A Case of Langerhans Cell Histiocytosis with Hypothalamic Mass.
- Author:
Woe Sook YOON
1
;
Ji Hae KANG
;
Yoo Rah HONG
;
Jae Sun PARK
;
Bong Kwon CHUN
;
Mi Hee JUNG
;
Chang Woo MOON
Author Information
1. Department of Pediatrics, College of Medicine, Kosin University, Busan, Korea. pjs@ns.kosinmed.or.kr
- Publication Type:Case Report
- Keywords:
Langerhans cell histiocytosis;
Hypothalamic mass;
Radiation
- MeSH:
Cyclophosphamide;
Diabetes Insipidus;
Drug Therapy;
Etoposide;
Female;
Histiocytosis, Langerhans-Cell*;
Humans;
Hypothalamus;
Infant;
Magnetic Resonance Imaging;
Obesity;
Polydipsia;
Prednisone;
Recurrence;
Sepsis;
Skin;
Vincristine;
Weight Gain
- From:Korean Journal of Pediatric Hematology-Oncology
2005;12(1):131-137
- CountryRepublic of Korea
- Language:Korean
-
Abstract:
A 14-month-old girl presented with petechial skin lesions and polydipsia was diagnosed as Langerhans cell histiocytosis (LCH) and responded fairly well to multiple chemotherapies using vincristine, cyclophosphamide, and prednisone. 3 years later, relapses were more common with short periods of remissions in spite of using more intensive therapy with vinblatine and etoposide. At age of 4.5, sudden weight gain and abnormal behavior led to MRI study and revealed an hypothalamic mass. Radiation of 1, 800 cGy was given to the mass and followed by a 75% decrease in measuring and remission of the obesity. Although, there was no evidence of tumor progression in the hypothalamus, she died of sepsis due to systemic progression of the disease at age of 5. LCH commonly present with the symptoms of diabetes insipidus, but hypothalamic mass is not common. We report this case with a brief review of literatures.
