Chondrosarcoma of the Mandible: A Challenging Lesion

Hajira Khatoon, Sahana NS, Suresh T, Meghashyama Kulkarni, Renuga S, Rhea Verghese
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Abstract

Chondrosarcomas of the maxillofacial region are rare and account for 1-3 of chondrosarcomas of the entire body with a predilection towards the anterior maxilla and a lower incidence in mandible. Clinical and radiographic findings are non-specific hence histopathological evaluation forms the basis for definitive diagnosis. This is a case of a 27-year-old female with a complaint of pain and swelling in her lower right back tooth region for one month. The swelling caused gross facial disfigurement with no secondary changes. Intraorally the growth involved both buccal and lingual sulci causing mobility of involved teeth. Radiographically severe periodontal bone loss was noted surrounding 47. Incisional biopsy was taken and sent for histopathological evaluation. The histopathological examination revealed lobular pattern of growth filled with sheets of round to polygonal cells showing pleomorphism and bizarre hyperchromatic nuclei along with a typical mitotic figures surrounded by densely packed spindle-shaped cells. In some areas chondroid matrix deposition with lacunae containing atypical chondrocytes was evident. Ki-67 IHC was done which showed 10 positivity. Hence the lesion was diagnosed as Grade I Chondrosarcoma. It must be differentiated from chondroblastic osteosarcoma as it bears close resemblance with it. The treatment is completely different for both entities. Chondrosarcoma is chemo-resistant and radioresistant whereas osteosarcoma is a highly chemosensitive tumor. Surgical resection is the treatment of choice in chondrosarcoma hence hemi-mandibulectomy was carried out in this case.
下颌骨软骨肉瘤:一个具有挑战性的病变
颌面部软骨肉瘤是一种罕见的软骨肉瘤,占全身软骨肉瘤的1-3,多发于上颌前部,下颌骨发病率较低。临床和影像学表现是非特异性的,因此组织病理学评估是明确诊断的基础。这是一个27岁女性的病例,主诉在她的右下后牙区域疼痛和肿胀一个月。肿胀引起严重的面部畸形,无继发变化。口腔内的生长累及颊沟和舌沟,引起受累牙齿的活动。x线片显示47周围严重的牙周骨丢失。采取切口活检并送去组织病理学评估。组织病理学检查显示小叶型生长,充满圆形到多边形的细胞片,具有多形性和奇异的深染核,并有典型的有丝分裂象,被密集排列的纺锤形细胞包围。在某些区域,软骨样基质沉积与含非典型软骨细胞的腔隙明显。Ki-67免疫组化显示10例阳性。因此诊断为I级软骨肉瘤。它与成软骨性骨肉瘤有相似之处,必须与之鉴别。这两个实体的处理方式完全不同。软骨肉瘤具有化疗耐药和放射耐药,而骨肉瘤是一种高度化疗敏感的肿瘤。手术切除是软骨肉瘤的治疗选择,因此本病例进行了半下颌切除术。
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