Clinical course of the bony lesion of single-system single-site Langerhans cell histiocytosis - Is appropriate follow-up sufficient treatment?

BACKGROUND: Langerhans cell histiocytosis (LCH) is categorized into three types, which include single-system single-site (SS-s), single-system multiple-site (SS-m) and multisystem (MS). The most commonly affected site in LCH is bone, and the bony lesion of SS-s LCH has a good prognosis. The bony lesion of SS-s LCH has been thought to regress spontaneously. Although treatments such as curettage, direct injection of corticosteroids, and chemotherapy have been performed, regular follow-up is the first line of treatment for the bony lesion of SS-s LCH. For preventing orthopedic sequelae, strict and appropriate follow-up should be performed, but the appropriate period and method of follow-up has not yet been established.

METHODS: In the present study, we retrospectively analyzed a series of 7 cases of patients with SS-s LCH with a bony lesion treated in the Department of Orthopedic Surgery at Kagoshima University Hospital (Kagoshima, Japan) from 2006 to 2015.

RESULTS: The bony lesion regressed spontaneously in all patients. Factors such as location, size, preoperative C-reactive protein (CRP) value, standardized uptake (SUV) value of positron emission tomography (PET), age, sex and direct steroid injection were not related to the clinical course. Temporary expansion of the lesion occurred in 3 patients and a temporary worsening of pain occurred in 1 patient during the follow-up period. These events occurred within 6 weeks after biopsy.

CONCLUSION: Careful follow-up and the use of an appropriate orthosis can lead to a good clinical course for the bony lesion of SS-s LCH. Future research should seek to determine the appropriate follow-up period.