Transsphenoidal surgery in Cushing disease: 10 years of experience in 34 consecutive cases.

OBJECT: Cushing disease is a rare disorder. Because of their small size the adrenocorticotropic hormone (ACTH)-producing tumors are often not detectable on neuroimaging studies. To obtain a cure with transsphenoidal surgery (TSS) may therefore be difficult. In this report the authors present 10 years of experience in the treatment of patients with Cushing disease who were followed up with the same protocol and treated by the same surgeon. METHODS: Thirty-four patients, 26 of them female and eight of them male (mean age 40 years, range 13-74 years) were studied. All had obvious clinical signs and symptoms of Cushing syndrome. Magnetic resonance (MR) imaging was performed in all patients, and inferior petrosal sinus (IPS) sampling was done in 14. In 12 patients MR imaging indicated a pituitary tumor; 10 were microadenomas and two were macroadenomas. In six patients with no visible tumor, the results of IPS sampling supported the diagnosis. All patients underwent TSS; the mean follow-up duration was 6 +/- 0.5 years. Selective adenomectomy was performed in 32 and hemihypophysectomy in the other two patients. A cure was obtained in 31 patients (91%) after one TSS and in two more patients after further TSS; one patient was not cured despite two TSSs and one underwent bilateral adrenalectomy. Disease recurrence was seen in two patients after 3 years, and they were successfully treated with stereotactic gamma knife surgery. Half of the patients had an ACTH deficiency postoperatively, whereas one third had other pituitary hormone insufficiencies. There were no serious complications attributable to the surgical intervention. CONCLUSIONS: Transsphenoidal surgery with selective adenomectomy is an effective and safe treatment for Cushing disease. In the patients presented in this study, the surgical outcome seemed to depend on careful preoperative evaluation and the surgeon's experience. For optimal results in this rare disease the authors therefore suggest that the endocrinological, radiological, and surgical procedures be coordinated in a specialized center.

Galanin in pituitary adenomas.

Tumor galanin content was measured in extracts from human pituitary adenomas using a specific RIA method for monitoring human galanin. Twenty-two out of twenty-four tumors contained galanin with notably high levels in corticotroph adenomas, varying levels in clinically inactive tumors, and low levels in GH secreting adenomas. Tumor galanin and ACTH contents were closely correlated in all tumors. In four young patients with microadenomas and highly active Mb Cushing tumor galanin was inversely related to tumor volume. The molecular form of tumor galanin, studied with reverse-phase HPLC, was homogeneous with the majority of tumor galanin coeluting with standard human galanin. In the tumors analysed with in situ hybridization there was a good correlation between galanin peptide levels and galanin mRNA expression. In some tumors galanin mRNA and POMC levels coexisted, in others they were essentially in different cell populations. Levels of plasma galanin-LI were not related to tumor galanin concentration, and galanin levels were in the same range in sinus petrosus close to the pituitary venous drainage as in peripheral blood. Corticotrophin releasing hormone injections in two patients caused ACTH, but no detectable galanin release into sinus petrosus. Our results demonstrate that corticotroph, but not GH adenomas, express high levels of galanin, in addition to ACTH, and that in some tumors both polypeptides are synthesised in the same cell population. However, galanin levels in plasma were not influenced by the tumor galanin content.

Decreasing incidence of malignant tumors of the paranasal sinuses in Sweden. An analysis of 141 consecutive cases at Karolinska Hospital from 1960 to 1980.

We reviewed 141 cases of paranasal sinus tumors treated at Karolinska Hospital from 1960 to 1980. Of these tumors, 100 were located in the maxillary sinus, 32 in the ethmoidal sinuses, 8 in both the ethmoidal and maxillary regions, and 1 in the sphenoidal sinus. The male-to-female ratio was 2.1 to 1. Squamous cell carcinoma and adenocarcinoma were the most frequent types of tumors (55% and 13%, respectively). Treatment included surgery, irradiation, or both. The 5-year survival rate was 34% for squamous cell carcinomas and 64% for adenocarcinomas. When compared to a previous material of patients treated at the same hospital from 1940 to 1950, the proportion of poorly differentiated squamous cell carcinomas had increased significantly. The age-adjusted incidence rate decreased from 1.2 to 0.4 for male patients and from 0.7 to 0.3 for female patients between 1960 and 1980. We conclude that the incidence of malignant paranasal sinus tumors has decreased, and that squamous cell tumors now seem to be generally less differentiated than they were 50 years ago.