A 48-year-old woman with diabetes insipidus, galactorrhea, and fatigue for 10 months
A
B
C
What is your diagnosis?
The answer is Langerhans Cell Histiocytosis
Hypothalamic-pituitary Langerhans cell histiocytosis is rare in adulthood.
Panhypopituitarism, including diabetes insipidus, is a common presenting symptom.
The classic MR imaging features include a strongly contrast-enhanced mass in the hypothalamic-pituitary region on T1WI (arrow in A) and infiltration of tumor in the hypothalamus (red arrow in B) and optic tracts (yellow arrows in B) on T2 FLAIR.
Noncontrast T1WI may show loss of posterior pituitary high signal (not shown).
Photomicrograph from surgical specimen (H&E, x 400 ) shows Langerhans cells with abundant, pale eosinophilic cytoplasm, and irregular and elongated nuclei with prominent nuclear grooves (arrows in C).
中文版:
女性,48歲,尿崩症,溢乳,疲乏10月餘
診斷:朗格漢斯組織細胞增生症
發生於成人下丘腦-垂體區域朗格漢斯組織細胞增生症罕見。
常見症狀為垂體功能減退,包括尿崩症。
MRI典型表現:T1WI示下丘腦-垂體區域佔位性病變,增強呈明顯強化(A圖箭頭),T2FLAIR示腫瘤侵犯下丘腦(B圖紅箭頭)及視束(B圖黃箭頭)。
T1WI平掃可表現為垂體後葉高信號影消失(圖片未顯示)。
外科標本顯微鏡下(HE染色,400倍)可見朗格漢斯細胞,其細胞質蒼白、嗜酸性,可見細長的細胞核以及明顯的核溝(C圖箭頭)。
From AJNR Classic Case