Gutjahr P, Jung H
Laryngol Rhinol Otol (Stuttg). 1976 May;55(5):404-9.
Histiocytosis X is an entity of neoplastic diseases of unknown etiology and pathogenesis. In the own case material, half of the patients presented with clinical symptoms originating in the head and neck areas (7/14). With respect to the clinical course, localized histiocytosis X should be distinguished from acute-disseminated and chronic-disseminated disease, the three corresponding with the classical eosinophilic granuloma, Hand-Schüller-Christian's and Abt-Letterer-Siwe's disease; transitional forms are well-known. Therapy is surgical in cases of localized eosinophilic granuloma and cytostatic in cases of disseminated disease; in the latter Vinca-alkaloids and corticosteroids are preferably used. Any kind of treatment, however, has to be individualized and modifications result from the thorough observation of the clinical course, which is unpredictable for the individual case. Planning an individual concept of therapy for each patient is most important and should be done by surgeons together with the pediatric oncologist.
组织细胞增多症X是一组病因和发病机制不明的肿瘤性疾病。在本病例资料中,半数患者出现起源于头颈部区域的临床症状(7/14)。就临床病程而言,局限性组织细胞增多症X应与急性播散性和慢性播散性疾病相区分,这三种情况分别对应经典的嗜酸性肉芽肿、汉-许-克病和勒-雪病;过渡形式也很常见。局限性嗜酸性肉芽肿病例采用手术治疗,播散性疾病采用细胞毒性药物治疗;后者优选使用长春花生物碱和皮质类固醇。然而,任何一种治疗都必须个体化,治疗方案的调整源于对临床病程的密切观察,而临床病程对于个别病例来说是不可预测的。为每位患者制定个体化的治疗方案至关重要,应由外科医生与儿科肿瘤学家共同完成。