An Institutional Audit and Pictorial Review of Langerhans’ Cell Histiocytosis Presented with Intracranial Manifestations

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DD Rasalkar, C Tong, FWT Cheng, CK Li, BK Paunipagar, WCW Chu

Hong Kong J Radiol 2010;13:46-51

Objective: To review the intracranial manifestations and role of imaging in Langerhans’ cell histiocytosis.

Methods: This is a retrospective institutional review of clinical data and radiological findings of Langerhans’ cell histiocytosis patients presenting to a tertiary Cancer Centre over the past 11 years. Of 23 patients with confirmed Langerhans’ cell histiocytosis, 6 had central nervous system involvement (1 female, 5 male; age range, 2-59 years; mean age, 16 years). One of these patients only affected the hypothalamic pituitary axis, while 5 had additional bone or multisystem involvement. All the patients underwent radiographic skeletal survey, chest radiography, and abdominal ultrasound. Brain computed tomography and magnetic resonance imaging limited to areas of suspected histiocytic infiltration were performed.

Results: Four patients had an absent T1 bright signal of the posterior pituitary gland and nodular enhancement of the infundibular stalk. After treatment, the latter feature resolved. One patient had an empty sella on long-term follow-up. Two patients had brain parenchymal involvement in the form of a focal lesion in the right temporal lobe with excessive perilesional white matter oedema and multiple small nodular-enhancing lesions involving both the cerebrum and brainstem. The patient with brainstem lesion endured a rapidly deteriorating clinical course and succumbed due to urosepsis and respiratory failure.

Conclusions: There was intracranial involvement in 26% of this patient cohort with Langerhans’ cell histiocytosis, and manifested 2 extreme patterns of central nervous system involvement. The less severe form (67%) involved the hypothalamic-pituitary axis; the patient suffered chronic pituitary gland insufficiency despite radiological regression after treatment. The more severe pattern (33%) entailed brain parenchymal infiltration resulting in variable neurological deficits.




DD Rasalkar、唐倩儂、鄭偉才、李志光、BK Paunipagar、朱昭穎


方法:回顧過去11年內就診於一所三級癌症中心的蘭格罕細胞組織球增生病人的臨床及影像學資 料。23位確診患者中,6位中樞神經系統受累(5男1女;年齡介乎2至59歲,平均16歲);其中1名患 者只累及下丘腦—垂體軸,其餘5名還累及骨骼或多個系統。所有病人均接受全身骨骼及胸部X光檢 查及腹部超聲波檢查,並對懷疑有組織細胞浸潤的部位進行腦部電腦斷層及磁力共振影像。

結果:4名病人T1加權像垂體後葉高信號消失,漏斗柄呈結節狀强化。治療後,漏斗柄强化消失。 其中1名病人長期隨訪時有空蝶鞍。兩名病人的右側顳葉局部腦實質內出現病灶伴週邊腦白質大量水 腫,及大腦半球和腦幹多發小結節狀强化病灶。腦幹病變患者的病情急轉直下,最終因敗血症和呼 吸衰竭死亡。

結論:本組蘭格罕細胞組織球增生症患者中,26%的病人有顱內侵犯,病情表現為中樞神經系统受 累的兩種極端模式。輕者(67%)為下丘腦—垂體軸受累,雖然治療後影像學表現可改善,但仍有 慢性腦垂體功能不全。重者(33%)腦實質浸潤,導致不同程度的神經功能缺損。