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Bogdana Zoica

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Articole semnate de acelasi autor in Revista Romana de Pediatrie:

Adrenal rest tumours in an adolescent with congenital adrenal hyperplasia – one year follow-up

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Revista Romana de PEDIATRIE | Volumul LXI, Nr. 1, An 2012
ISSN 1454-0398  |  e-ISSN 2069-6175
ISSN-L 1454-0398
DOI: 10.37897/RJP

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Adrenal rest tumours in an adolescent with congenital adrenal hyperplasia – one year follow-up

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ABSTRACT

Congenital adrenal hyperplasia (CAH) is an inherited disorder resulting in impaired production of cortisol and aldosterone. Male patients with CAH may present testicular adrenal rest tumours (TART).

Aim. To discuss the embryological, clinical features and treatment options of TART in a patient with CAH.

Case report. We present a case of TART in a 16 years and 6 months adolescent with salt-wasting type of CAH. The patient had periodic clinical evaluations, complete metabolic and endocrinological profile which guided the treatment. He was diagnosed at 6 weeks, when he begun substitution treatment with hydrocortisone. He was a compliant patient until age 14 years and 8 months, when he started high school and moved from home. At the age of 15 years and 6 months the clinical exam showed bilateral testis enlargement. The scrotal ultrasound sowed bilateral non-homogeneous, extensive masses. Hormone profile revealed increased ACTH and 17-OHprogesterone levels. Treatment was made by changing the night dose of hydrocortisone into dexamethasone to prolong ACTH suppression. After one year of treatment the testicular masses underwent important volume decrease.

Conclusions. Puberty has a negative impact on treatment compliance, so increasing the risk for TART. They are not malignant, but can result in gonadal dysfunction and infertility. It is important to screen for these tumours in CAH patients, to inform, discuss and offer cryopreservation of semen as soon as possible.

Key words: congenital adrenal hyperplasia, testicular adrenal rest tumours, adolescent

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TUMORI ADRENOTESTICULARE LA UN ADOLESCENT CU SINDROM ADRENOGENITAL – REZULTATE DUPA UN AN

SELECT ISSUE

Revista Romana de PEDIATRIE | Volumul LXI, Nr. 1, An 2012
ISSN 1454-0398  |  e-ISSN 2069-6175
ISSN-L 1454-0398
DOI: 10.37897/RJP

Indexed

DOI - Crossref
Similarity Check by iThenticate, worldwide No 1 professional plagiarism checking system
DOAJ
Scopus
NLM Catalog
Ebsco Host - Medline
Google Academic
Semantic Scholar

HIGHLIGHTS

National Awards “Science and Research”

NEW! RJP has announced the annually National Award for "Science and Research" for the best scientific articles published throughout the year in the official journal.

ICMJE- Recommendations

Read the Recommendations for the Conduct, Reporting, Editing, and Publication of Scholarly work in Medical Journals.

Promoting Global Health

The published medical research literature is a global public good. Medical journal editors have a social responsibility to promote global health by publishing, whenever possible, research that furthers health worldwide.

TUMORI ADRENOTESTICULARE LA UN ADOLESCENT CU SINDROM ADRENOGENITAL – REZULTATE DUPA UN AN

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REZUMAT

Introducere. Sindromul adrenogenital este o boală genetică care are ca rezultat producţia scăzută de cortizol şi aldosteron. Pacienţii de sex masculin cu sindrom adrenogenital pot prezenta tumori adrenotesticulare.

Scop. Discutarea caracteristicilor embriologice, clinice şi opţiunilor terapeutice la un adolescent cu sindrom adrenogenital, formă completă.

Prezentarea cazului. Prezentăm un adolescent de 16 ani şi 6 luni cu sindrom adrenogenital, cu pierdere de sare. A fost diagnosticat la 6 săptămâni, când a început tratamentul de substituţie cu hidrocortizon. Pentru adaptarea periodică a tratamentului, pacientul a efectuat evaluări clinice, metabolice şi endocrine complete. Datorită suportului familial, a fost un pacient compliant până la vârsta de 14 ani şi 8 luni, când a început liceul şi s-a mutat de acasă. La vârsta de 15 ani şi 6 luni, clinic, se observă creşterea în volum a testiculelor. Ecografia testiculară confirmă mase tumorale extinse, neomogene, bilaterale. Profilul hormonal relevă ACTH şi 17-OH progesteron crescute. Tratamentul a fost realizat prin înlocuirea dozei de noapte de hidrocortizon cu dexametazonă, pentru a prelungi suprimarea ACTH. După un an de tratament, tumorile testiculare au scăzut substanţial în volum.

Concluzii. Pubertatea are un impact negativ asupra complianţei la tratament, crescând astfel riscul de tumori adrenotesticulare. Acestea nu sunt maligne, dar pot duce la disfuncţie gonadală şi infertilitate. Este important să se efectueze screening-ul acestor tumori la pacienţii cu sindrom adrenogenital.

Cuvinte cheie: sindrom adrenogenital, tumoră adrenotesticulară, adolescent

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