Pituitary irradiation for recurrent steroid-responsive hypophysitis in three patients
F Coyle1, R Beaney4, J Miell1, P Bullock3, S Al-Sarraj2, AM McGregor1 & SJB Aylwin1
We have evaluated the effectiveness of radiotherapy in three patients with recurrent symptomatic hypophysitis following surgical decompression.
(54 F); presented with diabetes insipidus, hypopituitarism and a large hypophyseal mass. Following trans-sphenoidal surgery (TSS), a diagnosis of xanthomatous hypophysitis was made. Two years later severe headaches returned, and despite a second trans-sphenoidal procedure, visual acuity further deteriorated in the left eye (V/A 6/24) with a bitemporal quadrantinopia. She was treated with dexamethasone with prompt improvement in vision (L: V/A 6/9).
(33 F); presented post-partum with headache, amenorrhoea and a pituitary mass lesion with suprasellar extension. Following TSS, histology demonstrated lymphocytic hypophysitis. Four months later, headaches returned and an MRI showed a recurrent mass lesion. Glucocorticoid therapy induced resolution, but high doses were required.
(29 F): presented at 14 weeks gestation with headaches, blurred vision and diabetes insipidus. MRI demonstrated a pituitary lesion with suprasellar extension. Management was conservative through pregnancy, but four months post-partum visual disturbance occurred with radiological mass expansion, leading to TSS. Histology revealed granulomatous hypophysitis. Eight months later she represented with deteriorating vision (6/18 R; 6/9 L) and a bitemporal hemianopia. Dexamethasone treatment led to rapid resolution of symptoms and a radiological reduction in the mass.
These patients (all pan-hypopituitary) had biopsy proven hypophysitis and had demonstrated steroid-responsive disease. Steroid treatment was required at an unacceptably high dose and they were therefore offered pituitary irradiation. Patient 1: 45 Gy, 25 fractions, 3 fields; patients 2 and 3: 24 Gy, 3 fields, 12 fractions. Patients 1 and 2 are in remission (>6/12). Patient 3 had resolution of the pituitary mass, but subsequently developed systemic sarcoidosis.
The experience in these three patients supports the use of fractionated pituitary radiotherapy in the treatment of steroid responsive hypophysitis.