Langerhans' cell histiocytosis involving the pituitary, thyroid, lung, and liver.
OBJECTIVE
To report the development, in an adult patient, of Langerhans' cell histiocytosis (LCH) involving the thyroid and most probably the pituitary gland, lungs, and liver.


METHODS
We present a case report of a 29-year-old woman who requested a medical consultation because of polyuria and was found to have pituitary dysfunction. We describe the subsequent follow-up, which revealed progressive liver disease that necessitated transplantation and also the presence of a goiter, and discuss the unpredictable pathologic features of LCH.


RESULTS
After the diagnosis of central diabetes insipidus and partial hypopituitarism, the patient was diagnosed 2 years later with sclerosing cholangitis. The hepatic involvement was progressive, and she required transplantation and immunosuppression. Three months before liver transplantation, a goiter was discovered. Fine-needle aspiration of the thyroid revealed infiltration by LCH. The goiter decreased in size after chemotherapy with vinblastine and prednisone. Computed tomography of the chest showed bilateral thin-walled cysts, consistent with eosinophilic granulomas.


CONCLUSIONS
In patients with central diabetes insipidus and pituitary stalk thickening on imaging studies, LCH should be considered in the differential diagnosis. Other hormonal deficiencies may be present initially or may evolve after many years. Thus, continual surveillance is necessary. In addition, an ongoing potential exists for involvement of other organ systems such as the thyroid, liver, and lungs. We suggest consideration of LCH as a possible cause of a goiter in such patients. In our patient, it remains to be seen what effect the immunosuppressive therapy for the liver transplant has on the LCH disease process.
Division of Endocrinology, Department of Pathology and Laboratory Medicine, Indiana University School of Medicine, Indianapolis, Indiana 46202, USA.
Sujatha
S
Sampathkumar
Cheryl
C
Younger
Harvey
H
Cramer
Naga
N
Chalasani
Paul A
PA
Skierczynski
D000328:Adult; D001707:Biopsy, Needle; D003919:Diabetes Insipidus / Q000209:etiology; D003937:Diagnosis, Differential; D005260:Female; D006042:Goiter / Q000209:etiology; D006646:Histiocytosis, Langerhans-Cell / Q000150:complications* / Q000473:pathology / Q000628:therapy; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008107:Liver Diseases / Q000209:etiology* / Q000473:pathology / Q000601:surgery; D016031:Liver Transplantation; D008171:Lung Diseases / Q000209:etiology* / Q000473:pathology; D008279:Magnetic Resonance Imaging; D008854:Microscopy, Electron; D010900:Pituitary Diseases / Q000209:etiology* / Q000473:pathology; D013959:Thyroid Diseases / Q000209:etiology* / Q000473:pathology
D007166:Immunosuppressive Agents