Craniospinal Langerhans cell histiocytosis in children: 30 years' experience at a single institution

Abstract

<jats:sec> <jats:title>Object</jats:title> <jats:p>The goal of this study was to review a large series of patients with Langerhans cell histiocytosis (LCH) who had craniospinal lesions to assess the long-term course, outcome, and efficacy of treatment of the disease.</jats:p></jats:sec> <jats:sec> <jats:title>Methods</jats:title> <jats:p>Forty-four patients with LCH who presented to a single pediatric neurosurgical department between 1976 and 2006 were retrospectively reviewed.</jats:p></jats:sec> <jats:sec> <jats:title>Results</jats:title> <jats:p>This series included 29 boys and 15 girls, ranging in age from 2 months to 13 years, with a mean follow-up duration of 4.5 years. Twenty-seven patients (61%) had unifocal bone lesions, 12 (27%) had multifocal bone disease, 2 (5%) had solitary hypothalamic–pituitary axis lesions, and 3 (7%) had multiple organ involvement at presentation. Five (19%) of the 27 patients with unifocal bone disease and 4 (33%) of the 12 patients with multifocal bone disease had delayed development of new bone lesions during the follow-up period. The time to development of new bone lesions ranged from 1 month to 1 year. Two of the 3 patients with multiple-organ LCH died. Patient age ≤ 2 years at the time of initial presentation was a risk factor for both initial multifocality and eventual dissemination. In all patients with initial multifocal bone involvement or later dissemination of unifocal bone disease, LCH was controlled by chemotherapy, except for 2 who were treated by surgery alone. Three patients had histological evidence of spontaneous resolution of their lesions.</jats:p></jats:sec> <jats:sec> <jats:title>Conclusions</jats:title> <jats:p>Patients with unifocal LCH can be effectively treated with surgery alone. Very young patients are more likely to have multifocal disease and disseminations, and will usually require chemotherapy to control their disease. Spontaneously regressing lesions need not be resected; however, a biopsy procedure can be performed for diagnostic purposes.</jats:p></jats:sec>

Langerhans cell histiocytosis of bone in children: a clinicopathologic study of 108 cases

“…[6] The incidence of LCH in children is 1 to 5 children per million per year. [7] LCH almost affects any organ in the form of unifocal or multifocal lesions. [3] In children, bone is the most common single organ and the skull is the most affected site.…”

Diagnosis, Pathophysiology, and Management of Mood Disorders in Pregnant and Postpartum Women

“…In the multicenter Depression Network Study, investigators diagnosed postpartum major depressive disorder in 42% of 31 women with a sister who had experienced major depressive disorder after delivery compared with only 15% of 59 women whose sisters had not experienced postpartum major depressive disorder. 60 A report from the Genetics of Recurrent Early-Onset major depressive disorder study revealed that having a sibling with major depressive disorder during pregnancy or after delivery increased the odds of a similar episode in the other sibling by 2.28 (95% CI 1.13-4.58); a sibling with postpartum major depressive disorder increased the odds of having postpartum major depressive disorder by 4.96 (95% CI 1.51-10.42). 61…”

Langerhans cell histiocytosis with multifocal bone lesions: comparative clinical features between single and multi-systems

“…Another report describing how JLSG-02 compares to JLSG-96 in terms of the therapeutic results of the entire cohort (and with longer follow-up times) is currently being prepared as a separate paper. Our recent literature survey has shown that of the studies discussing the management of bone LCH, most mix patients with single bone (monostotic) disease with patients with multiple bone (polyostotic) disease [10][11][12][13]; many also do not separate the pediatric patients from the adult patients [4,5]. Thus, the bone involvement of pediatric cases with SMFBand MSB-type LCH has not been analyzed systematically.…”