Langerhans cell histiocytosis with spinal, pulmonary and pituitary involvement: What about ACTH deficiency without diabetes insipidus? A propos of a case.

Jake E Ryan, Vijayakumar Ganesh, Afroditi Karathanasi, Michele Moschetta, Matin Sheriff, John B Schofield, Stergios Boussios

Journal of B.U.ON. : official journal of the Balkan Union of Oncology 2020 Mar-Apr

filter terms:

Langerhans cell histiocytosis (LCH) is disease process characterized by clonal proliferation of CD1a+ dendritic cells within an inflammatory infiltrate of hematopoietic derived cells. LCH can manifest with a broad spectrum of symptoms and can involve single organs or have a multisystem distribution. Central nervous system (CNS) involvement of LCH can manifest as granulomatous parenchymal or pituitary mass lesions. Focal, space-occupying lesions, such as masses in the meninges, choroid plexus, and brain parenchyma may contain CD1a+ LCH cells, lymphocytes, and macrophages with histology similar to that of extracranial lesions. Here, we describe a rare case of multisystem LCH in an adult patient presenting with spinal lesions and isolated adrenocorticotropic (ACTH) deficiency without diabetes insipidus (DI). In addition, we review the literature summarizing the few reports of hypopituitarism in LCH in the absence of DI.

Citation

Jake E Ryan, Vijayakumar Ganesh, Afroditi Karathanasi, Michele Moschetta, Matin Sheriff, John B Schofield, Stergios Boussios. Langerhans cell histiocytosis with spinal, pulmonary and pituitary involvement: What about ACTH deficiency without diabetes insipidus? A propos of a case. Journal of B.U.ON. : official journal of the Balkan Union of Oncology. 2020 Mar-Apr;25(2):612-617