MALIGNANT ENDOCRINE TUMORS IN CHILDHOOD AND ADOLESCENCE - RESULTS OF A RETROSPECTIVE ANALYSIS

Background Malignant endocrine tumours (MET) are rare neoplasms in chi ldhood and data on such tumours are scarcely available. The aim of thi s retrospective study was to collect data over a period of 11 years (1 984-1995) 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 questionna ire. 35% of the departments answered (however, 75% of the paediatric d epartments). Results 96 children with MET were reported: 73 with thyro id 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 adre nocortical cancer and 11 with other malignant endocrine tumours. Metas tases were found at diagnosis in 41 of 65 children with papillary or f ollicular carcinoma. 37 patients (pts.) are in first continuous comple te 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 CC R, 1 patient is living in PR, 1 in relapse. 4 children are lost of fol low up (lfu.). In sex-ratio no difference was found in 12 pts. sufferi ng from adrenocortical cancer (mean age 5 1/4, years, median age 2 1/2 , years). 11 tumours showed hormonal activity. 5 children disclosed me tastases at diagnosis. All patients were treated by surgery, 6 receive d chemotherapy additionally. 4 children are living in CCR, 3 pts. in r emission of unknown state, 4 died (all of them with metastases at diag nosis), 1 patient is lfu. The other MET reported: 8 carcinoids (7 x ap pendix, 1 x lung), 2 phaeochromocytomas, 1 islet cell cancer. 8 pts. a re in CCR, 2 are lfu. The child with the islet cell carcinoma died. Co nclusions Since only 35% of the clinics answered this retrospective an alysis cannot give any statement about the incidence of MET in childre n. As to the prognosis thyroid cancer seems to have a favourable progn osis in childhood and adolescence. In contrast, metastatic adrenocorti cal cancer is incurable in this age group. Carcinoids of the appendix can be treated curatively by appendectomy. To better understand the bi ology of MET in children and adolescence and to achieve a better progn osis for some types of these tumours, much more data ape needed. For t hese reason a multicenter prospective therapy study for childhood MET seems to be necessary.