Choo Choon Sean, Wan Abdul Rahman Wan Faiziah, Jaafar Hasnan, Ramli Ramiza Ramza
Department of Otorhinolaryngology - Head and Neck Surgery, School of Medical Sciences, Universiti Sains Malaysia, Health Campus, Kota Bharu, Kelantan, Malaysia.
Department of Pathology, School of Medical Sciences, Universiti Sains Malaysia, Health Campus, Kota Bharu, Kelantan, Malaysia.
BMJ Case Rep. 2019 Mar 9;12(3):e228969. doi: 10.1136/bcr-2018-228969.
Chondrosarcoma (CS) is a malignant tumour of long and flat bone characterised by the formation of cartilage. Mesenchymal chondrosarcoma (MCS) is a rare subtype of CS that is more aggressive and may lead to erroneous diagnosis in a limited biopsy. The diagnosis is mainly based on the histopathological appearance of biphasic pattern of undifferentiated small round cells separated by islands of well-differentiated hyaline cartilage. We report a case of 13-year-old boy who initially presented with gum swelling and the biopsy result suggested a benign fibrous lesion. Following an extensive lesion shown in radiologic findings, the tumour excision was done and finally was diagnosed as an MCS of the maxilla. The patient was given postoperative chemotherapy (EURO-EWING 99 regimen), and now on regular follow-up for monitoring of local recurrence or tumour metastasis.
软骨肉瘤（CS）是一种发生于长骨和扁骨的恶性肿瘤，其特征为软骨形成。间叶性软骨肉瘤（MCS）是CS的一种罕见亚型，侵袭性更强，在有限的活检中可能导致误诊。诊断主要基于组织病理学表现，即由分化良好的透明软骨岛分隔的未分化小圆形细胞的双相模式。我们报告一例13岁男孩，最初表现为牙龈肿胀，活检结果提示为良性纤维病变。根据影像学检查发现的广泛病变，进行了肿瘤切除，最终诊断为上颌骨MCS。患者接受了术后化疗（EURO-EWING 99方案），目前正在定期随访，以监测局部复发或肿瘤转移。
