An extremely rare association of TSH-secreting pituitary adenoma, metastatic neuroendocrine tumor and Cushing’s syndrome in a patient with MEN-1 gene mutation

Autori

  • Giuseppe Giuffrida Department of Clinical and Experimental Medicine, University of Messina
  • Erika Messina Unit of Endocrinology, “G. Martino” University Hospital, Messina
  • Petronilla Daniela Romeo Department of Human Pathology of childhood and adulthood “G. Barresi”, University of Messina
  • Adriana Albani Department of Human Pathology of childhood and adulthood “G. Barresi”, University of Messina
  • Valeria Barresi Department of Human Pathology of childhood and adulthood “G. Barresi”, University of Messina
  • Sergio Baldari Unit of Nuclear Medicine, Department of Biomedical and Dental Sciences and Morphofunctional Imaging, University of Messina
  • Giuseppe Navarra Department of Human Pathology of childhood and adulthood “G. Barresi”, University of Messina
  • Francesco Ferraù Department of Human Pathology of childhood and adulthood “G. Barresi”, University of Messina
  • Salvatore Cannavò Department of Human Pathology of childhood and adulthood “G. Barresi”, University of Messina

DOI:

https://doi.org/10.6092/1828-6550/APMB.106.1.2018.A4

Parole chiave:

MEN-1, TSH-secreting pituitary adenomas, adrenal hyperplasia, Cushing’s syndrome, hyperparathyroidism

Abstract

Multiple endocrine neoplasia (MEN)-1 syndrome is a rare disorder, due to the loss of function of the tumor suppressor menin. It consists of the association of two or more endocrine tumors, often presenting in a familial setting, being inherited in an autosomal dominant fashion. The most frequent manifestations of MEN-1 syndrome are primary hyperparathyroidism, followed by pituitary adenomas (mainly prolactinomas) and gastrointestinal neuroendocrine tumors, but several other associated conditions have been reported. Herein we describe the case of a male patient, affected by sporadic MEN-1, diagnosed with primary hyperparathyroidism, TSH-secreting pituitary adenoma and bilateral adrenal hyperplasia causing Cushing’s syndrome, due to a de novo MEN-1 gene mutation. The patient has been successfully treated with first generation somatostatin analog Octreotide LAR (30 mg every 28 days) -with stabilization of the known neuroendocrine lesions and shrinkage of the pituitary adenoma- and with bilateral adrenalectomy. The patient is still regularly followed-up at our Endocrine Unit, and his clinical conditions are stable.

Riferimenti bibliografici

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Dowloads

Pubblicato

2018-06-14

Fascicolo

V. 106 N. 1 (2018)

Sezione

CLINICAL CASE SEMINAR

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