Chi Angela C, Barnes Jeffrey D, Budnick Steven, Agresta Samuel V, Neville Brad
Division of Oral Pathology, Department of Stomatology, College of Dental Medicine, Medical University of South Carolina, Charleston, SC 29425, USA.
J Periodontol. 2007 Sep;78(9):1839-45. doi: 10.1902/jop.2007.060454.
Rhabdomyosarcoma is a malignant neoplasm of primitive mesenchyme exhibiting skeletal muscle differentiation. Oral rhabdomyosarcoma is rare and accounts for only 0.04% of all head and neck malignancies.
A 33-year-old woman presented with an erythematous gingival mass involving the anterior maxillary gingiva. The lesion had been present for > or =13 months before presentation, and in recent months, it had become intermittently painful.
Clinical examination exhibited erythema and enlargement of the interdental papillae between the left maxillary canine, lateral incisor, and central incisor. The tissue was boggy and tender on palpation. Incisional biopsies were performed, and microscopic examination showed a cellular proliferation of spindle-shaped to ovoid cells with hyperchromatic, enlarged, and pleomorphic nuclei. Many of the tumor cells exhibited abundant eosinophilic cytoplasm. Immunohistochemical stains showed the tumor cells to be positive for desmin, myogenin, and myogenic differentiation 1 (MyoD1). A diagnosis of embryonal rhabdomyosarcoma was made. The patient was treated by surgical resection with postoperative chemotherapy and radiation. The patient had no evidence of disease at a follow-up examination 1 month after completion of therapy.
Oral rhabdomyosarcoma can develop insidiously. Pain is a variable presenting symptom, and early lesions may be mistaken for benign neoplastic, inflammatory, or infectious processes. Over several decades, a multidisciplinary treatment approach that includes surgical removal if resectable, in combination with multiagent chemotherapy and possibly radiation therapy, has improved survival rates.
横纹肌肉瘤是一种具有骨骼肌分化的原始间充质恶性肿瘤。口腔横纹肌肉瘤罕见,仅占所有头颈部恶性肿瘤的0.04%。
一名33岁女性患者,上颌前部牙龈出现红斑性牙龈肿物。该病变在就诊前已存在≥13个月,近几个月间歇性疼痛。
临床检查发现左上颌尖牙、侧切牙和中切牙之间的牙间乳头红斑及肿大。触诊时组织质地松软且有压痛。进行了切开活检,显微镜检查显示梭形至卵圆形细胞呈细胞增殖,细胞核深染、增大且形态多样。许多肿瘤细胞显示有丰富的嗜酸性细胞质。免疫组化染色显示肿瘤细胞结蛋白、肌细胞生成素和肌源性分化1(MyoD1)呈阳性。诊断为胚胎性横纹肌肉瘤。患者接受了手术切除,并术后进行化疗和放疗。在治疗完成后1个月的随访检查中,患者无疾病证据。
口腔横纹肌肉瘤可隐匿发生。疼痛是一种可变的症状表现,早期病变可能被误诊为良性肿瘤、炎症或感染性病变。几十年来,多学科治疗方法,包括可切除时手术切除,联合多药化疗以及可能的放疗,提高了生存率。