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[Corticosuprarenaloma in children]
Archives Francaises De Pediatrie
|May 1, 1975
Summary
Pediatric corticosurrenaloma, often presenting as virilism, is more common in young girls. Early surgical intervention is recommended, with op
Area of Science:
- Pediatric Endocrinology
- Pediatric Oncology
- Pediatric Surgery
Background:
- Corticosurrenaloma (adrenocortical tumors) in children presents unique challenges.
- Early onset and specific clinical manifestations are observed in pediatric cases.
Purpose of the Study:
- To analyze clinical features, diagnosis, and treatment outcomes of pediatric corticosurrenaloma.
- To identify prognostic factors and evaluate therapeutic strategies.
Main Methods:
- Retrospective study of 34 pediatric corticosurrenaloma cases with onset before age 15.
- Review of clinical presentation, hormonal assays, diagnostic imaging (urography, cavography, arteriography), and histological data.
- Analysis of treatment regimens including surgery, radiation, and op'DDD (mitotane).
Main Results:
- Higher incidence in girls (65%) and young children (80% < 5 years).
- Virilism (83%) is the predominant symptom, often with hypercortisolism or feminization.
- Radiological diagnosis is generally effective; hormonal assays have limited value.
- Prognosis is difficult to evaluate; malignancy suggested by large mass and metastases.
- Surgery is crucial; op'DDD showed potential in managing advanced disease or metastases.
Conclusions:
- Pediatric corticosurrenaloma requires prompt surgical management.
- Early diagnosis and intervention are key, despite diagnostic challenges.
- Op'DDD may offer a therapeutic option for advanced or metastatic disease.