Department of Pathology and Biopathology Unit, Clinical Research Center, MOHAMMED VI University Hospital of Marrakech, Faculty of Medicine and Pharmacy of Marrakech, Cadi Ayyad University of Marrakech, Marrakech, Morocco.
Department of Oral and Maxillofacial surgery, MOHAMMED VI University Hospital of Marrakech, Faculty of Medicine and Pharmacy of Marrakech, Cadi Ayyad University of Marrakech, Marrakech, Morocco.
J Med Case Rep. 2022 Apr 28;16(1):176. doi: 10.1186/s13256-022-03359-x.
Mandible can be the site of benign or malignant lesions of different origins, including odontogenic and non-odontogenic lesions. Cartilage-forming tumors have been rarely reported at this site. Chondrosarcoma is a rare malignant cartilage-producing neoplasm that is extremely rare in the mandible. The rarity of cartilage-forming tumor occurrence in the mandible can make diagnosis difficult for pathologists, as they do not expect this type of tumor at this anatomical site. Here we report a case of chondrosarcoma of mandibular angle.
A 70-year-old Moroccan male patient consulted a dentist for wisdom tooth pain. Wisdom tooth extraction was conducted. After 6 months, the patient reported the recurrence of pain associated with swelling in the mandibular area and paresthesia along the path of the mandibular nerve. A panoramic radiograph demonstrated a mixed radiolucent-opaque lesion involving the mandibular angle. Computed tomography showed a large osteolytic spontaneously hypointense and multilobulated lesion. A biopsy was done. Histopathological examination revealed sheets and irregular lobules of atypical cells presenting cartilaginous differentiation. Tumor cells showed severe nuclear atypia and were located within a hyaline cartilage matrix. Some foci of necrosis were noted. Osteoid deposits were not found. The patient was diagnosed with grade III chondrosarcoma and underwent a right segmental mandibulectomy with submandibular lymph node dissection. Macroscopically, the tumor was localized in the mandibular angle with extension in the mandibular body. Histopathology confirmed the previous diagnosis of grade III chondrosarcoma and did not show any lymph node metastasis.
Owing to many histological similarities, grade III chondrosarcoma must be distinguished from chondroblastic osteosarcoma and metastatic lesions. In addition, chondroblastic osteosarcoma of the jawbones has a worse prognosis than chondrosarcoma, making the distinction between these two malignant tumors the most important concern of the pathologist when dealing with a cartilage-forming tumor at this site. Surgery with wide excision margins remains the best therapeutic approach, while the role of radiotherapy is controversial. The management of mandibular chondrosarcoma requires a multidisciplinary approach involving maxillofacial surgeons, radiologists, pathologists, and oncologists.
下颌骨可发生来自不同来源的良性或恶性病变，包括牙源性和非牙源性病变。在此部位很少报道有软骨形成性肿瘤。软骨肉瘤是一种罕见的恶性软骨性肿瘤，在下颌骨中极为罕见。下颌骨中软骨形成性肿瘤的罕见性使得病理学家难以诊断，因为他们在这个解剖部位不会预期到这种类型的肿瘤。在此，我们报告一例下颌角软骨肉瘤。
一名 70 岁的摩洛哥男性患者因智齿疼痛咨询牙医。进行了智齿拔除术。6 个月后，患者报告下颌区域疼痛复发，并伴有下颌神经路径的肿胀和感觉异常。全景片显示下颌角混合性透亮-不透光病变。计算机断层扫描显示大的溶骨性、自发性低信号、多叶状病变。进行了活检。组织病理学检查显示片状和不规则小叶状非典型细胞呈现软骨分化。肿瘤细胞显示严重的核异型性，位于透明软骨基质内。注意到一些坏死灶。未发现骨样沉积物。患者被诊断为 III 级软骨肉瘤，并接受了右侧下颌骨节段切除术和下颌下淋巴结清扫术。大体上，肿瘤位于下颌角，下颌体延伸。组织病理学证实了先前的 III 级软骨肉瘤诊断，并且未显示任何淋巴结转移。
由于许多组织学相似性，III 级软骨肉瘤必须与成软骨性骨肉瘤和转移性病变区分开来。此外，下颌骨的成软骨性骨肉瘤比软骨肉瘤预后更差，因此病理学家在处理该部位的软骨形成性肿瘤时，最关心的是区分这两种恶性肿瘤。广泛切除边缘的手术仍然是最佳治疗方法，而放射治疗的作用存在争议。下颌骨软骨肉瘤的治疗需要多学科方法，包括颌面外科医生、放射科医生、病理学家和肿瘤学家。
