[Immunohistochemical detection of calcitonin, ACTH, beta-MSH, beta-endorphin and somatostatin in medullary carcinoma of the thyroid. Immunoperoxidase study (PAP, ABC) of 10 cases]. / Détection immunocytochimique de calcitonine, d'ACTH, de beta MSH, de beta-endorphine et de somatostatine dans les carcinomes médullaires de la thyroïde. Etude en immunoperoxydase (PAP, ABC) de 10 cas.

Ten cases of Medullary carcinoma of Thyroid gland (MCT) were examined (two familial, eight sporadic) in patients with increased serum calcitonin levels and ranging age from 31 to 87 years. Immunocytochemical reactions were performed using Peroxidase-Antiperoxidase (PAP) complex and/or Avidin-Biotin-Peroxidase complex (ABC) by light microscopy on fixed tumorous specimens (10 cases) and additionally by electron microscopy (3 cases). The tumors displayed light microscopic features typical of MCT. The cells were polyhedral, angular or fusiform and showed high affinity for Grimelius stain. Large deposits of amyloid were Congo red stained and disclosed a green birefringence in polarized light. In the familial cases sections of non-tumorous thyroid tissue showed C cell hyperplasia. Electron microscopic studies revealed intracytoplasmic secretory granules and amyloid fibrils. The immunoperoxidase technique demonstrated calcitonin in the tumor cells in all the cases, with a weaker staining than in para-and intra-follicular C cells of adjacent non tumorous tissue and than in C cells hyperplasia foci, when present. Immunoreactive tumor cells were also observed with anti SRIF in 4 cases, with anti-ACTH and anti-beta MSH in 3 cases, and with anti-beta endorphin in 2 cases. These results are discussed with a review of the literature.

[Plasma tumor markers (calcitonin and carcinoembryonic antigen) in medullary thyroid cancers]. / Les marqueurs tumoraux plasmatiques (calcitonine et antigène carcino-embryonnaire) au cours des cancers médullaires thyroïdiens.

The authors have studied 43 patients operated on for medullary thyroïd carcinoma. Plasma calcitonin was measured regularly in all patients, while carcinoembryonic antigen assay was performed in only 30 patients. Calcitonin assay was found to be useful for preoperative diagnosis of medullary carcinoma, and the level of plasma calcitonin appeared to be roughly correlated with tumor extension. After surgery, simultaneous assay of calcitonin and carcinoembryonic antigen was performed, in order to obtain more accurate information concerning the evolution and prognosis of the disease. In most cases in which no metastatic lymph nodes had been discovered at operation, the level of the two markers rapidly fell to undetectable values. It was observed that in patients with lymph node involvement, cervico-mediastinal radiation treatment did not change the slow and progressive evolution of the disease. However, a rapid increase in titre of carcinoembryonic antigen occurred simultaneously with the discovery of metastases, even when calcitonin levels did not dramatically change.

[Diagnosis of familial medullary cancers]. / Dépistage des cancers médullaires familiaux.

Medullary thyroid carcinoma (MTC) is hereditary in 20 to 25% of cases. It is inherited as an autosomal dominant trait. MTC can be considered as a sporadic form only after a clinical and biological survey of the two parents, siblings and children of the patient, using pentagastrin stimulation test. The authors have studied 36 patients from 26 families. Hereditary MTC with different clinical features, were discovered in two kindreds. The systematic investigation leads to the discovery of 7 cases in the first family, and of 3 in the second. The treatment of the disease at the first stage of its evolution has been possible when an early diagnosis had been made, such as in the second family.

Calcitonin, somatostatin and ACTH immunoreactive cells in a case of familial bilateral thyroid medullary carcinoma.

Bilateral medullary carcinoma of the thyroid (MCT) was discovered in a symptomless patient of a high-risk MCT family. Raised serum calcitonin responding abnormally to pentagastrin led to a thyroidectomy. Grossly, the thyroid showed one nodule in the upper third of each lobe. Light microscopy revealed typical MCT with amyloid deposits. Nontumoral thyroid tissue showed C cell parafollicular hyperplasia. Electron microscopy detected intracellular secretory granules (mean diameter, 150 nm) and typical amyloid fibrills. Immunochemistry revealed numerous calcitonin immunoreactive cells in the nodules, in normal para and intrafollicular C-cells and hyperplastic C-cells. Somatostatin and ACTH were detected in certain tumor cells, but not in normal and hyperplastic C-cells. MCT hormonal production potential is discussed.

[Hypoparathyroidism in adults (author's transl)]. / L'insuffisance parathyroïdienne de l'adulte.

The authors studied the clinical characteristics of primary and post-operative hypoparathyroidism in 39 patients. Laboratory follow-up data were compared under two different treatment programs using either AT 10 or 25 Hydroxycholecalciferol (25 OHCC). Clinical analysis revealed the atypical characteristics of primary hypoparathyroidism. From a therapeutic standpoint, AT 10 and 25 OHCC were equally effective in provoking a return to normal plasma calcium levels, except in complex cases of vitamin D resistance. 25 OHCC proved much easier to manipulate than at 10 and offered a higher security with respect tothe risk of hypercalcemia. The biological activity of 25 OHCC seems to differ from that of AT 10, especially regarding phosphorus metabolism.

[The spine in Recklinghausen's neurofibromatosis]. / Le rachis dans la neurofibromatose de Recklinghausen

The authors have studied the various spinal manifestations of Rechlinghausen's disease. They observed static disorders, such as progressive and non progressive kyphoscoliosis, tumours outside the spinal cord but within the vertebral column of neurinoma type or meningiomas and intraspinal glial tumours. Pseudo-tumoral dystrophic malformations, together with diffuse osteo-dystrophy the skeleton were also observedmthe most interesting and rarest manifestation, as there are to date only 23 published cases, is festooning or vertebral scalloping, which may be demonstrated by tomographic examination and completed by the use of contrast media, e.g. opaque myelography giving the pitted appearance and gas myelography showing the integrity of the spinal cord. The physiopatholoty of the latter form is not clearly established. This may be either of meningeal origin or bony origin, with a dystrophic character.