Background: Malignant endocrine tumours (MET) are rare neoplasms in childhood and data on such tumours are scarcely available. The aim of this retrospective study was to collect data over a period of 11 years (1984-1995) and to give the basis for a prospective study.
Patients and method: 180 departments of paediatrics, nuclear medicine and children's surgery were asked for reporting of patients with MET by a questionnaire. 35% of the departments answered (however, 75% of the paediatric departments).
Results: 96 children with MET were reported: 73 with thyroid cancer (50 papillary, 15 follicular, 7 medullar, 1 anaplastic; 1:1,9 boys to girls, both mean and median age 11 3/4 years), 12 with adrenocortical cancer and 11 with other malignant endocrine tumours. Metastases were found at diagnosis in 41 of 65 children with papillary or follicular carcinoma. 37 patients (pts.) are in first continuous complete remission (CCR), 4 in partial remission (PR) and in 24 (!) children the remission state or the outcome is not known. 6 of 7 children with medullary cancer have had metastases at diagnosis. 1 patient is in CCR, 1 patient is living in PR, 1 in relapse. 4 children are lost of follow up (lfu.). In sex-ratio no difference was found in 12 pts. suffering from adrenocortical cancer (mean age 5 1/4 years, median age 2 1/2 years). 11 tumours showed hormonal activity. 5 children disclosed metastases at diagnosis. All patients were treated by surgery, 6 received chemotherapy additionally. 4 children are living in CCR, 3 pts. in remission of unknown state, 4 died (all of them with metastases at diagnosis), 1 patient is lfu. The other MET reported: 8 carcinoids (7 x appendix, 1 x lung), 2 phaeochromocytomas, 1 islet cell cancer. 8 pts. are in CCR, 2 are lfu. The child with the islet cell carcinoma died.
Conclusions: Since only 35% of the clinics answered this retrospective analysis cannot give any statement about the incidence of MET in children. As to the prognosis thyroid cancer seems to have a favourable prognosis in childhood and adolescence. In contrast, metastatic adrenocortical cancer is incurable in this age group. Carcinoids of the appendix can be treated curatively by appendectomy. To better understand the biology of MET in children and adolescence and to achieve a better prognosis for some types of these tumours, much more data are needed. For these reason a multicenter prospective therapy study for childhood MET seems to be necessary.