb;76(1):1290-1297. doi: 10.1007/s12070-023-04271-6. Epub 2023 Oct 14.
Chondrosarcomas are rare malignancies of the cartilage and myxoid chondrosarcoma is its variant which commonly occurs in soft tissue of extremities. Extraskeletal chondrosarcoma is a rare malignant neoplasm of bone or soft tissue origin and is characterized by the presence of spindle cells admixed with well differentiated cartilage or chondroid stroma. They are mostly radioresistant tumours and surgical resections with adequate margins is considered as the ideal treatment modality with adjuvant radiotherapy in high grade tumours and add on chemotherapy, in case of presence of poor prognostic factors.
A 51-year-old diabetic, hypertensive female patient presented to our outpatient department with difficulty in chewing food for a duration of 6 months. On clinical examination, she had an ulceroproliferative growth involving right lower alveolus and floor of mouth. MRI face and neck with contrast showed a 4.1 × 2.9 × 4.5 cm lesion involving right lower alveolus extending to floor of mouth. Biopsy showed features of extraskeletal myxoid chondrosarcoma. She was planned for upfront surgery (Right composite resection with modified radical neck dissection with free fibula flap). Patient was stable post-surgery and was discharged in stable condition. Final histopathology report was high grade myxoid chondrosarcoma. The case was presented in tumour board and the patient was planned for adjuvant radiotherapy. She has been on regular follow up for the past 2 years and shows no signs of recurrence.
Extraskeletal myxoid chondrosarcoma of oral cavity is a rare entity and very few cases are reported. It is a malignant neoplasm which is diagnosed with the help of immunohistochemistry. Surgery is the ideal modality of treatment accompanied by adjuvant radiotherapy in cases of high-grade tumours.
软骨肉瘤是一种罕见的软骨恶性肿瘤,黏液样软骨肉瘤是其变体,常见于四肢软组织。骨外软骨肉瘤是一种罕见的起源于骨或软组织的恶性肿瘤,其特征是存在与分化良好的软骨或软骨样基质混合的梭形细胞。它们大多是放射抗拒性肿瘤,手术切除切缘足够被认为是理想的治疗方式,对于高级别肿瘤可辅助放疗,若存在不良预后因素则加用化疗。
一名51岁患有糖尿病、高血压的女性患者因咀嚼食物困难6个月前来我院门诊就诊。临床检查发现,她有一个溃疡增生性肿物,累及右下牙槽和口腔底部。面部和颈部增强MRI显示一个4.1×2.9×4.5厘米的病变,累及右下牙槽并延伸至口腔底部。活检显示为骨外黏液样软骨肉瘤特征。她被安排进行一期手术(右侧联合切除加改良根治性颈清扫术及游离腓骨瓣)。患者术后情况稳定,出院时病情平稳。最终组织病理学报告为高级别黏液样软骨肉瘤。该病例在肿瘤讨论会上进行了汇报,患者被安排进行辅助放疗。在过去2年里她一直定期随访,未出现复发迹象。
口腔骨外黏液样软骨肉瘤是一种罕见的疾病,报道的病例很少。它是一种借助免疫组织化学进行诊断的恶性肿瘤。手术是理想的治疗方式,对于高级别肿瘤可辅以辅助放疗。