Langerhans cell histiocytosis limited to the pituatary-hypothalamic axis

Ayako Isoo, Keisuke Ueki, Tsuyoshi Ishida, Tetsushi Yoshikawa, Takamitsu Fujimaki, Ichiro Suzuki, Tomio Sasaki, Takaaki Kirino

Research output: Contribution to journalArticle

14 Citations (Scopus)

Abstract

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 benign lesion, as opposed to attempting total removal of the mass.

Original languageEnglish
Pages (from-to)532-535
Number of pages4
JournalNeurologia medico-chirurgica
Volume40
Issue number10
DOIs
Publication statusPublished - 01-01-2000

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Langerhans Cell Histiocytosis
Hypopituitarism
Magnetic Resonance Imaging
Neurogenic Diabetes Insipidus
Germinoma
Polydipsia
Polyuria
Diabetes Insipidus
Histiocytes
Craniotomy
Amenorrhea
Pituitary Gland
Eosinophils
Neuroimaging
Cluster Analysis
Biopsy
Therapeutics

All Science Journal Classification (ASJC) codes

  • Surgery
  • Clinical Neurology

Cite this

Isoo, A., Ueki, K., Ishida, T., Yoshikawa, T., Fujimaki, T., Suzuki, I., ... Kirino, T. (2000). Langerhans cell histiocytosis limited to the pituatary-hypothalamic axis. Neurologia medico-chirurgica, 40(10), 532-535. https://doi.org/10.2176/nmc.40.532
Isoo, Ayako ; Ueki, Keisuke ; Ishida, Tsuyoshi ; Yoshikawa, Tetsushi ; Fujimaki, Takamitsu ; Suzuki, Ichiro ; Sasaki, Tomio ; Kirino, Takaaki. / Langerhans cell histiocytosis limited to the pituatary-hypothalamic axis. In: Neurologia medico-chirurgica. 2000 ; Vol. 40, No. 10. pp. 532-535.
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Isoo, A, Ueki, K, Ishida, T, Yoshikawa, T, Fujimaki, T, Suzuki, I, Sasaki, T & Kirino, T 2000, 'Langerhans cell histiocytosis limited to the pituatary-hypothalamic axis', Neurologia medico-chirurgica, vol. 40, no. 10, pp. 532-535. https://doi.org/10.2176/nmc.40.532

Langerhans cell histiocytosis limited to the pituatary-hypothalamic axis. / Isoo, Ayako; Ueki, Keisuke; Ishida, Tsuyoshi; Yoshikawa, Tetsushi; Fujimaki, Takamitsu; Suzuki, Ichiro; Sasaki, Tomio; Kirino, Takaaki.

In: Neurologia medico-chirurgica, Vol. 40, No. 10, 01.01.2000, p. 532-535.

Research output: Contribution to journalArticle

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T1 - Langerhans cell histiocytosis limited to the pituatary-hypothalamic axis

AU - Isoo, Ayako

AU - Ueki, Keisuke

AU - Ishida, Tsuyoshi

AU - Yoshikawa, Tetsushi

AU - Fujimaki, Takamitsu

AU - Suzuki, Ichiro

AU - Sasaki, Tomio

AU - Kirino, Takaaki

PY - 2000/1/1

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N2 - 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 benign lesion, as opposed to attempting total removal of the mass.

AB - 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 benign lesion, as opposed to attempting total removal of the mass.

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