SJR uses a similar algorithm as the Google page rank; it provides a quantitative and qualitative measure of the journal’s impact. Moderately elevated serum prolactin levels have been reported in adults with LCH, 10 being attributed to infundibular infiltration unrelated to gonadotropin deficiency. The two patients with ECD were treated with systemic corticosteroids and immunomodulators as described below. Services on Demand Article. In , the common pathology of these three conditions was recognised and the term histiocytosis X was introduced to name them collectively. During follow-up, the sellar and parasellar masses increased in size, requiring repeat surgery and the subsequent administration of fractionated stereotactic RT.

Pediatr Blood Cancer, 48 , pp. Thyroid ophthalmopathy was suspected, and steroid treatment was administered for two years, achieving a remission of the clinical signs. Histological examination of a biopsy from the lesion was consistent with ECD. Another patient showed hypothalamic involvement with polyphagia, hypodipsia, hyperthermia, and recent memory impairment. Pituitary MRI revealed a sellar mass with suprasellar extension Fig. Radiology, , pp.

hypopituitarism in langerhans cell histiocytosis seven cases and literature review

As regards bone involvement, lesions were found in the proximal and distal thirds of the tibia and distal femur in both legs, and the intertrochanteric region of the right femur and humerus, ulna, and radius in both arms.

As regards RT, it is used for hypopituitarsim lesions with compression symptoms, but response is usually less marked than in LCH.

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

Laboratory tests showed subclinical hyperthyroidism. The three patients with normal pituitary MRI at diagnosis showed no radiographic changes during follow-up. When bone involvement exists, if the symptoms require active treatment, low-dose RT, intralesional glucocorticoids or excisional biopsy may be considered. Previous article Next article.


Sellar and suprasellar mass hsitiocytosis and 1. Change over time in hormone deficiencies and pituitary MRI at diagnosis. A rib resection was performed and once again histological examination confirmed LCH. Report from the International Registry of the Histiocyte Society. Mod Pathol, 14pp.

hypopituitarism in langerhans cell histiocytosis seven cases and literature review

Lesions are either solitary or few, and mostly involve the bones. 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.

Diagnosis is based on the presence ccases histiocytes with an absence of Birbeck granules, positive CD68, and negative CD1a.

Other uncommonly reported nonpituitary endocrine manifestations include primary thyroid involvement due to the infiltration of Langerhans cells, 19 primary hypoparathyroidism in the setting of diffuse thyroid and parathyroid infiltration, 19 ovarian involvement in cases of disseminated disease, 20 adrenal gland infiltration, and direct pancreas involvement in the setting of systemic disease.

Results Median age at diagnosis was 38 years range, 3— Its clinical heterogeneity is illustrated by the fact that it was historically described as three different disease entities, based on disease extent and severity.

The boy was referred to a paediatric oncologist for further management. No patient died during follow-up. Orphanet J Rare Dis, 8pp.

Pituitary TD 10 Gy 0. Regular monitoring of these patients is recommended. Langerhans cell histiocytosis LCH is a disease of unknown aetiology. Discussion LCH is a disease of unknown aetiology that is characterised by extreme clinical heterogeneity. Progression of skin involvement without oral corticosteroid treatment. Localized litterature of LCH usually have a slow course and a good response to treatment, 4 while systemic forms involving liver, spleen, bone or lung, young adults, or poor response to chemotherapy are poor prognostic factors.


hypopituitarism in langerhans cell histiocytosis seven cases and literature review

Thyrotropin deficiency was diagnosed in that same year. Hematol Oncol Clin North Am, 12pp.

For the interpretation of IGF-I, age- and sex-adjusted reference values were used. Today most investigators consider LCH sevven result from an aberrant immune response, but the stimulus for clonal Langerhans cell proliferation remains unknown. Treatment of patients with hypothalamic—pituitary lesions as adult-onset Langerhans cell histiocytosis.

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

Once the diagnosis has been confirmed, all cases should be referred to a paediatric oncologist for risk stratification and further management. Services on Demand Article. Pituitary MRI only showed an absence of hypersignal of the neurohypophysis.

Histological examination confirmed the diagnosis of Langerhans cell histiocytosis LCH. LCH is primarily a paediatric disease: