Year: 2010
Type: article
Study design: Case Report
Abstract: A six-year-old boy was referred to a paediatric hematologist/oncologist with recurrent, painful skull masses. Skeletal survey and computed tomography revealed lytic lesions in his calvarium (Figure 1) and a silent lesion in his right humerus. Positron emission tomography-computed tomography scan confirmed involvement of these sites. A biopsy of the skull mass showed infiltrates of pathogenic Langerhans cells, staining positive for CD1a, Langerin and S100, and containing Birbeck granules on electron microscopy. Of note, 14 months earlier, the boy had a soft tissue mass removed by surgical curettage from the lateral wall of his left orbit but no adjuvant therapy. The pathology of both lesions was consistent with Langerhans cell histiocytosis (LCH). ... The boy's treatment at this time included vinblastine and prednisone for a year, with regression of all lesions. Three years later, another skull mass appeared and surgical curettage again showed pathogenic Langerhans cells. Meanwhile, he had developed excessive thirst and polyuria. A water deprivation test confirmed diabetes insipidus. A brain magnetic resonance imaging scan showed enhancement and thickening of his infundibular pituitary stalk, suggestive of LCH involvement. In view of this new development, a paediatric endocrinologist became involved in managing the diabetes insipidus and is closely observing any further pituitary complications. Because LCH has the potential to recur despite successful therapies, sometimes with significant sequelae, close supervision by a multidisciplinary care team is essential (1).
Source: Paediatrics & Child Health
DOI: 10.1093/pch/15.2.69
Authors: Bruce Crooks, Danielle Grenier
Language: English
Subfield: Hematology
Field: Medicine
Domain: Health Sciences
Open Access status: bronze
OpenAlex ID: W12950544