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01-05-2016 | Case Report

Solitary Langerhans cell histiocytosis located in the neurohypophysis with a positive titer HCG-β in the cerebrospinal fluid

Authors: Yasuyuki Kinoshita, Fumiyuki Yamasaki, Satoshi Usui, Vishwa Jeet Amatya, Atsushi Tominaga, Kazuhiko Sugiyama, Kaoru Kurisu

Published in: Child's Nervous System | Issue 5/2016

Abstract

Introduction

Beta-human chorionic gonadotropin (HCG-β) is considered to be a useful tumor marker for germ cell tumors (GCTs); however, various tumors other than GCTs, including cystic pituitary adenomas, Rathke’s cleft cysts, and craniopharyngiomas, were reported to express HCG-β.

Case report

We herein present the case of a 5-year-old boy who presented with polyuria and had a solitary lesion in the neurohypophysis with a positive HCG-β titer in the cerebrospinal fluid. Under a preoperative diagnosis of germinoma, a biopsy was performed from the posterior pituitary lobe via the transsphenoidal endoscopic approach and the histological diagnosis was revealed to be Langerhans cell histiocytosis (LCH).

Conclusions

The finding of a slightly positive HCG-β titer in the cerebrospinal fluid (CSF) cannot exclude the possibility of LCH, and we strongly recommend a histological diagnosis for the diagnosis of a solitary neurohypophysial lesion.

Bellodi A, Rivoli G, Arboscello E, Fornarini G, Orcioni G, Avenoso D et al (2015) Hepatosplenic T-cell lymphoma with aberrant expression of serum β-HCG: a case report. Tumori. doi:10.5301/tj.5000373

Bromberg JEC, Baumert BG, de Vos F, Gijtenbeek JMM, Kurt E, Westermann AM et al (2013) Primary intracranial germ-cell tumors in adults: a practical review. J Neurooncol 113:175–183CrossRefPubMed

Demirtas E, Krishnamurthy S, Tulandi T (2007) Elevated serum beta-human chorionic gonadotropin in nonpregnant conditions. Obstet Gynecol Surv 62:675–679CrossRefPubMed

Di Iorgi N, Allegri AEM, Napoli F, Calcagno A, Calandra E, Fratangeli N et al (2014) Central diabetes insipidus in children and young adults: etiological diagnosis and long-term outcome of idiopathic cases. J Clin Endocrinol Metab 99:1264–1272CrossRefPubMed

Harris PE, Perry L, Chard T, Chaudry L, Cooke BA, Touzel R et al (1988) Immunoreactive human chorionic gonadotropin from the cyst fluid and CSF of patients with craniopharyngioma. Clin Endocrinol (Oxf) 29:503–508CrossRef

Honegger J, Mann K, Thierauf P, Zrinzo A, Fahlbusch R (1995) Human chorionic gonadotropin immunoactivity in cystic intracranial tumors. Clin Endocrinol (Oxf) 42:235–241CrossRef

Howarth DM, Gilchrist GS, Mullan BP, Wiseman GA, Edmonson JH, Schomberg PJ (1999) Langerhans cell histiocytosis diagnosis, natural history, management, and outcome. Cancer 85:2278–2290CrossRefPubMed

Jian F, Bian L, Sun S, Yang J, Chen X, Chen Y et al (2014) Surgical biopsies in patients with central diabetes insipidus and thickened pituitary stalks. Endocrine 47:325–335CrossRefPubMed

Jinguji S, Nishiyama K, Yoshimura J, Yoneoka Y, Harada A, Sano M et al (2013) Endoscopic biopsies of lesions associated with a thickened pituitary stalk. Acta Neurochir (Wien) 155:119–124CrossRef

10.

Kinoshita Y, Tominaga A, Usui S, Kurisu K (2014) A craniopharyngioma with spontaneous involution of a gadolinium-enhanced region on magnetic resonance imaging. Surg Neurol Int 5:128CrossRefPubMedPubMedCentral

11.

Kugasia I, Alkayem M, Patel J (2014) A rare case of β-hCG production by a solitary fibrous tumor of the pleura. Am J Case Rep 15:518–522CrossRefPubMedPubMedCentral

12.

Nagasaki K, Tsumanuma I, Yoneoka Y, Ogawa Y, Kikuchi T, Uchiyama M (2009) Spontaneous regression of isolated neurohypophyseal Langerhans cell histiocytosis with diabetes insipidus. Endocr J 56:721–725CrossRefPubMed

13.

Prosch H, Grois N, Prayer D, Waldhauser F, Steiner M, Minkov M et al (2004) Central diabetes insipidus as presenting symptom of Langerhans cell histiocytosis. Pediatr Blood Cancer 43:594–599CrossRefPubMed

14.

Stenman UH, Alfthan H, Hotakainen K (2004) Human chorionic gonadotropin in cancer. Clin Biochem 37:549–561CrossRefPubMed

15.

Tachibana O, Yamashima T, Yamashita J, Takabatake Y (1994) Immunohistochemical expression of human chorionic gonadotropin and P-glycoprotein in human pituitary glands and craniopharyngiomas. J Neurosurg 80:79–84CrossRefPubMed

16.

Takami H, Fukushima S, Fukuoka K, Suzuki T, Yanagisawa T, Matsushita Y et al (2015) Human chorionic gonadotropin is expressed virtually in all intracranial germ cell tumors. J Neurooncol 124:23–32CrossRefPubMed

17.

The committee of the brain tumor registry of Japan: report of brain tumor registry of Japan (2001–2004) 13th edition (2014). Neurol Med Chir (Tokyo) 54:9–102

Title: Solitary Langerhans cell histiocytosis located in the neurohypophysis with a positive titer HCG-β in the cerebrospinal fluid
Authors: Yasuyuki Kinoshita
Fumiyuki Yamasaki
Satoshi Usui
Vishwa Jeet Amatya
Atsushi Tominaga
Kazuhiko Sugiyama
Kaoru Kurisu
Publication date: 01-05-2016
Publisher: Springer Berlin Heidelberg
Published in: Child's Nervous System / Issue 5/2016
Print ISSN: 0256-7040
Electronic ISSN: 1433-0350
DOI: https://doi.org/10.1007/s00381-015-2949-2

ACC 2024 Congress

Springer Medicine

Solitary Langerhans cell histiocytosis located in the neurohypophysis with a positive titer HCG-β in the cerebrospinal fluid

Abstract

Introduction

Case report

Conclusions

ACC 2024 Congress

Springer Medicine

Abstract

Introduction

Case report

Conclusions

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