Etude comparative des epithéliomas médullaires du corps thyroïde avec et sans stroma amyloïde

Abstract

Besides the medullary cancer with an amyloid stroma of the thyroid which is a well known entity there are other histopathological aspects of medullary cancer without amyloid stroma which have been relatively little studied. There can be confusion from histopathological view-point because the amyloid stroma can be present in the primitive tumour and absent in metastases or vice versa. To find out whether these two pathological aspects correspond to one or two morbid entities, the clinical and biological aspects of 24 medullary cancers with amyloid and 10 without amyloid which were observed at the Gustave Roussy Institute between 1933 and 1968 have been compared. The study of the medullary carcinomas with amyloid stroma shows that the tumour mass as well as the lymph nodes are tender and that the diffuse adenopathy is often very difficult to delineate. It confirms the familial character of the disease (we have the case of a family in which this kind of cancer appears in three generations), as well as the existence of classical associations, especially a diarrhoea which fluctates with the disease, multiple neuromas of the mucous membranes and malformations of the amyothropic type. The clinical characters of medullary cancers without amyloid stroma are different. They predominate in males. Tumours and lymph nodes are not tender. No associated signs have been observed. The lymph nodes and distant metastases are similar to those of other thyroid cancers. From a biological view-point we observed the presence of thryocalcitonine in the tumour or the plasma of all patients with medullary cancers with amyloid stroma in which it was looked for. In a case of medullary cancer without amyloid stroma there was no thyrocalcitonine in the plasma. The survival time in cancers with amyloid stroma is similar of our series with that of other epitheliomas of the thyroid. The survival in medullary cancers without amyloid stroma is much shorter, approaching that of the anaplastic cancers of the thyroid. Consequently medullary carcinomas with and without amyloid stroma must be considered as separate entities since they differ clinically, biologically and in regard to prognosis.