The treatment of choice for Cushing's disease is surgical removal of tumor, the source of ACTH overproduction. In occasional patients in whom a surgical approach including total adrenalectomy is not feasible or surgical removal of tumor is not complete, medical treatment may be necessary because pituitary irradiation requires a long 1ag time to remission. Although ketoconazole, an imidazole derivative with inhibitory activity on adrenal steroidogenesis has been reported to be effective in the treatment of Cushing's disease, the limited effectiveness in lowering very high level of cortisol and occasional hepatotoxicity restrains its wide use. In this report, we describe a woman with Cushing's disease due to pituitary microadenoma. Transsphenoidal pituitary adenomeetomy followed by ketoconzole treatment had been unsuccessful in achieving remission of the disease, but combined treatment with ketoconazole and octreotide accomplished successful reduction in cortisol production.
Adrenalectomy ; Adrenocorticotropic Hormone ; Female ; Humans ; Hydrocortisone ; Ketoconazole* ; Octreotide* ; Pituitary Irradiation

OBJECTIVE: Cancer pain has been treated by gamma knife radiosurgery(GKS), targeted to the pituitary gland-stalk, as an alternative new pain control method. The purpose of this study is to prove the efficacy and the safety of this treatment. METHODS: Seven patients with intractable cancer pain underwent pituitary gland-stalk irradiation by gamma knife. Selections for patient inclusion in this treatment protocol were no other effective pain treatment options, general condition rated as greater than 40 on Karnofsky Performance Scale, and pain relief by morphine though not satisfactory. The target was the junction between the pituitary stalk and the neurohypophysis. The maximum dose was 150~160Gy with one isocenter in 8mm collimator or two isocenters in 4mm collimator keeping the radiation dose to the optic nerve less than 8Gy. RESULTS: In all seven cases, the significant pain reduction was obtained during immediate post-GKS period without serious complications except one patient who developed transient hypopituitarism and diabetes insipidus. Pain relief was observed within several days, and this effect was prolonged for a quite long time. At a follow up of 1.5 to 13 months, pain recurred in two patients and no hormonal and visual dysfunctions were observed. CONCLUSION: Despite insufficient experience, the efficacy and the safety of GKS for intractable cancer pain were demonstrated in seven patients. This treatment has the potential to ameliorate cancer pain, and GKS will play a more important role in the treatment of intractable pain.
Clinical Protocols ; Diabetes Insipidus ; Follow-Up Studies ; Humans ; Hypopituitarism ; Morphine ; Optic Nerve ; Pain, Intractable ; Pituitary Gland ; Pituitary Gland, Posterior ; Pituitary Irradiation* ; Radiosurgery

Pituitary tumors occur in 15-50% of patients with multiple endocrine neoplasia of type 1 (MEN1). To the best of our knowledge, no MEN1 case in which the initial lesion was an invasive giant prolactinoma has been reported from Korea. We describe a patient in whom a skull-base tumor involved the sellar and parasellar spaces. A 49 year-old female presented with headache and diplopia. The tumor was ultimately identified as a giant prolactinoma; the serum prolactin concentration increased from 155.6 ng/mL to 3,234.3 ng/mL after cranial irradiation. She was evaluated in terms of incidental hypercalcemia and was found to have parathyroid hyperplasia. Genetic analysis revealed a missense mutation in the MEN1 gene (c.643G>A, p.Val215Met). Two years of treatment with a dopamine agonist reduced, but did not normalize, the serum prolactin concentration. We highlight the aggressive behavior of the giant skull-base tumor, and the diagnostic delay caused by a high-dose hook effect of the MEN1-related prolactinoma.
Cranial Irradiation ; Diplopia ; Dopamine Agonists ; Female ; Headache ; Humans ; Hypercalcemia ; Hyperplasia ; Korea ; Multiple Endocrine Neoplasia Type 1* ; Multiple Endocrine Neoplasia* ; Mutation, Missense ; Pituitary Neoplasms ; Prolactin ; Prolactinoma*