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43 - Langerhans Cell Histiocytosis

from Section 2 - Sellar, Perisellar and Midline Lesions

Published online by Cambridge University Press:  05 August 2013

Zoran Rumboldt
Affiliation:
Medical University of South Carolina
Andrea Rossi
Affiliation:
Children’s Research Hospital, Genoa, Italy
Zoran Rumboldt
Affiliation:
Medical University of South Carolina
Mauricio Castillo
Affiliation:
University of North Carolina, Chapel Hill
Benjamin Huang
Affiliation:
University of North Carolina, Chapel Hill
Andrea Rossi
Affiliation:
G. Gaslini Children's Research Hospital
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Summary

Specific Imaging Findings

Cranial MRI findings in Langerhans cell histiocytosis (LCH) include: (1) lesions of the facial bones, skull base, and calvarium; (2) intracranial extra-axial lesions (hypothalamic–pituitary region, meninges, circumventricular organs); (3) intra-axial changes; and (4) cerebral atrophy. Intracranial findings are typically associated with central diabetes insipidus (DI) and characterized by lack of high signal intensity of the posterior pituitary gland on T1-weighted images (from loss of ADH storage granules) and thickened enhancing pituitary stalk, which measures over 3 and even 7 mm in diameter. However, the posterior pituitary bright spot may persist in DI patients; on the other hand, other disease processes may present in a similar fashion. Hypothalamic (along the floor of the third ventricle) involvement with contrast enhancement can be seen, while pituitary and optic chiasm infiltration are found in some cases. In a minority of patients the infundibulum may be very thin and thread-like, under 1 mm in diameter; it can even be normal on MRI. The pineal gland may also show mild enlargement and contrast enhancement. Progressive reduction in size of the anterior pituitary on MRI is associated with a higher risk of additional endocrine defects. Repeated MRI studies in DI patients are of limited value for assessing a response to therapy, but are important for monitoring bone lesions and possible parenchymal CNS disease.

Pertinent Clinical Information

LCH mainly affects children between 2 and 5 years of age with widely varying clinical manifestations and it may also occur in adults. CNS is affected in around 16% of patients, and DI is the most common manifestation (in 25%), followed by GH deficiency (in 10%) – usually diagnosed years after DI. However, a number of other diseases can cause DI and 30–50% of cases are considered idiopathic. LCH is usually self-limited in the absence of organ dysfunction. Brain involvement appears to be the single most important factor in determining quality of life.

Type
Chapter
Information
Brain Imaging with MRI and CT
An Image Pattern Approach
, pp. 89 - 90
Publisher: Cambridge University Press
Print publication year: 2012

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References

1. Prayer, D, Grois, N, Prosch, H, et al.MR imaging presentation of intracranial disease associated with Langerhans cell histiocytosis. AJNR 2004;25:880–91.Google ScholarPubMed
2. Makras, P, Samara, C, Antoniou, M, et al.Evolving radiological features of hypothalamo-pituitary lesions in adult patients with Langerhans cell histiocytosis (LCH). Neuroradiology 2006;48:37–44.CrossRefGoogle Scholar
3. D'Ambrosio, N, Soohoo, S, Warshall, C, et al.Craniofacial and intracranial manifestations of Langerhans cell histiocytosis: report of findings in 100 patients. AJR 2008;191:589–97.CrossRefGoogle ScholarPubMed
4. Marchand, I, Barkaoui, MA, Garel, C, et al.Central diabetes insipidus as the inaugural manifestation of Langerhans cell histiocytosis: natural history and medical evaluation of 26 children and adolescents. J Clin Endocrinol Metab 2011;96:E1352–60.CrossRefGoogle ScholarPubMed
5. Maghnie, M, Cosi, G, Genovese, E, et al.Central diabetes insipidus in children and young adults. N Engl J Med 2000;343:998–1007.CrossRefGoogle ScholarPubMed

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