Modern classifications of pituitary neuroendocrine tumors

Pronin, Vyacheslav; Antsiferov, M.B. Antsiferov; Alekseeva, T.M.; Pronin, Eugeny

doi:10.33029/2304-9529-2021-10-2-48-64

Cited by 1 publication

(1 citation statement)

References 0 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…В настоящее время в литературе описано 14 клинических случаев гипофизарной бластомы. Для всех пациентов был характерен ранний дебют заболевания — от 7 до 18 месяцев, что позволяет расценивать образование как врожденное [ 23 ]. Заболевание у всех пациентов проявлялось болезнью Кушинга, повышением уровня АКТГ и кортизола, офтальмоплегией, у одной пациентки отмечалась еще и клиника несахарного диабета [ 23 ].…”

Section: эндокринные проявления дайсеропатийunclassified

DICER1 syndrome: clinical variety endocrine manifestations and features of diagnostics

Novokreshennih,

Kolodkina,

Bezlepkina

2023

Probl Endokrinol (Mosk)

View full text Add to dashboard Cite

DICER1 syndrome is a rare genetic disorder with the progressive development of malignant and non-malignant diseases in childhood. The cause of this syndrome is a dusfunction of the endoribonuclease DICER, which plays an important role in the processing of microRNAs with subsequent regulation of the control of the expression of oncogenes and tumor suppressor genes. Clinical manifestations of dyseropathies is very different and may include both endocrine manifestations – multinodular goiter, differentiated thyroid cancers, ovarian stromal tumors, pituitary blastoma, and non–endocrine formations — pleuropulmonary blastoma, cystic nephroma, pineoblastoma. The presence of somatic mutations of the DICER1 gene is a resultant stage in the pathogenesis of dyseropathies, determining the further path of oncogenesis. At present, DICER1 syndrome is diagnosed extremely rarely, which leads to late detection of the components of the disease in the patient, late diagnosis of neoplasms, lack of family counseling. Diagnosis at the early stages of the disease, the development of screening programs for the management of these patients allows minimizing the risks of developing more malignant, aggressive forms of the disease.

show abstract

Section: эндокринные проявления дайсеропатийunclassified