scholarly journals Serial assessments of anterior pituitary hormones in a case of mixed histiocytosis representing Langerhans cell histiocytosis overlapping with Erdheim–Chester disease

2021 ◽  
Vol 2021 ◽  
Author(s):  
Kei Ito ◽  
Jun Ito ◽  
Yuki Yamamoto ◽  
Rikako Nakajima ◽  
Masanao Fujii ◽  
...  
Keyword(s):  
Anterior Pituitary ◽  
Langerhans Cell Histiocytosis ◽  
Pituitary Hormones ◽  
Langerhans Cell ◽  
Erdheim Chester Disease ◽  
Erdheim Chester ◽  
Serial Assessments ◽  
Chester Disease ◽  
Posterior Pituitary Lobe

Summary A 61-year-old man developed central diabetes insipidus caused by mixed histiocytosis (MH) representing Langerhans cell histiocytosis overlapping with Erdheim–Chester disease. Bone, skin, vascular, and retroperitoneal involvements were also observed. Dynamic hormonal testing showed normal responses for anterior pituitary hormones, except for impaired secretion of growth hormone (GH). MRI of the brain showed thickening of the pituitary stalk with slightly reduced signal hyperintensity in the posterior pituitary lobe on T1-weighted imaging. During 2 years of follow-up without radical treatment for MH, imaging studies suggested extension of vascular and retroperitoneal involvements. In contrast, brain MRI did not show any particular interval changes, except for the disappearance of hyperintense signalling in the posterior pituitary lobe. Moreover, no other anterior pituitary dysfunctions beyond GH deficiency emerged during the 2 years of follow-up. The natural history of MH in this case is described, focusing on serial assessments of pituitary functions using dynamic tests. Learning points Erdheim–Chester disease and Langerhans cell histiocytosis overlapping as MH was described, focusing on pituitary functions. MH caused both GH deficiency and central diabetes insipidus. Despite a lack of radical therapy for MH, no other anterior pituitary dysfunctions emerged for 2 years. Radiological images showed no particular interval changes in pituitary stalk lesions, while vascular and retroperitoneal involvements extended.

Erdheim?Chester disease of the breast associated with Langerhans-cell histiocytosis of the hard palate

2004 ◽  
Vol 445 (4) ◽  
pp. 405-409 ◽  
Author(s):  
V. P. Andrade ◽  
C. C. V. Nemer ◽  
A. N. L. Prezotti ◽  
W. S. L. Goulart
Keyword(s):  
Langerhans Cell Histiocytosis ◽  
Hard Palate ◽  
Langerhans Cell ◽  
Erdheim Chester Disease ◽  
Erdheim Chester ◽  
Chester Disease

scholarly journals Dramatic efficacy of dabrafenib in Erdheim-Chester disease (ECD): a pediatric patient with multiple large intracranial ECD lesions hidden by refractory Langerhans cell histiocytosis

2019 ◽  
Vol 23 (1) ◽  
pp. 48-53 ◽  
Author(s):  
Xiaolei Hao ◽  
Ruie Feng ◽  
Yalan Bi ◽  
Yuhan Liu ◽  
Chunde Li ◽  
...  
Keyword(s):  
Pediatric Patient ◽  
Langerhans Cell Histiocytosis ◽  
Langerhans Cell ◽  
Neurological Involvement ◽  
Treatment Processes ◽  
Erdheim Chester Disease ◽  
Intracranial Lesions ◽  
The Central Nervous System ◽  
Erdheim Chester ◽  
Chester Disease

Erdheim-Chester disease (ECD) is a rare non–Langerhans cell form of histiocytosis that can affect the central nervous system. ECD predominantly affects adults, and only a few pediatric cases have been reported. The co-occurrence of ECD and Langerhans cell histiocytosis (LCH) is exceedingly rare. An 11-year-old boy, who was diagnosed with LCH 7 years previously, presented with multiple giant intracranial lesions. At the time of his initial diagnosis, only one intracranial lesion was observed, and it began to enlarge. Currently, up to 7 intracranial lesions can be observed in this patient. However, the diagnosis of ECD was not confirmed until this most recent open resection. The BRAF V600E mutation was detected in both LCH and ECD lesions. Dabrafenib therapy exhibited dramatic efficacy in this pediatric patient. This case represents the first successful application of dabrafenib in a pediatric patient with intracranial ECD lesions as well as mixed ECD and LCH. In this article, the authors describe the intricate diagnosis and treatment processes in this patient. Recent studies regarding treatment with BRAF inhibitors for neurological involvement in mixed ECD and LCH are also reviewed.

Mixed Langerhans Cell Histiocytosis and Erdheim-Chester Disease in a Girl

2020 ◽  
Vol Publish Ahead of Print ◽  
Author(s):  
Suheyla Ocak ◽  
Zuhal Bayramoglu ◽  
Deniz Tugcu ◽  
Serap Karaman ◽  
Aysegul Unuvar ◽  
...  
Keyword(s):  
Langerhans Cell Histiocytosis ◽  
Langerhans Cell ◽  
Erdheim Chester Disease ◽  
Erdheim Chester ◽  
Chester Disease

18F-Fluoride PET/CT Aspect of an Unusual Case of Erdheim-Chester Disease With Histologic Features of Langerhans Cell Histiocytosis

2013 ◽  
Vol 38 (7) ◽  
pp. 541-542 ◽  
Author(s):  
Cécile Caoduro ◽  
Constantin Marius Ungureanu ◽  
Boris Rudenko ◽  
Orland Angoue ◽  
Oleg Blagosklonov ◽  
...  
Keyword(s):  
Langerhans Cell Histiocytosis ◽  
Unusual Case ◽  
Langerhans Cell ◽  
Erdheim Chester Disease ◽  
Pet Ct ◽  
Erdheim Chester ◽  
18F Fluoride ◽  
Chester Disease

Erdheim—Chester Disease

2003 ◽  
Vol 7 (2) ◽  
pp. 129-132
Author(s):  
Susan E. Lenahan ◽  
Klaus F. Helm ◽  
Kenneth D. Hopper
Keyword(s):  
Differential Diagnosis ◽  
Case Report ◽  
Langerhans Cell Histiocytosis ◽  
Langerhans Cell ◽  
Morbidity And Mortality ◽  
Significant Morbidity ◽  
Erdheim Chester Disease ◽  
Pathologic Findings ◽  
Erdheim Chester ◽  
Chester Disease

Background: Erdheim–Chester disease is a rare non-Langerhans' cell histiocytosis. Objective: This case report is presented to familiarize clinicians with Erdheim–Chester disease and its differential diagnosis. Results and Conclusion: Erdheim–Chester disease presents with unique clinical and pathologic findings. Its xanthoma-like lesions can cause significant morbidity and mortality.

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