Dinakar J, Gowri S, Ann Tryphena E T
Department of Oral Pathology, Rajas Dental College and Hospital, Kavalkinaru, Tirunelveli, Tamil Nadu, India.
Department of Oral Pathology and Microbiology, Sri Ramakrishna Dental College and Hospital, Coimbatore, Tamil Nadu, India.
J Oral Maxillofac Pathol. 2023 Apr-Jun;27(2):406-410. doi: 10.4103/jomfp.jomfp_534_22. Epub 2023 Jul 13.
Rhabdomyosarcoma (RMS) is the most common sarcoma among children and accounts for 20% of soft tissue sarcomas. In children, close to 50% of rhabdomyosarcomas arise in the head and neck. RMS of the oral cavity is rare and is seen in only 10-12% of all head and neck lesions and the involvement of the jaws is extremely rare. Histopathologically, the various types are pleomorphic type, botryoid type, spindle cell type, embryonal, and alveolar type of RMS. The alveolar variant accounts for almost 30% of all rhabdomyosarcomas and tends to arise in patients of the age group 10-25 years. We present a case of orofacial RMS in a young adult who was referred to our Institution for the management of an odontogenic lesion of the maxilla. The clinicopathological aspects and poor survival rate as a consequence of delayed diagnosis are discussed. We dentists may misdiagnose it as an odontogenic tumour due to its location in the oral and maxillofacial region. Careful clinical history and examination and investigations may help to narrow down the diagnosis. Expert opinion and referrals to oral pathologists and oncologists are essential to arrive at early diagnosis and to initiate the treatment.
横纹肌肉瘤（RMS）是儿童中最常见的肉瘤，占软组织肉瘤的20%。在儿童中，近50%的横纹肌肉瘤发生于头颈部。口腔横纹肌肉瘤很罕见，仅占所有头颈部病变的10 - 12%，累及颌骨的情况极为罕见。组织病理学上，横纹肌肉瘤的各种类型包括多形性型、葡萄状型、梭形细胞型、胚胎型和肺泡型。肺泡型变体占所有横纹肌肉瘤的近30%，倾向于发生在10 - 25岁年龄组的患者中。我们报告一例年轻成人的口面部横纹肌肉瘤病例，该患者因上颌骨牙源性病变被转诊至我院。本文讨论了该病例的临床病理特征以及因诊断延迟导致的低生存率。由于其位于口腔颌面部区域，我们牙医可能会将其误诊为牙源性肿瘤。仔细的临床病史、检查和调查有助于缩小诊断范围。专家意见以及转诊至口腔病理学家和肿瘤学家对于早期诊断和启动治疗至关重要。
