Long-term prognosis of medullary thyroid carcinoma.

OBJECTIVE: The clinical course of patients with medullary thyroid carcinoma (MTC) is variable, even in the subgroup of patients after surgery with curative intent and postoperatively persistent elevated calcitonin levels. This study aimed to evaluate the long-term prognosis of survival in patients with MTC. PATIENTS: Long-term survival was analysed in 32 patients with MTC being treated in an endocrine centre over a 40-year period. Patients were classified as having sporadic MTC, familial MTC (FMTC), multiple endocrine neoplasia (MEN) IIA or MEN IIB. RESULTS: Seventeen patients had sporadic MTC (53.1%), eight had MEN IIA (25%) and three had MEN IIB (9.4%); the remaining four patients (12.5%) had not undergone genetic analysis until now. The overall average age at diagnosis was 42.0 years, and the median follow-up time was 9.5 years (range 0.5-39 years). Mortality due to progressive MTC was 15.6%. The 5-year survival rate was 96% (95% CI 89-100), the 10-year survival rate 91% (95% CI 79-100), and the 15-year survival rate 85% (95% CI 78-100). The estimated mean survival time after initial diagnosis was 31 years (95% CI 26.7-37.0). There is a significant difference in survival time between patients achieving complete remission compared with patients with biochemical persistent disease (P = 0.038) or metastasis (P = 0.0003). In five patients, advanced imaging with positron emission tomography/computed tomography (PET/CT) identified additional sites of tumour load. Eight more lymph node metastases were found in four patients and one local tumour recurrence in one patient by PET/CT. CONCLUSION: The overall prognosis of MTC is favourable, even if the rate of biochemical cure is lower in MTC than in differentiated types of thyroid cancer. This is also true for patients with biochemically persistent disease. Whether the identification of further tumour sites by advanced imaging procedures such as PET/CT translates into a better prognosis in patients with persistently elevated calcitonin levels remains to be investigated.

Malignant glioma as a secondary malignant neoplasm after radiation therapy for craniopharyngioma: report of a case and review of reported cases.

BACKGROUND: The development of a secondary neoplasm in childhood cancer survivors attains growing importance due to the reported excellent survival and therefore the long exposure to potentially carcinogenic effects of treatment. CASE REPORT: We report a 14-year-old girl in whom a large craniopharyngioma (CP) was diagnosed. After surgery, radiation therapy (RT) was given for residual tumour. Discrete progression necessitated further surgery, resulting in permanent tumour control. Soon after the second surgery hypothalamic-pituitary dysfunction developed together with obesity. Supportive hormone therapy was initiated. Growth hormone (GH) therapy was also given for 15 months. Four years after the diagnosis, a cerebropontine anaplastic astrocytoma WHO grade III was detected, with the main lesion being at the dorsal edge of the irradiated area. The girl died 1 month later from this secondary presumably radiation-induced tumour. Only recently a second child with RT for a CP was diagnosed with malignant glioma in our hospital. CASE REPORTS IN THE LITERATURE: 12 other cases of malignant glioma have been reported after RT for CP. Including our present cases, the mean latency period was 10.7 years (median 9.6 years). However, the shortest latency periods were found in patients who had received GH therapy. In numerous cases, the secondary tumour was seen at the edge of the irradiated volume, and not in the region with the highest absorbed dose. CONCLUSIONS: Therapy-induced secondary gliomas after treatment of CP or other intracranial tumours are rare but dramatic late events with a very poor prognosis. Including our own 2 patients, we reviewed 14 cases of CP with occurrence of a secondary, probably radiation-induced malignant glioma. The short latency periods for patients treated with GH is remarkable. We therefore suspect that GH therapy may accelerate the development of a secondary brain tumour. We are reluctant to recommend GH therapy in conventionally irradiated CP patients. In order to seriously answer the questions about therapy-induced secondary neoplasms, a life-long follow-up is mandatory for all patients who are survivors of childhood cancer.

Recurrence following transsphenoidal surgery for acromegaly.

The long-term results of transsphenoidal adenomectomy and the incidence of possible recurrences were studied in 61 patients who had normal basal serum growth hormone (GH) levels 1 week after surgery. The patients were followed up for an average of 6.0 years (range: 1.5-14.0 years) by repeated measurements of GH, oral glucose tolerance testing (OGTT) and at the last follow-up visit also by assaying somatomedin C levels. In 4 of the patients, the basal GH levels had increased to values above 5 ng/ml. In 43 patients, a normal suppression of GH during an oral glucose load was observed shortly after surgery. In only 2 of these cases did a transiently inadequate suppression develop during the follow-up period, although clinical acromegaly did not recur and the somatomedin C levels remained normal. It is concluded that recurrence of active acromegaly is unlikely to occur in patients who achieve a normal glucose-induced suppression of GH levels shortly after adenomectomy. As such, an OGTT provides better prognostic information than basal human GH level measurements and may give a clearer and earlier indication of surgical success.

[Therapy of thyroid cysts with fine needle aspiration]. / Therapie von Strumazysten durch Feinnadelaspiration.

In a study extending over a period of 4 years, therapeutic fine-needle aspirations were carried out in 194 thyroid patients with thyroid cysts. The cyst contents of 3-130 ml cyst fluid were completely removed by suction in 2-3 month intervals by 1-17 fine-needle aspirations on an outpatient basis. Malignancy was ruled out in all patients by repeated cytological tests. Complete emptying of the cyst, verified by twice-repeated check at 3-month intervals, was achieved in 86 patients (44.3%). A reduction of the cyst fluid to less than 30% was achieved in 13 patients (6.7%). In 17 patients (8.8%) the cyst fluid diminished to 31-50%. In 44 patients (22.7%) there was no diminution in the cyst fluid or a reduction of less than 50%. There was an increase in cyst fluid compared with the first puncture in 34 patients (17.5%). The success of puncture cannot be predicted, although cysts with brown cyst fluids can be emptied significantly more frequently than cysts with yellow cyst fluids (63% as compared with 43.5%, p less than 0.005). Cyst fluid volume, T3- and T4-contents of the cyst fluid, age and sex had no influence on therapeutic effect. Up to the 5th puncture the prospects of success are approximately constant, but even in cases of more frequent punctures emptying still takes place. Thyroid hormone therapy with 50-100 micrograms 1-T4 daily (n = 134), and a single intracystic injection of 40 mg methylprednisolone-crystal suspension (n = 42) or of 0.02 g hydroxypolyaethoxydodecan 1% (n = 11) did not show better results than the single aspiration. There were no complications except for 2 cases of afterbleeding in the cysts immediately after puncture, and these were easily dealt with. Fine-needle aspiration is a simple, effective and cost-saving method for the elimination of the frequent cosmetically disturbing thyroid cysts in half of patients, thus avoiding surgery.