Langerhans cell histiocytosis (LCH) has diverse clinical manifestations, including intracranial mass lesions. We
report a case of LCH that manifested as a suprasellar mass, and initially misdiagnosed as a
germ cell tumor. A 29-year-old
woman presented with
polyuria,
polydipsia and
amenorrhea.
Laboratory findings revealed
hypopituitarism with
central diabetes insipidus, and a suprasellar mass and a pineal mass were observed on
magnetic resonance imaging. Under the clinical impression of a
germ cell tumor, the
patient was treated with
germ cell tumor chemotherapy (
cisplatin and
etoposide) and
radiation therapy without
biopsy. After initial shrinkage of the lesions, further
growth of the
tumor was observed and a
biopsy was performed. The histopathology revealed LCH. After
chemotherapy according to the LCH III protocol, the
tumor disappeared. She is on regular follow up for 5 years without
relapse. The present findings indicate that LCH should be included in the
differential diagnosis of a suprasellar mass, even in
adults, especially when it manifests with
diabetes insipidus. This case also underscores the importance of a histopathologic
diagnosis in
patients with suprasellar
tumors before the initiation of a specific
therapy, even if the clinical findings are highly suggestive of a specific
diagnosis.