Nakhleh R E, Swanson P E, Dehner L P
Division of Surgical Pathology, University of Minnesota Medical School, Minneapolis.
Cancer. 1991 Feb 15;67(4):1019-24. doi: 10.1002/1097-0142(19910215)67:4<1019::aid-cncr2820670426>3.0.co;2-7.
Sites in the head and neck region (orbit, nasopharynx, nasal cavity, etc.) are among the most frequent locations for juvenile (embryonal and alveolar) rhabdomyosarcomas in patients younger than 15 years; however, comparable neoplasms in adults are very uncommon. A clinicopathologic and immunohistochemical study of 12 juvenile rhabdomyosarcomas in patients between the ages of 18 and 36 years is presented. There was a female:male ratio of 2:1. The orbit with or without contiguous paranasal sinus involvement, nasal cavity, sphenoid sinus, middle ear, and soft tissues of the neck and preauricular region were the primary sites. Seven tumors involved a parameningeal site and eight cases were alveolar rhabdomyosarcomas which together contributed to the adverse outcome. Only two patients were long-term, disease-free survivors. Six patients have died of tumor and two others are alive with persistent disease. Immunohistochemical study in 11 cases demonstrated reactivity for vimentin and muscle-specific actin (HHF-35) and desmin in ten cases. Juvenile rhabdomyosarcoma rarely presents in the head and neck of adults but should be considered in the differential diagnosis of a small cell neoplasm in patients during the third and fourth decades of life.
头颈部区域（眼眶、鼻咽、鼻腔等）是15岁以下患者中青少年（胚胎型和腺泡型）横纹肌肉瘤最常见的发病部位之一；然而，成人中类似的肿瘤非常罕见。本文介绍了一项对18至36岁患者的12例青少年横纹肌肉瘤的临床病理和免疫组织化学研究。男女比例为2:1。眼眶伴或不伴有相邻鼻窦受累、鼻腔、蝶窦、中耳以及颈部和耳前区域的软组织是主要发病部位。7例肿瘤累及脑膜旁部位，8例为腺泡型横纹肌肉瘤，这些共同导致了不良预后。只有2例患者为长期无病生存者。6例患者死于肿瘤，另外2例患者仍存活但疾病持续存在。11例患者的免疫组织化学研究显示波形蛋白、肌肉特异性肌动蛋白（HHF-35）呈阳性反应，10例患者结蛋白呈阳性反应。青少年横纹肌肉瘤很少出现在成人的头颈部，但在二三十岁患者小细胞肿瘤的鉴别诊断中应予以考虑。
