A family in which several members have medullary carcinoma and phaeochromocytoma is described. Four of the 5 patients in this family who had a phaeochromocytoma have died; in one of these the medullary carcinoma may have contributed to death but in general the thyroid cancers were slow growing. In our experience successful diagnosis and treatment of the phaeochromocytomas have presented the greatest challenge. The welfare of future generations with this syndrome would seem to depend upon regular clinical and biochemical surveillance of the individuals and expeditious diagnosis and treatment of their tumours, particularly the phaeochromocytomas.

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http://dx.doi.org/10.1002/bjs.1800620404DOI Listing

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