Combinations of 5-fluorouracil (5-FU) and streptozocin and 5-FU and dacarbazine were given alternately to 20 patients with metastatic medullary thyroid carcinoma. Three partial responses and 11 long-term stabilizations were observed. No unexpected toxicity occurred.

Summary Selective venous sampling catheterisation was performed in 19 patients with medullary thyroid carcinoma without known distant metastases for persistent hypercalcitoninaemia after surgery. Calcitonin (CT) gradients were found in the neck and/or the mediastinum in 18 patients and in five patients at distant sites also. After venous catheterisation, 13 patients were subjected to repeat surgery. Neck and/or mediastinal tumour foci were found in 12 patients at the sites of the CT gradients. Of Medullary thyroid carcinoma (MTC) spreads early to regional lymph nodes in the neck and mediastinum and to distant sites in the liver, lungs and bones (Grauer et al., 1990).The aim of initial surgery is to remove all neoplastic foci. It consists of a total thyroidectomy with bilateral lymph node dissection in the neck and the upper mediastinum (Wahl & Roher, 1988). The normalisation of the serum calcitonin (CT) level after surgery is a strong indicator that neoplastic tissue has been totally removed. However, this is achieved in only 20% of patients with clinical disease (Parmentier et al., 1985). In others, persistently elevated CT levels indicate the presence of residual disease. If localised, this may warrant further surgery. However, a complete work-up, including ultrasonography, computerised tomography or magnetic resonance imaging and bone scintigraphy, frequently yields no positive evidence of localised tumours in these patients with elevated CT levels. Furthermore, owing to previous surgical procedures, the significance of any abnormality may be ambiguous. Scintigraphic procedures have not proved to be sensitive enough to be useful as a routine.Selective venous sampling catheterisation appeared to be a sensitive and specific tool for localising the origin of serum CT in patients with elevated CT levels and no other evidence of disease (Ben Mrad et al., 1989;Gautvik et al., 1989; Frank-Raue et al., 1992). However, given the slow growth rate of most MTCs, the follow-up was too short in these series to elucidate the significance of extracervical gradients and to conclude that this technique could be useful, in terms of relapse and survival rates.The present study is based on 19 patients, with a mean follow-up of 5.5 years after selective venous catheterisation. Eight of these patients have previously been reported (Ben Mrad et al., 1989).Before selective venous catheterisation, all patients were subjected to a clinical examination, including chest radiography, ultrasonography of the neck and liver, computerised tomography of the neck, chest and abdomen and bone scintigraphy. This work-up only permitted the discovery of involved cervical or mediastinal lymph nodes in six patients (nos. 1, 4, 6, 13, 18 and 19).The selective venous sampling catheterisation procedure has already been reported (Ben Mrad et al., 1989). It was performed by the femoral route using a Cook SF 2-cm-long catheter with a 1200 angle and one side-hole tip (Cook, SARL, Paris, France). A standard procedure was used: a mean of 25 samples was obt...

Ectopic ACTH secretion is a rare cause of hypercortisolism. Induced metabolic disturbances are often serious, and the management of such patients may be difficult. We report here our experience with four medullary thyroid carcinoma (MTC) patients with distant metastases in whom an ectopic ACTH syndrome occurred. The clinical presentation was significant by the severity and the rapidity of the hypercortisolism. Diagnosis and follow-up were realized by measurements of plasma cortisol, urinary free cortisol, urinary 17-hydroxycorticosteroid, plasma ACTH, plasma LPH, serum calcitonin and carcino-embryonic antigen. Initial treatment with adrenalytic medical therapy failed to control the disease. Only bilateral adrenalectomy cured the excessive cortisol production, and for a long time despite tumor progression. In conclusion, bilateral adrenalectomy should be considered in MTC patients with Cushing's syndrome even at the stage of distant spread. In fact, with regard to the slow growth rate of most MTC's, they may survive for years.

Neurological endemic cretinism is highly prevalent in severe endemic goiter areas. Often associated to euthyroid goiter, it is probably related to iodine deficiency. However the exact pathogenetic mechanism is yet unclear. We report the biochemical study of thyroid tissue obtained from a 26 year-old female cretin with a grade III multinodular goiter, neurological signs and euthyroidism. After surgery, thyroid tissue was analysed: iodoproteins where characterized by gel filtration, electrophoresis, sedimentation coefficient and antigenicity. Iodoalbumin was predominant while thyroglobulin was quantitatively reduced and poorly iodinated. In vitro, iodination with hog thyroid peroxidase was normal. There was no difference in peroxidase affinity for iodide in the oxidation reaction but a significantly reduced ability to iodinate in vitro thyroglobulin and free tyrosine. Oxidation of acetyltyrosilamide into bityrosine was also markedly reduced. These abnormal findings are known to occur in sporadic cases with or without hypothyroidism. The neurological defects could be linked to transient hypothyroidism during the critical period of nervous system maturation, however a role of iodine deficiency per se cannot be ruled out.