Go Ronald S, Jacobsen Eric, Baiocchi Robert, Buhtoiarov Ilia, Butler Erin B, Campbell Patrick K, Coulter Don W, Diamond Eli, Flagg Aron, Goodman Aaron M, Goyal Gaurav, Gratzinger Dita, Hendrie Paul C, Higman Meghan, Hogarty Michael D, Janku Filip, Karmali Reem, Morgan David, Raldow Anne C, Stefanovic Alexandra, Tantravahi Srinivas K, Walkovich Kelly, Zhang Ling, Bergman Mary Anne, Darlow Susan D
Mayo Clinic Cancer Center.
Dana-Farber/Brigham and Women's Cancer Center | Massachusetts General Hospital Cancer Center.
J Natl Compr Canc Netw. 2021 Nov;19(11):1277-1303. doi: 10.6004/jnccn.2021.0053.
Histiocytic neoplasms are rare hematologic disorders accounting for less than 1% of cancers of the soft tissue and lymph nodes. Clinical presentation and prognosis of these disorders can be highly variable, leading to challenges for diagnosis and optimal management of these patients. Treatment often consists of systemic therapy, and recent studies support use of targeted therapies for patients with these disorders. Observation ("watch and wait") may be sufficient for select patients with mild disease. These NCCN Guidelines for Histiocytic Neoplasms include recommendations for diagnosis and treatment of adults with the most common histiocytic disorders: Langerhans cell histiocytosis, Erdheim-Chester disease, and Rosai-Dorfman disease.
组织细胞肿瘤是罕见的血液系统疾病,在软组织和淋巴结癌症中占比不到1%。这些疾病的临床表现和预后差异很大,给这些患者的诊断和最佳治疗带来了挑战。治疗通常包括全身治疗,最近的研究支持对这些疾病的患者使用靶向治疗。对于部分病情较轻的患者,观察(“观察和等待”)可能就足够了。这些NCCN组织细胞肿瘤指南包括针对患有最常见组织细胞疾病的成人的诊断和治疗建议:朗格汉斯细胞组织细胞增多症、 Erdheim-Chester病和Rosai-Dorfman病。