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Endocrine Abstracts (2015) 37 EP819 | DOI: 10.1530/endoabs.37.EP819

1Department of Endocrinology, Diabetes and Metabolism, Centro Hospitalar e Universitário de Coimbra, Coimbra, Portugal; 2Faculty of Medicine, University of Coimbra, Coimbra, Portugal; 3Department of Endocrinology, Centro Hospitalar do Baixo Vouga, Aveiro, Portugal.

Introduction: Langerhans cell histiocytosis (LCH) is a rare disorder characterized by proliferation/accumulation of cells phenotypically similar to skin Langerhans cells. Clinical presentation and aggressiveness are very heterogeneous, from benign to disseminated forms that cause significant morbi-mortality, particularly endocrine failures.

Case 1: A 15-year-old Caucasian woman presented with a 14-month history of headaches, secondary amenorrhea. She reported polyuria and polydipsia in the last 2 years. Secondary evaluation revealed hypogonadism hypogonadotrophic, central hypothyroidism, and a 1 cm suprasellar lesion. Surgical excision of the lesion was performed, though histologic examination was inconclusive, and pan-hypopituitarism developed after surgery. Radiotherapy was prescribed due to persistent disease. Two years later, biopsy of recurrent vulvar ulcers revealed HCL. Subsequent studies showed active disease in the vulvar region, thyroid and hypothalamic–pituitary axis, which remitted after several cycles of vulvar radiotherapy, and systemic chemotherapy with prednisolone, methotrexate, and vinblastine (PDN/MTX/VBA).

Case 2: A 37-year-old Caucasian man presented with a 6-month history of otalgy, otorrhea, weight loss, and skin brown maculo-papular rash. He reported polyuria and polydipsia in the previous five years. Secondary evaluation revealed pan-hypopituitarism and a suprasellar mass of 2 cm. Temporal bone biopsy revealed HCL. Patient was treated.

Case 3: A 60-year-old Caucasian woman presented with 6-month history of jaw pain and weight loss. Secondary evaluation revealed polyostotic HCL and the patient was treated with zoledronic acid. Three years later the patient complained of headaches, polyuria, and polydipsia. Complimentary evaluation revealed diabetes insipidus, hypogonadism hypogonadotrophic, central hypothyroidism, and pituitary stalk enlargement. All patients required permanent hormonal substitution for the failed HPA sectors.

Conclusion: All cases described had hypothalamo-pituitary axis involvement with pituitary failure. The dominant manifestation was diabetes insipidus. If not treated promptly LCH may have a considerable morbi-mortality with permanent endocrine failures, that require proper hormonal substitution. Relapses may occur and appropriate follow-up is recommended.

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