What symptoms would adrenal tumors present with in children?

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Simptomi tumora nadbubrežne žlezde kod dece

Tumori nadbubrežne žlezde kod dece najčešće se manifestuju znacima viška hormona, pri čemu su virilizacija i Cushingov sindrom dominantni simptomi, dok se manji broj slučajeva prezentuje simptomima povezanim sa masom tumora ili hipertenzijom usled kateholamina.

Simptomi prema tipu tumora

Adrenokortikalni tumori (najčešći)

Hormonski aktivni tumori (60-70% slučajeva):

Kod prepubertetskih devojčica:

  • Pubarha/prekomerna dlakavost (68%) 1
  • Hipertrofija klitorisa (49%) 1
  • Povećanje telesne težine (32%) 1
  • Akne 1, 2

Kod prepubertetskih dečaka:

  • Pubarha (56%) 1
  • Povećanje penisa (47%) 1
  • Akne (32%) 1
  • Znaci prevremenog puberteta 3

Kod pubertetskih pacijenata:

  • Simptomi viška androgena često se ne prepoznaju jer se pripisuju normalnom pubertetu 1
  • Cushingov sindrom postaje očigledniji: cushingoidni izgled lica, centralna gojaznost, hipertenzija (36%), strije 4, 1
  • Kod devojčica: oligomenoreja/amenoreja 4
  • Kod dečaka: ginekomastija (ako tumor luči estrogen) 4

Cushingov sindrom (kada je prisutan):

  • Povećanje telesne težine
  • Slabost (posebno proksimalnih mišića)
  • Hipertenzija
  • Psihijatrijski poremećaji
  • Hirzutizam
  • Centralna gojaznost
  • Purpurne strije
  • "Buffalo hump" (nakupljanje masti na vratu)
  • Hiperglikemija i hipokalemija 4

Hormonski neaktivni tumori (30-40% slučajeva):

  • Abdominalni bol
  • Bol u leđima
  • Rano zasićenje
  • Gubitak težine
  • Palpabilna masa 4, 5

Feohromocitom/Paragangliom

Simptomi viška kateholamina:

  • Hipertenzija (može biti paroksizmalna)
  • Glavobolja
  • Znojenje (dijaforeza)
  • Palpitacije
  • Tahikardija 6, 7

Napomena: Paragangliomi u bazi lobanje i vratu obično ne luče kateholamine i manifestuju se efektom mase (disfagija, gubitak sluha, dizartrija) 7.

Ključne karakteristike po uzrastu

  • Najčešći uzrast: Pre 5. godine života, sa vrhom u ranom detinjstvu 8, 5
  • Polna distribucija: Češće kod devojčica (odnos 1.5:1) 8
  • Trajanje simptoma: Medijana 4-6 meseci pre dijagnoze 1, 2, mada neki slučajevi mogu imati produženo trajanje 9

Važna upozorenja

Genetski sindromi: Tumori nadbubrežne žlezde kod dece često su deo naslednih sindroma:

  • Li-Fraumeni sindrom (TP53 mutacije) 8, 2, 3
  • Beckwith-Wiedemann sindrom 8, 5
  • MEN 1 8
  • Von Hippel-Lindau (za feohromocitom) 7
  • SDHx mutacije (za paragangliome) 7

Deca sa genetskim defektima se prezentuju u mlađem uzrastu sa većim tumorima 2.

Klinička zamka: Kod pubertetskih pacijenata, simptomi virilizacije se često ne prepoznaju jer se pripisuju normalnom pubertetu, što dovodi do kašnjenja u dijagnozi 1. Cushingov sindrom je očigledniji kod starije dece i treba ga aktivno tražiti.

Urgentna evaluacija: Deca mlađa od 40 godina sa adrenalnom masom zahtevaju hitnu procenu, jer imaju veći rizik od maligniteta 10.

Dijagnostički pristup

Kada se sumnja na tumor nadbubrežne žlezde:

  1. Hormonska evaluacija (obavezna za sve slučajeve):

    • Glukokortikoidi: deksametazonski supresioni test, slobodni kortizol u urinu, bazalni kortizol i ACTH 8
    • Polni steroidi i prekursori: DHEA-S, 17-OH-progesteron, androstenedion, testosteron 8
    • Kateholamini: plazma metanefrini i normetanefrini (za feohromocitom) 6

  2. Imaging: CT ili MRI abdomena 8

  3. Genetsko testiranje: Preporučuje se kod sve dece sa tumorima nadbubrežne žlezde 2, 3

References

1
Research

The Endocrine Phenotype Induced by Pediatric Adrenocortical Tumors Is Age- and Sex-Dependent.

The Journal of clinical endocrinology and metabolism, 2024

2
Research

Clinical Characteristics and Long-Term Outcomes of Adrenal Tumors in Children and Adolescents.

Experimental and clinical endocrinology & diabetes : official journal, German Society of Endocrinology [and] German Diabetes Association, 2023

4
Guideline

neuroendocrine tumors.

Journal of the National Comprehensive Cancer Network : JNCCN, 2012

5
Research

Adrenocortical neoplasms in children: radiologic-pathologic correlation.

Radiographics : a review publication of the Radiological Society of North America, Inc, 1999

6
Guideline

pheochromocytoma and paraganglioma: an endocrine society clinical practice guideline.

The Journal of clinical endocrinology and metabolism, 2014

10
Guideline

imaging of adrenal masses.

Endocrine practice : official journal of the American College of Endocrinology and the American Association of Clinical Endocrinologist, 2025

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