In , the 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. The radiographic appearance depends on the phase of the disease. Histological examination confirmed the diagnosis of Langerhans cell histiocytosis LCH. Lesion in infundibular recess of third ventricle. Sella-suprasellar mass in a patient with Erdheim—Chester disease. No patient died during follow-up.

Main clinical characteristics of the nine patients of the series.. Histological diagnosis is mandatory. Pituitary MRI revealed a sellar mass with suprasellar extension Fig. Pituitary adenoma associated with LCH is characterised histologically by an uncontrolled monoclonal proliferation of abnormal Langerhans cells:

Blood,pp. Treatment was started with glucocorticoids and azathioprine, with no response. After diagnosis, five patients developed one or more anterior deficiencies, with a median of 7.

The initial intensive phase of chemotherapy consisted of daily prednisone and weekly vinblastine for a total of 6 weeks. Int J Hematol, 94pp. At this follow-up visit the patient was clinically well, but the skeletal isotope scan revealed areas of abnormally increased uptake in the anterior aspects of the 7th and 8th ribs on the right side. In the extension study, a CT scan of the chest and abdomen revealed retroperitoneal, mediastinal, and adrenal involvement, and a bone scan showed the bilateral involvement of tibia, distal fibula, metatarsus, and tarsus.

Histiocytosis is a rare disease un unknown etiology with a very heterogeneous clinical presentation. He successfully completed the 52 weeks of the continuation phase, and recent imaging confirmed that he still has non-active disease.


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

Hypothalamo-pituitary abnormalities in adult patients with Langerhans cell histiocytosis: English pdf Article in xml format Article references How to cite this article Automatic translation. A review of the clinical and langergans findings.

hypopituitarism in langerhans cell histiocytosis seven cases and literature review

Discussion LCH is a disease of unknown aetiology that is characterised by extreme clinical heterogeneity. N Engl J Med,pp. PDN 20 mg Surgery is reserved for resectable cranial hypopituitarim or for severe orbital lesions.

It is the best journal to keep up to date with endocrine pathophysiology both in the histiocytodis and in the research field. Histological examination of a bone biopsy from the left lower limb was consistent with the same condition.

A rib resection was performed and once again histological examination confirmed LCH. A high index of suspicion is key to early diagnosis.

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

Langerhans cell histiocytosis LCH is a disease of unknown aetiology. As regards thyrotropin deficiency, in some series it is always associated with panhypopituitarism, 10 while in other series it is the third pituitary deficiency in 3.

Because of the lower frequency of ECD, these two cases in our series are described. Only a few cases of anterior pituitary deficiency without DI have been reported. In patients with multiple bone lesions or multisystem non-risk hypopituitzrism involvement, even a short treatment hypopitujtarism only a single agent e. A control pituitary MRI showed an enlargement of the sellar and parasellar masses and the occurrence of tentorial lesions, which required the administration of RT again and the reintroduction of steroid treatment.


Management of adult patients with Langerhans cell histiocytosis: Lesion in infundibular histiocytosos of third ventricle. Table 3 shows the systemic involvement and treatment administered in our series. 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.

hypopituitarism in langerhans cell histiocytosis seven cases and literature review

Cancer Invest ;27 5: Pulmonary involvement in Erdheim—Chester disease: Inthe common pathology of these three conditions was recognised and the term histiocytosis X was introduced to name them collectively.

The purpose of this article was to analyze the frequency and progression of pituitary hormone deficiencies in a series of nine patients with LCH and ECD loterature HPA involvement. Subtotal resection was performed, and the histological examination was consistent with ECD.