Endocrine manifestations of Langerhans cell histiocytosis diagnosed in adults

Gallo, Marco; Martínez, María Paz; Abalovich, Marcos; Gutiérrez, Sílvia; Guitelman, Mirtha

doi:10.1007/s11102-010-0233-8

Cited by 38 publications

(8 citation statements)

References 22 publications

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“…ED was more prevalent in the CO forms than the AO forms. As previously reported, posterior pituitary dysfunction is a common endocrinological manifestation of LCH, and DI is usually the first endocrine manifestation (9,10,26,27,28,29,30). DI was the first endocrinological manifestation in 2.1% of CO patients in the French national LCH registry, but evaluations were not done systematically (31).…”

Section: Discussionmentioning

confidence: 92%

Endocrine manifestations in a cohort of 63 adulthood and childhood onset patients with Langerhans cell histiocytosis

Sagna

Courtillot

Drabo³

et al. 2019

European Journal of Endocrinology

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Objective Langerhans cell histiocytosis (LCH) is a rare inflammatory myeloid neoplasm which can infiltrate any organ or tissue. Endocrine involvement has mostly been described in case reports and small retrospective studies. We aimed to describe endocrine manifestations in a large cohort of adulthood onset (AO) and childhood onset (CO) patients with LCH. Design Single-center observational study conducted between January 2002 and December 2017 at Pitié-Salpêtrière University Hospital (Paris, France), a tertiary care hospital. Method Clinical, biological and morphological evaluations of pituitary, gonadal, adrenal and thyroid function evaluations performed in 63 consecutive patients with LCH (AO patients: 40, CO patients: 23). Fifty-eight patients underwent follow-up assessments. Results Complete pituitary evaluation was performed in 38/63 patients (60.3%); at least one anterior pituitary dysfunction (APD) was found in 63.2% of them. In this subgroup of patients, the most prevalent deficiencies were diabetes insipidus (DI) and GHD (55.3% each), followed by gonadotropin deficiency (34.2%) and thyrotropin deficiency (23.7%). In the subgroup of the 25 incompletely evaluated patients, we found DI in 44%, GHD in 50%, gonadotropin deficiency in 30.4% and thyrotropin deficiency in 16%. APD was more common in CO patients (P = 0.003) but was not systematically associated with DI regardless of the age of onset. Endocrine dysfunction was most often permanent; moreover, occurrence of new deficiencies has been described during follow-up. Conclusion The spectrum of endocrine disorders appears to be large in LCH (both in AO and CO patients) and should be evaluated carefully at diagnosis and during follow-up. APD was not always associated with DI.

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Section: Discussionmentioning

confidence: 92%

Endocrine manifestations in a cohort of 63 adulthood and childhood onset patients with Langerhans cell histiocytosis

Sagna

Courtillot

Drabo³

et al. 2019

European Journal of Endocrinology

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“…MS-LCH is reported in <30% of LCH cases (3). The hematopoietic system, liver and/or spleen are considered high-risk organs rich in histiocytes.…”

Section: Introductionmentioning

confidence: 99%

Langerhans cell histiocytosis misdiagnosed as liver cancer and pituitary tumor in an adult: A case report and brief review of the literature

Jiang

Chen

et al. 2014

Oncology Letters

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Langerhans cell histiocytosis (LCH) is a rare proliferative disorder in which pathological Langerhans cells accumulate in a variety of organs. LCH usually affects the bone, skin and lymph nodes of children; however, LCH occasionally affects vital organs, including the liver, spleen and pituitary gland. The present study reports a case of an adult LCH patient with marked liver damage, splenomegaly and pituitary damage treated using a new therapeutic strategy. This case was misdiagnosed as liver cancer and pituitary tumor on the basis of abdominal ultrasound, abdominal magnetic resonance imaging (MRI) and head MRI. The final diagnosis was established by identifying the proliferation of cluster of differentiation 1a-positive LCs in liver tissues. A new regimen of combined 12-week therapy of prednisolone/desmopressin/vincristine and 10 months of maintenance therapy of prednisolone/vinblastine/6-mercaptopurine improved symptoms, liver function and blood cell tests.

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“…Intra-cranial LCH lesions exist in two different forms: hypothalamic-pituitary axis change (HPA-LCH), being the most common, and less frequently neurodegenerative change (ND-LCH). In HPA involvement, central diabetes insipidus (CDI) arises in up to 50% of cases, 1 and anterior pituitary hormone deficiencies in 20%. 2 CDI is rare and characterised by polyuria, polydipsia and hypotonic urine due to antidiuretic hormone deficiency and, whilst LCH is a cause, it is by no means the most common cause.…”

Section: Discussionmentioning

confidence: 99%

An Unusual Intracranial Inflammatory Process of Unknown Origin

Badran¹,

Abdelsadq²,

Abbas³

et al. 2017

Neuro – Open Journal

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We present an unusual case of steroid responsive inflammatory condition, involving sellar suprasellar region with further ependymal lesions. This is complicated by previous surgery due to pituitary adenoma, not thought to related to inflammatory process. The patient responded well to steroids, but deteriorated due to development of hydrocephalus caused by obstruction due to adhesions. Despite extensive literature review and consideration of all known pathological conditions, it was concluded that the condition represented another inflammatory entity not yet fully characterised. The case also highlights that despite the steroid responsive nature of the condition, the ependymal involvement can result in progressive acute obstructive hydrocephalus with clinical deterioration. This case also suggests close follow-up and early imaging for early detection and treatment of this complication. CASE REPORTThis case report pertains to a 46-year-old male Caucasian who had endoscopic treatment for a pituitary adenona three years previously. He required replacement hydrocortisone and testosterone. Prior to the surgery, he was not known to have any other neurological issues.A year after surgery following minor trauma, he underwent a computed tomography (CT) scan of the head. This confirmed no residual pituitary tumour (Figure 1). He was somewhat non-compliant with endocrine follow-up. Surveillance magnetic resonance imaging (MRI) imaging 2 years following initial surgery demonstrated post-up change with some enhancing tissue scaling the pituitary stalk and hypothalamic region; this was not clearly seen on the previous CT scan, possibly due to different modalities and absence of contrast imaging. Two months later, he developed polydipsia, fatigue and a partial left-sided homonymous visual field deficit. There was progressive confusion with fluent dysphasia and inattention. He was pyrexial. Gaze evoked nystagmus was present to the right. The rest of his examination was unremarkable. MRI brain demonstrated significant progression of the abnormalities seen on the earlier scan with increased enhancing tissue involving the pituitary stalk, hypothalamic area and floor of the 3 rd ventricle, also including the optic chiasm (Figure 2). Associated signal

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Endocrine manifestations of Langerhans cell histiocytosis diagnosed in adults

Cited by 38 publications

References 22 publications

Endocrine manifestations in a cohort of 63 adulthood and childhood onset patients with Langerhans cell histiocytosis

Endocrine manifestations in a cohort of 63 adulthood and childhood onset patients with Langerhans cell histiocytosis

Langerhans cell histiocytosis misdiagnosed as liver cancer and pituitary tumor in an adult: A case report and brief review of the literature

An Unusual Intracranial Inflammatory Process of Unknown Origin

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