Treatment recommendations are based on site and extension of the disease. Children must not be treated according to these recommendations (see childhood LCH).
Figure 1 Management of Langerhans Cell Histiocytosis in adults.
* Table 2, Table 3, Tabl ...
Careful observation, local or “mild systemic” therapy
In case of single system LCH with unifocal bone involvement of “non-CNS-Risk facial bones” local therapy and careful observation is recommended. The modality of treatment depends on location, size, and symptoms of the disease. Biopsy ...
Front line treatment
Systemic therapy should be considered in case of the following disease category:
MS-LCH with/without involvement of “risk organs”
SS-LCH with multifocal lesions
SS-LCH with “special site” lesions
There is no standard first li ...
Treatment options in case of reactivation
Reactivations of LCH in adults occur in about 25-38% of the patients (European national registry data and [Cantu et al 2012]). Patients may have further reactivations especially those with multisystem disease.
Reactivation of single system disease
In contrast to treatment of childhood LCH, radiotherapy is still a feasible and effective treatment option for adult patients with LCH. It has a long tradition and the first successful radiation of LCH was described in 1930 by Sosman. Sinc ...
Treatment and hormon replacement of endocrinopathies
Diabetes insipidus (DI) should be treated with desmopressin while the timing and dosage must be individualized. In proven LCH new onset DI is a sign of active disease and the LCH-CNS study group recommends the initiation of systemic therapy (chemothera ...
Central nervous system involvement
These lesions are most frequently observed in the hypothalamic-pituitary region. The tumor size ranges from discrete thickening of the pituitary stalk to larger tumors. Parenchymal, meningeal or choroid plexus lesions occur less freq ...