Relapse of the pituitary adenoma with a change of its hormonal activity in a female patient with multiple endocrine neoplasia syndrome type 1

Abstract

Multiple endocrine neoplasia syndrome type 1 (MEN1, Wermer's syndrome) is a group of heterogeneous inherited diseases, with its pathogenesis related to hyperplasia or neoplasms of several endocrine glands. This syndrome is characterized by autosomal dominant mode of inheritance, high penetrance and similar prevalence among males and females. Prevalence of MEN1 is estimated to be 1:100,000 of the population. An interesting feature of the presented clinical case is a relapse and transformation of pituitary tumor from a prolactin-secreting into the mixed one, with distinct compartments of ACTH- and prolactin-secreting, in a female patient with a family MEN1 syndrome, with involvement of the pancreas, parathyroid and pituitary glands. Her brother had a synchronous manifestation of the same types of tumors, except corticotropinoma. The presented clinical case highlights the necessity of a comprehensive and life-long follow-up of MEN1 patients for a timely detection of neoplasms and appropriate treatment.

About the authors

L. Ya. Rozhinskaya

Endocrinology Research Center

Email: fake@neicon.ru

Liudmila Yа. Rozhinskaya – MD, PhD, Professor, Chief Research Fellow, Department of Neuroendocrinology and Osteopathy.

11 Dmitriya Ul'yanova ul., Moscow, 117036

Russian Federation

P. M. Khandaeva

Endocrinology Research Center

Author for correspondence.
Email: pati_khandaeva@mail.ru

Patimat M. Khandaeva – MD, Research Fellow, Department of Neuroendocrinology and Osteopathy.

11 Dmitriya Ul'yanova ul., Moscow, 117036, tel.: +7 (906) 713 11 91

Russian Federation

A. S. Lutsenko

Endocrinology Research Center

Email: fake@neicon.ru

Alexander S. Lutsenko – MD, Research Fellow, Department of Neuroendocrinology and Osteopathy.

11 Dmitriya Ul'yanova ul., Moscow, 117036

Russian Federation

A. M. Lapshina

Endocrinology Research Center

Email: fake@neicon.ru

Anastasiya M. Lapshina – MD, PhD, Pathoanatomist, Department of Fundamental Pathomorphology.

11 Dmitriya Ul'yanova ul., Moscow, 117036

Russian Federation

A. Yu. Grigor'ev

Endocrinology Research Center

Email: fake@neicon.ru

Andrey Yu. Grigor'ev – MD, PhD, Head of Department of Neurosurgery.

11 Dmitriya Ul'yanova ul., Moscow, 117036

Russian Federation

S. D. Arapova

Endocrinology Research Center

Email: fake@neicon.ru

Svetlana D. Arapova – MD, Leading Research Fellow, Department of Neuroendocrinology and Osteopathy.

11 Dmitriya Ul'yanova ul., Moscow, 117036

Russian Federation

Zh. E. Belaya

Endocrinology Research Center

Email: fake@neicon.ru

Zhanna E. Belaya – MD, PhD, Chief Research Fellow, Head of Department of Neuroendocrinology and Osteopathy.

11 Dmitriya Ul'yanova ul., Moscow, 117036

Russian Federation

G. A. Mel'nichenko

Endocrinology Research Center

Email: fake@neicon.ru

Galina A. Mel'nichenko – Member of Russ. Acad. Sci., MD, PhD, Professor, Director of Institut of Clinical Endocrinology, Deputy Director.

11 Dmitriya Ul'yanova ul., Moscow, 117036

Russian Federation

References

  1. Симоненко ВБ, Дулин ПА, Маканин МА. Нейроэндокринные опухоли. М.: ГЭОТАР-Медиа; 2010. 237 с.
  2. Feng Z, Ma J, Hua X. Epigenetic regulation by the menin pathway. Endocr Relat Cancer. 2017;24(10):T147–59. doi: 10.1530/ERC-17-0298.
  3. Мамедова ЕО, Пржиялковская ЕГ, Пигарова ЕА, Мокрышева НГ, Дзеранова ЛК, Тюльпаков АН. Аденомы гипофиза в рамках наследственных синдромов. Проблемы эндокринологии. 2014;60(4): 51–9. doi: 10.14341/probl201460438-46.
  4. Friesen SR. The APUD syndromes. Prog Clin Cancer. 1982;8:75–87.
  5. Piecha G, Chudek J, Wiecek A. Multiple Endocrine Neoplasia type 1. Eur J Intern Med. 2008;19(2): 99–103. doi: 10.1016/j.ejim.2007.08.004.
  6. Ростомян ЛГ, Рожинская ЛЯ, Тюльпаков АН. Клинико-генетические характеристики синдрома множественных эндокринных неоплазий типа 1 и принципы его лечения. Фарматека. 2010;(3): 50–6.
  7. Ростомян ЛГ. Синдром множественных эндокринных неоплазий 1 типа: распространенность среди пациентов с первичным гиперпаратиреозом, клинические и молекулярно-генетические характеристики. Дис. ... кандидат медицинских наук. М., 2011.
  8. Uraki S, Ariyasu H, Doi A, Furuta H, Nishi M, Usui T, Yamaue H, Akamizu T. Hypersecretion of ACTH and PRL from pituitary adenoma in MEN1, adequately managed by medical therapy. Endocrinol Diabetes Metab Case Rep. 2017;2017. pii: 17–0027. doi: 10.1530/EDM-170027.
  9. T'Sjoen G, Defeyter I, Van De Saffele J, Rubens R, Vandeweghe M. Macroprolactinoma associated with Cushing's disease, successfully treated with cabergoline. J Endocrinol Invest. 2002;25(2): 172–5.
  10. Gheri RG, Boddi W, Ammannati F, Olivotto J, Nozzoli C, Franchi A, Bordi L, Luisi ML, Mennonna P. Two-step development of a pituitary adenoma: from hyperprolactinemic syndrome to Cushing's disease. J Endocrinol Invest. 1997;20(4): 240–4. doi: 10.1007/BF03346911.
  11. Brown RL, Wollman R, Weiss RE. Transformation of a pituitary macroadenoma into to a corticotropin-secreting carcinoma over 16 years. Endocr Pract. 2007;13(5): 463–71. doi: 10.4158/EP.13.5.463.

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Copyright (c) 2018 Rozhinskaya L.Y., Khandaeva P.M., Lutsenko A.S., Lapshina A.M., Grigor'ev A.Y., Arapova S.D., Belaya Z.E., Mel'nichenko G.A.

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