[Hereditary thyroid cancer can be cured by prophylactic surgery]. / Arftlig tyreoideacancer kan botas med profylaktisk kirurgi.

Multiple endocrine neoplasia type 2 (MEN 2) is a rare syndrome in which the consequences for the patient and family members are considerable. Mutation analysis of the RET proto-oncogene is crucial for decision-making regarding each patient. Today, carriers of MEN 2 mutations should be offered prophylactic thyroidectomy with the potential to eliminate the risk for potentially lethal medullary thyroid carcinoma (MTC). Here, we present the first Swedish experience of such operations performed mainly on the basis of genetic analysis. Twenty patients underwent total thyroidectomy at a mean age of 13.5 (6-43) years. In all cases, either manifest MTC (n = 11) or C-cell hyperplasia was found. So far, no patient has any sign of recurrence or developmental insufficiency at 1-5 years follow-up. As the medical and ethical problems in this group of patients are substantial, and as the operations are performed in otherwise healthy children, they should be treated at centers with adequate multidisciplinary expertise and competence.

Current trends in the surgical treatment of solitary parathyroid adenoma. A questionnaire study from 53 surgical departments in 14 countries.

Current trends in the surgical treatment of primary hyperparathyroidism due to solitary parathyroid adenoma were evaluated in a questionnaire study. Response was obtained from 53 departments highly active in endocrine surgery in 14 countries (response rate 95%). Intraoperative histologic examination is widely (70%) regarded as necessary, but utilized more in North America and Scandinavia (87%) than elsewhere (45%). Intraoperative fat staining is used in one-third of the departments. Excisional biopsy including one whole gland is preferred by 32% to multiple incisional biopsies. Gross inspection of three normal parathyroid glands without histologic identification is favoured in 21% of the departments, while 31% prefer bilateral exploration and incisional biopsy. When an adenoma is found on the first side to be explored, 17% advocate bilateral exploration with incisional biopsy of all three normal glands, whereas unilateral exploration with excisional or incisional biopsy of the ipsilateral normal gland is preferred by a similar percentage.

[Unilateral parathyroidectomy--a new surgical strategy in hyperparathyroidism due to solitary adenoma].

Unilateral parathyroidectomy (UPTX) was applied as surgical principle for parathyroid adenoma 102 consecutive patients. Intraoperative oil-red-O staining was used for distinction between autonomous and suppressed chief cells. UPTX without contralateral exploration was achieved in 43 patients. In 45 patients two microscopically normal parathyroids were found at the first side and UPTX was performed contralaterally where the adenoma was located. In 14 patients non-UPTX was performed. If more than one, macroscopically, normal parathyroid was found one was removed for histopathology. The intended gland identification was achieved in 93-97%. Supernumerary glands were found in seven patients. Postoperative hypocalcemia was more pronounced after "atypical" operations than UPTX following bilateral exploration. All patients were followed up at least one year. None has developed hypercalcemia. Vitamin D-requiring hypocalcemia is present in two "atypically" operated patients. Removal of one, macroscopically, normal gland, preferably from the adenoma side is advocated. If UPTX can be performed at the first side explored, the contralateral need no exploration.