Jump to ContentJump to Main Navigation
Show Summary Details
More options …

Journal of Pediatric Endocrinology and Metabolism

Editor-in-Chief: Kiess, Wieland

Ed. by Bereket, Abdullah / Darendeliler, Feyza / Dattani, Mehul / Gustafsson, Jan / Luo, Fei Hong / Mericq, Veronica / Ogata, Tsutomu / Toppari, Jorma

12 Issues per year


IMPACT FACTOR 2017: 1.086

CiteScore 2017: 1.07

SCImago Journal Rank (SJR) 2017: 0.465
Source Normalized Impact per Paper (SNIP) 2017: 0.580

Online
ISSN
2191-0251
See all formats and pricing
More options …
Volume 30, Issue 2

Issues

Turner syndrome and pituitary adenomas: a case report and review of literature

Tiffany Yeh
  • Department of Pediatrics, Rhode Island Hospital/Hasbro Children’s Hospital, Providence, RI, USA
  • Department of Medicine, Rhode Island Hospital, Providence, RI, USA
  • The Warren Alpert Medical School of Brown University, Rhode Island, Providence, RI, USA
  • Other articles by this author:
  • De Gruyter OnlineGoogle Scholar
/ Angela Ganan Soto / Jose Bernardo Quintos
  • The Warren Alpert Medical School of Brown University, Rhode Island, Providence, RI, USA
  • Division of Pediatric Endocrinology, Rhode Island Hospital/Hasbro Children’s Hospital, Providence, RI, USA
  • Other articles by this author:
  • De Gruyter OnlineGoogle Scholar
/ Lisa Swartz Topor
  • Corresponding author
  • The Warren Alpert Medical School of Brown University, Rhode Island, Providence, RI, USA
  • Division of Pediatric Endocrinology, Rhode Island Hospital/Hasbro Children’s Hospital, Providence, RI, USA
  • Email
  • Other articles by this author:
  • De Gruyter OnlineGoogle Scholar
Published Online: 2017-01-18 | DOI: https://doi.org/10.1515/jpem-2016-0202

Abstract

Background:

Turner syndrome (TS) is the most common sex chromosome abnormality in females, typically associated with primary amenorrhea and premature ovarian failure due to gonadal dysgenesis. The association of TS with hypopituitarism is an uncommon finding. The objective of the study was to describe an adolescent with TS with hypergonadotropic hypogonadism and subsequent hypogonadotropic hypogonadism.

Case presentation:

A 16-year-old female with primary amenorrhea was diagnosed with TS based on karyotype 45,XO. Other laboratory values included FSH 45.52 IU/L, LH 17.4 IU/L, undetectable estradiol, and prolactin 1.08 nmol/L. Two months later and before treatment, she presented with severe headache and a new left cranial nerve VI palsy. Brain MRI showed a 2.7-cm hemorrhagic pituitary macroadenoma expanding the sella. Laboratory evaluation showed FSH 5.9 IU/L, LH 0.9 IU/L, prolactin 0.09 nmol/L, and GH 1.03 ng/mL. She underwent transphenoidal hypophysectomy, and pathology revealed pituitary adenoma with immunohistochemical staining positive for growth hormone and prolactin. She subsequently developed multiple pituitary hormone deficiencies. Review of the literature identified eight case reports of women with TS who developed pituitary adenomas.

Conclusions:

This case illustrates an uncommon co-occurrence of TS and pituitary macroadenoma. Sequential gonadotropin measurements demonstrate the evolution of hypergonadotropic hypogonadism into hypogonadotropic hypogonadism due to hemorrhagic pituitary macroadenoma.

Keywords: hypergonadotropic hypogonadism; hypogonadotropic hypogonadism; pituitary adenoma; Turner syndrome

References

  • 1.

    Miguel-Neto J, Carvalho AB, Marques-de-Faria AP, Guerra-Junior G, Maciel-Guerra AT. New approach to phenotypic variability and karyotype-phenotype correlation in Turner syndrome. J Pediatr Endocrinol Metab 2016;29:475–9.Google Scholar

  • 2.

    Chrysis D, Spiliotis BE, Stene M, Cacciari E, Davenport ML. Gonadotropin secretion in girls with turner syndrome measured by an ultrasensitive immunochemiluminometric assay. Horm Res 2006;65:261–6.Google Scholar

  • 3.

    Hagen CP, Main KM, Kjaergaard S, Juul A. FSH, LH, inhibin B and estradiol levels in Turner syndrome depend on age and karyotype: longitudinal study of 70 Turner girls with or without spontaneous puberty. Hum Reprod 2010;25:3134–41.Google Scholar

  • 4.

    Pasquino AM, Passeri F, Pucarelli I, Segni M, Municchi G. Spontaneous pubertal development in Turner’s syndrome. Italian Study Group for Turner’s Syndrome. J Clin Endocrinol Metab 1997;82:1810–3.Google Scholar

  • 5.

