Langerhans Cell Histiocytosis Limited to the Pituitary-Hypothalamic Axis
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概要
- 論文の詳細を見る
Langerhans cell histiocytosis rarely presents as a solitary lesion in the pituitary-hypothalamic region, and is indistinguishable from germinoma, which occurs much more frequently, especially in Japanese. A 14-year-old girl and a 9-year-old girl presented with polydipsia and polyuria as the initial symptoms. Magnetic resonance(MR)imaging demonstrated a round mass at the pituitary stalk appearing as isointense on T_1-weighted imaging and hyperintense on T_2-weighted imaging. Endocrinological examination revealed mild hypopituitarism with central diabetes insipidus. Both patients underwent open craniotomy. Histological examination revealed granulomatous tissue with eosinophil infiltration and frequent Langerhans histiocyte clustering, compatible with the diagnosis of Langerhans cell histiocytosis. Low-dose local irradiation of 20 Gy was administered. First patient was followed up for 8 years, and her hypopituitarism gradually improved to a minimal level with only amenorrhea as the residual symptom. Recent MR imaging showed no residual mass at the region. Second patient was followed up for 15 months, and her diabetes insipidus is stable. MR imaging performed 5 months after the treatment showed marked reduction of the mass. These cases reemphasize the importance of histological diagnosis for lesions with similar neuroimaging appearances. Biopsy and low-dose irradiation are an effective treatment for this rare and essentially lesion, as opposed to attempting total removal of the mass.
- 日本脳神経外科学会の論文
- 2000-10-15
著者
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SASAKI Tomio
Department of Neurosurgery, Graduate School of Medical Sciences, Kyushu University
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YOSHIKAWA Tetsushi
Department of Pediatrics, Fujita Health University School of Medicine
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Yoshikawa Tetsushi
Department Of Pediatrics Fujita Health University School Of Medicine
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UEKI Keisuke
Department of Neurosurgery, Faculty of Medicine, University of Tokyo
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Fujimaki Takamitsu
Department Of Neurosurgery Teikyo University Itabashi Hospital
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Fujimaki Takamitsu
Department Of Neurosurgery
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ISOO Ayako
Department of Neurosurgery
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ISHIDA Tsuyoshi
Pathology, The University of Tokyo Hospital
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SUZUKI Ichiro
Department of Neurosurgery
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KIRINO Takaaki
Department of Neurosurgery
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Ueki Keisuke
Department Of Neurosurgery The University Of Tokyo Hospital
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Sasaki Tsutomu
Department Of Neurosurgery The University Of Tokyo Hospital
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Sasaki Tomio
Department Of Neurosurgery Faculty Of Medicine University Of Tokyo
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Kirino Takaaki
Department Of Neurosurgery The University Of Tokyo Hospital
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Ueki Keisuke
Department Of Neurosurgery Dokkyo University School Of Medicine
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Ishida Tsuyoshi
Department Of Pathology The University Of Tokyo Hospital
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Sasaki Tomio
Department Of Neurosurgery Faculty Of Medicine Tokyo University
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Suzuki Ichiro
Department Of Chemical And Energy Engineering Graduate School Of Engineering Yokohama National Unive
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Ueki Keisuke
Department Of Neurologic Surgery Dokkyo University
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YOSHIKAWA Tetsushi
Department of Pediatrics, Fujita Health University, School of Medicine
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