Tumores do córtex adrenal na infância

Abstract

Adrenocortical tumors (ACT) in children are uncommon. However, the incidence of these tumors in Paraná, Brazil, is 15 times higher than that worldwide. We describe the clinical, laboratory and treatment characteristics and outcome of 125 patients treated in a single institution in the State of Paraná. The median age at diagnosis was 4.3 years, with a female:male ratio of 2.6:1. The most common forms of presentation were isolated virilization (51.2%) and virilization and Cushing's syndrome (42%). Nonfunctioning tumors comprised 4.8% of the cases. Two patients (1.6%) had isolated Cushing's syndrome and 1 (0.8%) had Conn's syndrome. Fifty-six percent presented hypertension. Surgery is the only curative treatment. Our data show that disease stage 1, absence of spillage during surgery and absence of intravenous thrombus were associated with better survival rates.

Polycythemia and Adrenal Cortex Tumor in Children

“…Replacement doses of glucocorticoid and mineralocorticoid should be 2 to 3 times the physiological doses. 1,2,5,11 An international study is evaluating the effectiveness of the chemotherapy regimen consisting of etoposide, doxorubicin, and cisplatin, combined with mitotane versus streptozotocin and mitotane as first-line treatment (First International Randomized trial in locally advanced and Metastatic Adrenocortical Carcinoma Treatment). 8 The most singular aspect of this specific case is that the diagnosis of ACT was made during the investigation of polycythemia in a child.…”

“…1 This type of tumor is rare in childhood and adolescence; however, in the southern and southeastern Brazil, especially in the states of Parana and Sao Paulo, the incidence is 10 to 15 times higher than in the rest of the world. 2,3 These states have intense agricultural activity and studies suggest that environmental pollutants like pesticides, may contribute to adrenal tumorigenesis in individuals with R337H, germline mutation in TP53, which is present in most cases of childhood ACT in southeastern Brazil, with a penetrance of approximately 10%. 4,5 The ACT may occur at any age, but a bimodal distribution has been reported, a first peak occurring before the age of 5 and a second between the fourth and fifth decades of life.…”

Tratamento do tumor do córtex adrenal na infância

“…O estadiamento do TCA na infância (tabela 1), a presença de trombo intravenoso, a presença ou não de spillage (ruptura da cápsula do tumor durante a cirurgia ou exposição de trombo vascular tumoral durante a cirurgia) e a retirada completa do tumor e dos trombos são parâmetros importantes para o prognóstico (6).…”

“…O endocrinologista pediátrico deve seguir estes pacientes segundo um protocolo, em virtude dos vários eventos relacionados ao tumor (hipertensão, puberdade precoce, crescimento e estatura final) ou ao tratamento (insuficiência adrenal, por exemplo). Diferente do que acontece nos casos de TCA nos adultos, a maioria dos TCA na infância são funcionantes, sendo a virilização isolada ou associada à síndrome de Cushing as principais formas de apresentação clínica (6,7).…”

“…A terapêutica com maior índice de cura para o TCA na infância é a ressecção completa do tumor, sem ruptura da cápsula tumoral, especialmente nos tumores nos estádios I e II (6). A laparotomia transperitoneal é a via de acesso mais adequada para ressecção completa do tumor, dos linfonodos retroperitoneais regionais e inventário da cavidade abdominal (8).…”