Langerhans histiocytosis and panhypopituitarism in adults. Case report

Langerhans-cells Histiocytosis (LCH) is a rare disease characterized by the clonal proliferation of immature dendritic cells that locally or diffusely infiltrate different systems, mainly affecting children. We present the case of a 38-year-old man with a long-standing history of multiple tooth loss...

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Bibliographic Details
Published in:Revista medíca de Chile 2023-05, Vol.151 (5), p.659
Main Authors: Carvajal Moya, Cindy, Díaz Aracena, Jesús, Zapata Pizarro, Antonio, Lisott de Moro, Elena, Mancilla Sierpe, Edgardo, Carrasco Hartard, Cecilia
Format: Article
Language:Spanish
Subjects:
Adult
Histiocytosis, Langerhans-Cell - complications
Histiocytosis, Langerhans-Cell - diagnostic imaging
Histiocytosis, Langerhans-Cell - pathology
Humans
Hypopituitarism - etiology
Magnetic Resonance Imaging
Male
Tomography, X-Ray Computed
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Summary:Langerhans-cells Histiocytosis (LCH) is a rare disease characterized by the clonal proliferation of immature dendritic cells that locally or diffusely infiltrate different systems, mainly affecting children. We present the case of a 38-year-old man with a long-standing history of multiple tooth loss without association with trauma, smoking, or poor dental hygiene. Skull computed tomography (CT) showed multiple lytic jaw lesions. Jaw biopsy and immunohistochemical results were compatible with the finding of LCH. The patient evolved with polyuria, polydipsia, and nocturia, confirming the presence of diabetes insipidus and panhypopituitarism through hormonal studies. Magnetic resonance imaging (MRI) of the brain shows a hypothalamic neoplasm with infundibular involvement and an image suggestive of a pituitary granuloma. The treatment consisted of maxillobuccofacial defocusing, hormonal supplementation, chemotherapy, and radiotherapy with favorable evolution.
ISSN:0717-6163
0717-6163
DOI:10.4067/s0034-98872023000500659