Chigurupati R, Alfatooni A, Myall R W T, Hawkins D, Oda D
Department of Oral and Maxillofacial Surgery, School of Dentistry, University of Washington, Seattle, USA.
Oral Oncol. 2002 Jul;38(5):508-15. doi: 10.1016/s1368-8375(01)00087-2.
Rhabdomyosarcoma (RMS) is an aggressive malignant skeletal muscle neoplasm arising from embryonal mesenchyme. It accounts for over 50% of all pediatric soft tissue sarcomas. The head and neck region is the most common site for this tumor in children. Neonatal presentation of this tumor is rare. We present the management of one neonatal case and three additional cases of orofacial RMS in children under the age of 7 years. All four patients were seen in the department of oral and maxillofacial surgery at Children's Hospital and Regional Medical Center (CHRMC) in Seattle between 1992-2000. Three of the four cases were alveolar RMS and one was botryoid sub-type of embryonal RMS. Three patients were treated with a combination of surgery, chemotherapy and radiation, while the patient with botryoid RMS was treated with surgery and chemotherapy only. The patient with congenital RMS died at 2.5 years of age due to recurrent metastatic disease. The other three patients are alive without evidence of recurrent with a mean follow up was 5.5 years (range 2.5-8.5 years). We discuss the current management, diagnosis, biological behavior, histopathology, prognosis and survival of head and neck RMS in neonates and young children.
横纹肌肉瘤（RMS）是一种起源于胚胎间充质的侵袭性恶性骨骼肌肿瘤。它占所有儿童软组织肉瘤的50%以上。头颈部是儿童该肿瘤最常见的部位。新生儿期出现这种肿瘤很罕见。我们介绍了1例新生儿病例以及另外3例7岁以下儿童口面部RMS的治疗情况。1992年至2000年期间，所有4例患者均在西雅图儿童医院及区域医疗中心（CHRMC）的口腔颌面外科就诊。4例病例中有3例为肺泡型RMS，1例为胚胎型RMS的葡萄状亚型。3例患者接受了手术、化疗和放疗联合治疗，而葡萄状RMS患者仅接受了手术和化疗。先天性RMS患者因复发性转移性疾病于2.5岁死亡。其他3例患者存活，无复发迹象，平均随访时间为5.5年（范围2.5 - 8.5年）。我们讨论了新生儿和幼儿头颈部RMS的当前治疗、诊断、生物学行为、组织病理学、预后和生存情况。
