Endocrinol Metab.  2014 Sep;29(3):394-399. 10.3803/EnM.2014.29.3.394.

Adult Multisystem Langerhans Cell Histiocytosis Presenting with Central Diabetes Insipidus Successfully Treated with Chemotherapy

• Choi JE
• Lee HR
• Ohn JH
• Moon MK
• Park J
• Lee SJ
• Choi MG
• Yoo HJ
• Kim JH
• Hong EG

• KMID: 2169478
• DOI: http://doi.org/10.3803/EnM.2014.29.3.394

Abstract

We report the rare case of an adult who was diagnosed with recurrent multisystem Langerhans cell histiocytosis (LCH) involving the pituitary stalk and lung who present with central diabetes insipidus and was successfully treated with systemic steroids and chemotherapy. A 49-year-old man visited our hospital due to symptoms of polydipsia and polyuria that started 1 month prior. Two years prior to presentation, he underwent excision of right 6th and 7th rib lesions for the osteolytic lesion and chest pain, which were later confirmed to be LCH on pathology. After admission, the water deprivation test was done and the result indicated that he had central diabetes insipidus. Sella magnetic resonance imaging showed a mass on the pituitary stalk with loss of normal bright spot at the posterior lobe of the pituitary. Multiple patchy infiltrations were detected in both lung fields by computed tomography (CT). He was diagnosed with recurrent LCH and was subsequently treated with inhaled desmopressin, systemic steroids, vinblastine, and mercaptopurine. The pituitary mass disappeared after two months and both lungs were clear on chest CT after 11 months. Although clinical remission in multisystem LCH in adults is reportedly rare, our case of adult-onset multisystem LCH was treated successfully with systemic chemotherapy using prednisolone, vinblastine, and 6-mercaptopurine, which was well tolerated.

Keyword

Histiocytosis, Langerhans-cell; Drug therapy; Diabetes insipidus

MeSH Terms

6-Mercaptopurine
Adult*
Chest Pain
Deamino Arginine Vasopressin
Diabetes Insipidus
Diabetes Insipidus, Neurogenic*
Drug Therapy*
Histiocytosis, Langerhans-Cell*
Humans
Lung
Magnetic Resonance Imaging
Middle Aged
Pathology
Pituitary Gland
Polydipsia
Polyuria
Prednisolone
Ribs
Steroids
Tomography, X-Ray Computed
Vinblastine
Water Deprivation
6-Mercaptopurine
Deamino Arginine Vasopressin
Prednisolone
Steroids
Vinblastine

Figure

• 

  Fig. 1 The change in urine osmolality during the water deprivation test. Subcutaneous injection of five units of vasopressin markedly increased (more than 50% from baseline) urine osmolality, suggesting central complete diabetes insipidus.

• 

  Fig. 2 Sella magnetic resonance imaging (MRI) before and after chemotherapy. (A) At presentation, T1-weighted MRI of the pituitary gland revealed a solid mass in the infundibulum (6 mm) with loss of bright spot in posterior lobe (white arrow). (B) Two months after chemotherapy, there was no evidence of residual mass in the infundibulum on follow-up T1-weighted MRI.

• 

  Fig. 3 (A) Chest X-ray and (B) computed tomography (CT) of chest with contrast enhancement revealed multiple patchy infiltrations (black arrows) in both lung fields before chemotherapy. (C) follow-up chest X-ray and (D) CT of chest with contrast enhancement demonstrated complete remission of lung lesions.

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