Rare childhood tumors

What kind of tumors are these?

Endocrine tumors in children and adolescents represent rare but serious cancerwhose diagnosis and treatment require close interdisciplinary cooperation and experienced specialized teams.

Due to the low incidence, frequent hormonal activity and frequent genetic background of these tumors, it is essential comprehensive approach comprising:

• biochemical and hormonal examinations,
• genetic testing,
• modern imaging methods,
• multidisciplinary clinical trial.

The most common types of tumors

• Neuroblastoma
  The most common solid tumor in childhood (except brain tumors).
  It is often associated with an increase in catecholamine metabolites in the urine.
• Pheochromocytoma and paraganglioma
  They produce catecholamines — they lead to hypertension, sweating, palpitations and other symptoms.
  They often occur in both adrenal glands.
  They are very often genetically determined, which is why it is crucial to carry out genetic testing to determine the next diagnostic and treatment course.
• Adrenocortical carcinoma (ACC)
  A rare malignant tumor of the adrenal glands with possible hormonal activity (cortisol, androgens).
  In children, it can manifest precocious puberty, by virilization or Cushing's syndrome.
  Often associated with genetic syndromes such as Li-Fraumeni (TP53) or Beckwith-Wiedemann.

The Importance of Genetic Testing

In pediatric patients it is recommended to conduct a genetic examination in all cases of pheochromocytoma, paraganglioma and ACC, in accordance with international recommendations. The results can fundamentally affect the further diagnostic and therapeutic procedure, including monitoring family members.

Typical symptoms

Clinical manifestations vary according to tumor type and hormonal activity. Among the most common are:

• high blood pressure,
• headache, sweating, palpitations,
• signs of virilization or early puberty,
• weight loss, fatigue, behavioral changes,
• symptoms of Cushing's syndrome.

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