Abstract
Ninety-two patients from 12 kindreds with hereditary medullary thyroid carcinoma (MTC) were evaluated. We sought to determine if the stimulated plasma calcitonin (CT) level at the time of diagnosis was of prognostic significance. The patients were divided into four groups according to their preoperative stimulated plasma CT levels (1) 250-1,000 pg/ml (n=25); (2) 1,000-5,000 pg/ml (n=36); (3) 5,000-10,000 pg/ml (n=8); (4) greater than 10,000 pg/ml (n=23). Compared between the four groups were several parameters, including incidence of regional lymph node metastases, incidence of residual MTC post-thyroidectomy (as indicated by increased (greater than 300 pg/ml) plasma CT levels after operation), incidence of distant metastases, and incidence of death. Also compared were the incidences of microscopic or gross MTC in thyroidectomy specimens. The incidence of regional lymph node involvement ranged from a minimum of one (4%) of 25 patients in Group 1 to 13 (57%) of 23 patients in Group 4. Similarly, plasma CT levels were elevated in only one (4%) of 25 patients in Group 1 compared to 14 (61%) of 23 patients in Group 4. There was no evidence of distant metastases or death in the patients in Groups 1, 2, or 3. In the 23 patients in group 4, however, four (17.4%) had distant metastases and two (8.7%) died of disease during the period of observation. Of th 25 patients in Group 1, MTC was evident only by microscopic examination in 14 (56%). Eleven (44%) of the patients in Group 1 had macroscopically evident medullary thyroid carcinoma. This is in contrast with patients in Group 4 where all 23 had grossly evident MTC. These data indicate that the stimulated plasma CT level at the time of diagnosis is an excellent prognostic indicator of the extent of a disease in patients with hereditary MTC. Aggressive screening of kindred members at risk is of critical importance for establishing the diagnosis and instituting therapy at a time when the neoplasm is confined to the thyroid gland.
Full text
PDF
Selected References
These references are in PubMed. This may not be the complete list of references from this article.
- Graze K., Spiler I. J., Tashjian A. H., Jr, Melvin K. E., Cervi-Skinner S., Gagel R. F., Miller H. H., Wolfe H. J., DeLellis R. A., Leape L. Natural history of familial medullary thyroid carcinoma: effect of a program for early diagnosis. N Engl J Med. 1978 Nov 2;299(18):980–985. doi: 10.1056/NEJM197811022991804. [DOI] [PubMed] [Google Scholar]
- HAZARD J. B., HAWK W. A., CRILE G., Jr Medullary (solid) carcinoma of the thyroid; a clinicopathologic entity. J Clin Endocrinol Metab. 1959 Jan;19(1):152–161. doi: 10.1210/jcem-19-1-152. [DOI] [PubMed] [Google Scholar]
- Hennessy J. F., Wells S. A., Jr, Ontjes D. A., Cooper C. W. A comparison of pentagastrin injection and calcium infusion as provocative agents for the detection of medullary carcinoma of the thyroid. J Clin Endocrinol Metab. 1974 Sep;39(3):487–495. doi: 10.1210/jcem-39-3-487. [DOI] [PubMed] [Google Scholar]
- Mendelsohn G., Eggleston J. C., Weisburger W. R., Gann D. S., Baylin S. B. Calcitonin and histaminase in C-cell hyperplasia and medullary thyroid carcinoma. A light microscopic and immunohistochemical study. Am J Pathol. 1978 Jul;92(1):35–52. [PMC free article] [PubMed] [Google Scholar]
- Norton J. A., Froome L. C., Farrell R. E., Wells S. A., Jr Multiple endocrine neoplasia type IIb: the most aggressive form of medullary thyroid carcinoma. Surg Clin North Am. 1979 Feb;59(1):109–118. doi: 10.1016/s0039-6109(16)41737-8. [DOI] [PubMed] [Google Scholar]
- Tashijan A. H., Jr, Howland B. G., Melvin K. E., Hill C. S., Jr Immunoassay of human calcitonin. N Engl J Med. 1970 Oct 22;283(17):890–895. doi: 10.1056/NEJM197010222831702. [DOI] [PubMed] [Google Scholar]
- Wells S. A., Jr, Baylin S. B., Gann D. S., Farrell R. E., Dilley W. G., Preissig S. H., Linehan W. M., Cooper C. W. Medullary thyroid carcinoma: relationship of method of diagnosis to pathologic staging. Ann Surg. 1978 Sep;188(3):377–383. doi: 10.1097/00000658-197809000-00013. [DOI] [PMC free article] [PubMed] [Google Scholar]
- Wells S. A., Jr, Baylin S. B., Linehan W. M., Farrell R. E., Cox E. B., Cooper C. W. Provocative agents and the diagnosis of medullary carcinoma of the thyroid gland. Ann Surg. 1978 Aug;188(2):139–141. doi: 10.1097/00000658-197808000-00002. [DOI] [PMC free article] [PubMed] [Google Scholar]