Objective This study aimed to summarize the clinical characteristics, comprehensive treatment, and prognosis of adrenocortical carcinoma (ACC) in children. Methods The clinical data of eight children with definite diagnoses of ACC were retrospectively analyzed, and statistical methods were used to analyze the clinical characteristics, comprehensive treatment mode, and prognosis of these patients. Results (1) Clinical characteristics: two were males and six were females with the median age of onset was six-years old were involved. Four patients had a rash and precocious puberty as the symptoms of onset. European Network for the Study of Adrenal Tumors (ENSAT) staging: stage II, two patients; stage IV, six patients. (2) Comprehensive treatment: all eight patients underwent surgical treatment and received six cycles of chemotherapy: the regimen was "etoposide + pirarubicin + cisplatin + mitotane." (3) Prognosis analysis: among these eight patients, two patients died, two patients achieved complete remission, the disease was stable in four patients, and the overall five-year survival rate was 75%. Prognosis analyzed according to ENSAT staging (stage II versus stage IV) revealed that two-year survival rates of the two groups were 100% versus 65%, respectively, without statistical significant (χ 2 = 1.066, P = 0.302). Prognosis analyzed according to Weiss score (Weiss score was Conclusion The Weiss score is an important prognostic factor for ACC. The chemotherapy regimen "mitotane + etoposide + adriamycin + cisplatin" is recommended.

/pdf/adrenocortical-carcinoma-in-eight-children-a-report-and-4suoh00biz.pdf

Adrenocortical Carcinoma in Eight Children: A Report and Literature Review.

Objective
To analyze the clinical features and prognosis of adrenalcortical carcinoma in children and improve its diagnosis and treatment.


Methods
Medical records were retrospectively analyzed for 16 patients hospitalized from January 2002 to March 2012. And their clinical symptoms, endocrine function, imaging findings, pathological diagnosis and treatment were discussed. And follow-ups were conducted.


Results
All cases were pathologically diagnosed as adrenal cortical carcinoma. There were functional (n=10) and non-functional (n=6) tumors. Complete macroscopic resection of tumor was performed for 12 cases while another, 4 cases had gross residue. The postoperative period was uneventful. Outpatient or telephone follow-ups were conducted for 6-12 years.. All 4 cases of total resection survived with a normal development. One case with severe kidney adhesions underwent nephrectomy with a postoperative survival of 6 months. Three cases with tumor thrombus survived 1, 5 and 12 months respectively. One case on a 4-week chemotherapy died from pulmonary metastasis while another 2 cases died 6 months later from bone marrow suppression and infection due to chemotherapy. One case received a 2-year chemotherapy and died from sudden brain metastases. One case died in a car accident While another 3 cases were lost. The average survival time was 35 months.


Conclusions
Adrenal cortical carcinoma is a rare tumor with a high degree of malignancy and a poor prognosis in children. Surgical excision remains main treatment for this disease. The perioperative application of corticosteroids is absolutely necessary. Laparotomy is preferred over laparoscopic operation. In fact, preoperative and postoperative chemotherapies fail to improve the prognosis.


Key words: 
Adrenalcortical carcinoma; Diagnosis; Treatment

Diagnosis and treatment of adrenal cortical carcinoma in children

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Diagnosis and treatment of adrenal cortical carcinoma in children