Medullary carcinoma of the thyroid: Claudius-Regaud center's experience

From June 1971 to September 1989, 38 patients had been treated for medullary carcinoma of the thyroid (MCT). Four patients were excluded from the study, because they were rapidly lost of follow up. We have distinguished three groups: i) group 1 (infra-clinic tumor diagnosed at the time of family investigation): four cases treated by radical thyroidectomy and bilateral cervical evidement; ii) group 2 (bulky cervical tumor with metastatic spread): three patients treated with a palliative intent; iii) group 3 (bulky cervical tumor with metastatic spread): 27 patients treated by thyroidectomy (with cervical lymph mode dissection in 23 cases) and post-operative radiotherapy for 20 patients who had histopathologic invading nodes or a large extracapsular spreading. All patients from the group 1 are alive free of recurrence and the median follow up time is 35 months. In group 2: two patients died from the disease three and 10 months after initial therapy and one patient is alive with disease after 72 months of follow up. In group 3: median follow up time is 79 months. Ten patients (37%) had a local and locoregional failure (isolated nodal recurrence: three patients local and/or nodal relapse with metastatic failure: seven patients). For teen per cent of the irradiated patients relapsed in the target volume. Nine patients (33%) had a metastatic relapse without evidence of cervical recurrence. Median period of metastatic recurrence is 47 months. Specific survival and disease free survival of 65 and 45% at 5 years, 54 and 26% at 10 years respectively. From nine prognosis parameters considered, the post-therapy increasing of tumor markers (thyrocalcitonin and/or CE/A) had significant influence on disease free survival (P<0.02). Histological nodal status had no significant influence on actuarial metastatic rate, but 93% of metastatic patients were N+, and 65% of patients with N+ became M+. This study confirms that metastatic dissemination in patients with nodal invading is a frequent relapse feature. MCT is compatible with a long survival duration and an indolent course. The results of the post-operative irradiation are disappointing