J.Jpn. Surg. Soc.. 79(3): 177-187, 1978
Original article
CLINICOPATHOLOGIC AND ENDOCRINOLOGICAL STUDIES ON 33 CASES OF MEDULLARY CARCINOMA OF THE THYROID
Part 1 . Clinical features and endocrinological findings
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The Second Department of Surgery, Osaka University Medical School (Director: Prof. G. Kosaki) Akira Miyauchi |
By reviewing microscopic preparations, 24 medullary carcinomas were found in 1610 thyroid maligancies operated at Kuma Hospital during 16 years from 1961 to 1976, and 4 in 131 at the 2nd Department of Surgery, Osaka University Medical School during 5 years from 1972 to 1976, i.e., 1.5% and 3.0% of all thyroid malignancies respectively.
Female to male ratio was 27 : 6, and the average age was 36.0 years (range : 13-58). Of these 33 cases, 15 were familial cases including 6 of multiple endocrine neoplasia type 2A, and 18 were sporadic cases including 2 with ectopic ACTH syndrome. The average age of the familial and the sporadic group were 30.1 and 40.9 years respectively.
In the familial group, 5-year survival was excellent (100%), despite of frequent recurrence (60%) in the residual thyroid and neck nodes. Many cases survived for a long time even with the disease, suggesting the less aggresive nature of the neoplasia of this group. All but 1 patient had bilateral thyroid neoplasia ; the remaining patient had medullary carcinoma in one lobe and C cell hyperplasia in the other. Postoperative plasma calcitonin levels were normal only in the patients having total thyroidectomy.
In the sporadic group, the tumor occurred unilaterally in most cases, and about a half of them were completely removed by hemithyroidectomy or enucleation shown as normal postoperative calcitonin levels. The recurrence rate was low (39%), but 5-year survival was worse (71%) than that of the familial goup, because of a few cases of high grade malignancy.
Total thyroidectomy with modified radical neck dissection is recommended for familial medullary carcinoma. In the sporadic cases, the surgical spproach similar to that for papillary carcinoma of the thyroid is considered to be sufficient.
The clues of correct diagnoses were familial occurrence, presence of pheochromocytoma or parathyroid adenopathy, and characteristic symptoms such as intractable diarrea, flushing and palpitation. One of other diagnostic signs was small punctate calcification scattered in the tumor on X ray film. Bilateral or adenoma-like nodules found in upper and middle third of lateral lobes in association with marked cervical lymph node metastases should be suspected of medullary carcinoma.
Plasma calcitonin or carcinoembryonic antigen measurements were valuable aid for preoperative diagnosis, follow-up of postoperative patients and screening of high risk family members.
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