Changes to the Summary (08/15/2013)
The PDQ cancer information summaries are reviewed regularly and updated as new information becomes available. This section describes the latest changes made to this summary as of the date above.
Added text to state that this concept was further supported by a study reporting that the transcription profile of LCH cells was distinct from myeloid and plasmacytoid dendritic cells, as well as epidermal Langerhans cells (cited Hutter et al. as reference 5).
Added text about a group that tested flow-sorted CD1a cells from fresh lesions and found 11 of 16 had the BRAF mutation; they also identified another mutation, BRAF 600 DLAT, which demonstrated upregulation of ERK (cited Satoh et al. as reference 23). Also added text to state that a study of pulmonary lesions from five adults with lung LCH using a next-generation sequencing method to identify mutational hot spots in 46 cancer genes found two of five patients had the BRAF V600E mutation in all nodules tested (cited Yousem et al. as reference 24).
Revised text to state that in single-system LCH, as the name implies, the disease presents with involvement of a single site or organ, including skin and nails, oral cavity, bone, lymph nodes and thymus, pituitary gland, and thyroid.
The Skin and nails subsection was renamed from Skin.
Added text to state that fingernail involvement is an unusual finding that may present as a single site or with other sites of LCH involvement. There are longitudinal, discolored grooves and loss of nail tissue. This condition usually responds to the common LCH therapies (cited Ashena et al. as reference 6).
The Oral cavity subsection was renamed from Oral mucosa.
Revised text to state that in multisystem LCH, the disease presents in multiple organs or body systems including bone, abdominal/gastrointestinal system (liver and spleen), lung, bone marrow, endocrine system, eye, CNS, skin, and lymph nodes.
Added Odame et al. as reference 24.
Added Ocular as a new subsection.
Added Haupt et al. as reference 1.
Added text to state that many patients with multisystem disease will experience long-term sequelae due to their underlying disease and/or treatment. Endocrine and central nervous system sequelae are the most common; these long-term sequelae significantly affect health quality of life in many of these patients (cited Nanduri et al. as reference 4 and level of evidence 3iiiC).
Added text to state that the LCH-III study randomized risk organ–affected patients to either velban/prednisone/6-mercaptopurine or velban/prednisone/6-mercaptopurine plus methotrexate (cited Gadner et al. as reference 26). The response rates at 6 and 12 weeks and overall survival were not improved; however, there were significantly increased grade 3 and grade 4 toxicities in patients who received methotrexate.
Added text to state that patients without risk-organ involvement who were randomized to 12 months of velban/prednisone had a lower 5-year reactivation rate than patients who received only 6 months of treatment and patients treated with historical 6-month schedules.
Added Mottl et al. as reference 12 and level of evidence 3iiiDiv.
Added text to state that in a study of 40 patients who were carefully screened for late effects, adverse quality-of-life scores were found in more than 50% of patients. Seventy-five percent of patients had detectable long-term sequelae—hypothalamic/pituitary dysfunction (50%), cognitive dysfunction (20%), and cerebellar involvement (17.5%) being the most common (cited Nanduri et al. as reference 2).
Added text to state that a group of clinicians experienced in the care of adult LCH patients have published a consensus opinion on the evaluation and treatment of adult LCH patients (cited Girschikofsky et al. as reference 1).
The Skin and oral cavity subsection was renamed from Skin and oral mucosa.
Added text to state that a German cooperative radiation therapy group reported on a series of 98 adult LCH patients, most of whom (60 of 98) had only bone lesions, and 24 had multisystem disease including bone, treated with radiation therapy. Of 89 evaluable patients, 77% achieved a complete remission, 9% developed an infield recurrence, and 15.7% (14 of 89) experienced a progression outside the radiation field(s) (cited Olschewski et al. as reference 5 and level of evidence 3iiiDiv).
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