    Mermilliod JA, Gatchair-Rose A, Svec F. Pituitary tumor and low gonadotropins in a patient with Turner’s syndrome. J Louisiana St Med Soc 1995;147:540–3.Google Scholar

  • 6.

    Weibel HS, Dahan MH. Pituitary mass and subsequent involution causing fluctuations of serum follicle-stimulating hormone levels in a Turner syndrome patient with premature ovarian failure: a case report. J Reprod Med 2014;59:504–8.Google Scholar

  • 7.

    Gaspar L, Julesz J, Kocsis J, Pasztor E, Laszlo F. Mosaic Turner’s syndrome and pituitary microadenoma. Exp Clin Endocrinol 1985;86:87–92.Google Scholar

  • 8.

    Dotsch J, Schoof E, Hensen J, Dorr HG. Prolactinoma causing secondary amenorrhea in a woman with Ullrich-Turner syndrome. Horm Res 1999;51:256–7.Google Scholar

  • 9.

    Willemse CH. A patient suffering from Turner’s syndrome and acromegaly. Acta Endocrinol 1962;39:204–12.Google Scholar

  • 10.

    Bolanowski M, Lomna-Bogdanov E, Kosmala W, Malczewska J, Slezak R, et al. Turner’s syndrome followed by acromegaly in the third decade of life: an unusual coincidence of two rare conditions. Gynecol Endocrinol 2002;16:331–4.Google Scholar

  • 11.

    Yamazaki M, Sato A, Nishio S, Takeda T, Miyamoto T, et al. Acromegaly accompanied by Turner syndrome with 47,XXX/45,X/46,XX mosaicism. Intern Med 2009;48:447–53.Google Scholar

  • 12.

    Gelfand RA. Cushing’s disease associated with ovarian dysgenesis. Am J Med 1984;77:1108–10.Google Scholar

  • 13.

    Negreiros LP, Bolina ER, Guimaraes MM. Pubertal development profile in patients with Turner syndrome. J Pediatr Endocrinol Metab 2014;27:845–9.Google Scholar

  • 14.

    Lunding SA, Aksglaede L, Anderson RA, Main KM, Juul A, et al. AMH as predictor of premature ovarian insufficiency: a longitudinal study of 120 Turner syndrome patients. J Clin Endocrinol Metab 2015;100:E1030–8.Google Scholar

  • 15.

    Scheithauer BW, Kovacs K, Horvath E, Young WF, Jr., Lloyd RV. The pituitary in Turner syndrome. Endocr Pathol 2005;16:195–200.Google Scholar

  • 16.

    Guarneri MP, Abusrewil SA, Bernasconi S, Bona G, Cavallo L, et al. Turner’s syndrome. J Pediatr Endocrinol Metab 2001;14(Suppl 2):959–65.Google Scholar

  • 17.

    Bechtold S, Dalla Pozza R, Schmidt H, Bonfig W, Schwarz HP. Pubertal height gain in Ullrich-Turner syndrome. J Pediatr Endocrinol Metab 2006;19:987–93.Google Scholar

About the article

Corresponding author: Lisa Swartz Topor, MD, MMSc, Division of Pediatric Endocrinology, Rhode Island Hospital/Hasbro Children’s Hospital, 593 Eddy Street, Providence, RI 02903, USA, Phone: +401-444-5504, Fax: +401-444-2534


Received: 2016-05-20

Accepted: 2016-11-21

Published Online: 2017-01-18

Published in Print: 2017-02-01


Author contributions: All the authors have accepted responsibility for the entire content of this submitted manuscript and approved submission.

Research funding: None declared.

Employment or leadership: None declared.

Honorarium: None declared.

Competing interests: The funding organization(s) played no role in the study design; in the collection, analysis, and interpretation of data; in the writing of the report; or in the decision to submit the report for publication.


Citation Information: Journal of Pediatric Endocrinology and Metabolism, Volume 30, Issue 2, Pages 231–235, ISSN (Online) 2191-0251, ISSN (Print) 0334-018X, DOI: https://doi.org/10.1515/jpem-2016-0202.

Export Citation

©2017 Walter de Gruyter GmbH, Berlin/Boston.Get Permission

Citing Articles

Here you can find all Crossref-listed publications in which this article is cited. If you would like to receive automatic email messages as soon as this article is cited in other publications, simply activate the “Citation Alert” on the top of this page.

[1]
Wenai Song, Yi Lei, Shi Chen, Zhouxian Pan, Ji-Jiang Yang, Hui Pan, Xiaoliang Du, Wubin Cai, and Qing Wang
Computers in Industry, 2018, Volume 100, Page 85

Comments (0)

Please log in or register to comment.
Log in