Absence of hyperintensity of neurohypophysis. Tumour resection and a bone transplant were done. An accurate histological diagnosis is needed. Introduction Histiocytoses are a heterogeneous group of diseases characterized by the proliferation of cells from the mononuclear phagocyte system. Diagnosis of LCH was based on verification of the presence of Birbeck granules by electron microscopy and antigen CD1a or langerin CD on the cell surface. Langerhans cell histiocytosis infiltration into pancreas and kidney.

The variables analyzed included sex, age at diagnosis, disease duration, first symptom, form of disease localized or systemic , type of endocrine and non-endocrine involvement, treatment, and radiographic findings at symptom start and during the course of the disease.. Lancet, 1 , pp. DI is the most common involvement at diagnosis. Br J Cancer, Suppl. After diagnosis, five patients developed one or more anterior deficiencies, with a median of 7.

As regards imaging tests, pituitary MRI after the administration of gadolinium is the procedure of choice for assessing the HPA region.

Hypopituitarism in langerhans cell histiocytosis: seven cases and literature review.

Change over time in hormone deficiencies and pituitary MRI at diagnosis. Langerhans cell histiocytosis LCH: J Endocrinol Invest, 24pp.

Two of the seven patients with LCH had localized involvement, and five systemic diseases Table 1. The most common radiographic finding is the loss of the hyperintense signal of the neurohypophysis, present in almost histiocyosis patients with DI. Instructions for authors Submit an article Ethics in publishing. Inthe common pathology of these three conditions was recognised and the term histiocytosis X was introduced to name them collectively.

Hypopituitarism in langerhans cell histiocytosis: seven cases and literature review.

Drugs that inhibit BRAF serine—threonine kinase, such as vemurafenib 27,28 and imatinib, have shown promising results in small patient series and clinical trials in terms of clinical and radiographic disease improvement, as occurred in one patient from our series. Base of skull TD 20 Gy 1.


Literaure a few cases of anterior pituitary deficiency without DI have been reported.

The state of the disease was now defined as progressive or refractory. To improve our services and products, we use “cookies” own or third parties authorized to show advertising related to client preferences through the analyses of navigation customer behavior. Imaging 6 weeks later confirmed that the patient now had non-active disease i.

Skin-limited Langerhans cell histiocytosis in children. In adults, mean age at diagnosis is 33 years. All patients with LCH must be referred to a paediatric oncologist for proper risk stratification, an appropriate treatment plan and long-term follow-up.

Si continua navegando, consideramos que acepta su uso. Progression of skin involvement without oral corticosteroid treatment.

A rib resection was performed and once again histological examination confirmed LCH.

Inthe patient attended our hospital complaining of bone pain in the lower limbs over the previous three years and clinical symptoms of polyuria and polydipsia for 10 years. After confirmation of the BRAFVE mutation, the patient started treatment with vemurafenib, which has resulted in marked clinical and laboratory improvement.

Cancer Invest ;27 5: Since the pathogenesis of LCH is still unknown, the approach to treatment is empirical and not rational. A review of the clinical and radiological findings. Discussion LCH is a disease of unknown aetiology that is characterised by extreme clinical heterogeneity.


Langerhans cell histiocytosis: A case presentation and literature review

With a malignant disease, the goal would be to eradicate the malignant clone. It is a rare disease with an incidence of 3 – 5 per million children aged 1 – 14 years. Histiocytosis follows an unpredictable course, sometimes consisting of bouts of the disease.

hypopituitarism in langerhans cell histiocytosis seven cases and literature review

The authors state that they have no conflicts of interest. During follow-up, the sellar and parasellar masses hypoppituitarism in size, requiring repeat surgery and the subsequent administration of fractionated stereotactic RT. Material and methods A retrospective, observational study was conducted to analyze the clinical data from nine patients 5 women and 4 men with a mean age 52 years range, 21—74 with a histological diagnosis of histiocytosis and HPA involvement 7 with LCH and 2 with ECD.

LCH should also be considered in the case of idiopathic diabetes insipidus, or atypical or extensive skin lesions in infants or young children. Report from the International Registry of the Histiocyte Society.

hypopituitarism in langerhans cell histiocytosis seven cases and literature review

Continuing navigation will be considered as acceptance of this use. Liver involvement may lead to hypo-albuminaemia with ascites, hyperbilirubinaemia and clotting factor deficiencies.