JOURNAL OF THE ROYAL SOCIETY OF MEDICINE. Volume 88. February ... on immediate parasellar structures, in particular on the .... Surgery can rarely cure.
JOURNAL OF THE ROYAL SOCIETY OF MEDICINE
Two macroprolactinomas presenting with neurological signs Emran G Khan MB MRCP
Volume 88
February 1995
(a)
Trevor A Howlett MD FRCP
J R Soc Med 1995;88:111 P-1 12P CASE PRESENTED TO SECTION OF ENDOCRINOLOGY, OCTOBER 1993
Keywords: macroprolactinomas; visual field defects; neurological signs;
hemiparesis
Macroprolactinomas commonly cause pressure effects on immediate parasellar structures, in particular on the optic chiasm to cause visual field defects. Pressure on more distant brain structures is rarely reported. We describe two massive prolactinomas presenting with neurological signs, including signs of hemiparesis which, to our knowledge, has not been reported previously.
CASE REPORTS Case I A 27-year-old woman was admitted with an acute history of
drowsiness, urinary incontinence, poor coordination of right arm and leg, and a 1 year history of malaise, weight gain and personality changes. On firther enquiry, she had a 10 year history of secondary amenorrhoea. She appeared drowsy and apathetic, she had bilateral papilloedema but no visual field defect, a constant right parietal drift and intermittent bilateral extensor plantars and right pyramidal signs. Magnetic resonance imaging (MRI) scan (Figure Ia) showed a large, partially cystic, suprasellar mass compressing the brain stem and causing obstructive hydrocephalus. An urgent serum prolactin was 173000mU/l, cortisol (490 nmol/1), and total thyroxine (140mnmol/l) were normal, and serum human chorionic gonadotrophin (hCG) was undetectable. Since the diagnosis of macroprolactinoma was inescapable, she was treated with bromocriptine rather than seeking neurosurgical intervention. Within 4 days of bromocriptine she made a full recovery of her neurological signs. Mental function improved and her family reported that she was 'back to her usual self'. Two months later, she reported that she felt better than she had done 'for years', and MRI showed marked tumour shrinkage. Further MRI at 12 months
Department of Endocrinology, Leicester Royal infirmary, Lekcestr LE1 5WW, UK Correspondence to: Dr T A Howiott, Consultant Endocrinologist
(b)Omwsmm
I Figure 1 (a) Sagittal magnetic resonance Imaging scan of Case 1, showing a partially cystic macroprolactinoma with massive supraseilar extensive compressing the brain stem and causing hydrocephalus. (b) Case 1, after 12 months' treatment with bromocriptine showing substantial tumour shrinkage
(Figure lb) showed progressive tumour shrinkage, serum prolactin has fallen to 5718 mU/l but amenorrhoea persists. Case 2 A 49-year-old man was referred with a history of gradually increasing right-sided weakness, slurred speech and visual loss. Past medical history included hypothyroidism (20 years) and insulin dependent diabetes mellitus (10 years). Possible acromegalic features had been noted 8 years previously but not further investigated. On examination, he had bitemporal field loss, a right hemiparesis with facial weakness, and mild acromegalic features. Computerized tomography (CT) scan revealed a massive pituitary
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macroadenoma with suprasellar and lateral extension. Seruam prolactin was 473 000 mU/l, he was gonadotrophin deficient, cortisol response to IV tetracosactrin was normal and a glucose tolerance test confirmed mild acromegaly with non-suppression of growth hormone (GH; rose from 11 to 18 mU/l). A macroprolactinoma co-secreting growth hormone was diagnosed. Bromocriptine therapy caused rapid resolution of visual field defects and complete recovery of the hemiparesis within three months, serum prolactin fell to 6000mU/l and CT scan showed tumour shrinkage to within the pituitary fossa. Fifteen months later, during a prolonged admission for a traumatic vertebral fracture, recurrent visual field defects were noted. CT scan showed recurrence of suprasellar and lateral extensions, despite ongoing suppression of prolactin to 2100mU/l with bromocriptine and a stable GH of 6.2 mU/l. Pituitary irradiation was given to control tumour growth in the long term. DISCUSSION
Pituitary macroadenomas frequently compress the optic chiasm, occasionally cause cranial nerve lesions within the cavernous sinus and are rarely reported to cause more distant effects such as hydrocephalus, epilepsy and cerebrospinal fluid (CSF) rhinorrhoea, but we can find no previous report of such tumours presenting with hemiparesis. The shrinhge of macroprolactinomas with dopamine agonist therapy is also well described1-3, and these cases confirm that clinically-important shrinkage may also occur in patients with this type of massive tumour. The rapid clinical recovery of Case 1, with consequent avoidance of neurosurgical intervention, illustrates the usefulness of obtaining an urgent serum prolactin estimation in such patients with large midline intracranial tumours involving the suprasellar region, since macroprolactinomas will respond well to medical therapy, whereas most other lesions of this size would prove inoperable. In spite of the good initial response to bromocriptine, however, the recurrence which occurred in Case 2, despite continuing suppression of serum prolactin, illustrates the need for
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caution and to consider the use of additional long-term therapy to control tumour growth. Surgery can rarely cure such tumours4'5 and many workers would advocate pituitary irradiation after tumour shrinkage6'7. However, it should be noted that tumour recurrence may occur even after radiotherapy7, and it may well be that the type of recurrence seen in Case 2, or after radiotherapy, is related to the primary aggressive nature of the tumour, and/or the continued growth of one clone of cells within the tumour which are resistant to dopamine agonist or radiation therapy (and which in Case 2 presumably did not secrete prolactin). Our original proposal for both cases was to administer pituitary irradiation once maxinal tumour shrinkage has been achieved, and this remains our plan for Case 1. The optimal timing of radiotherapy is, however, difficult to judge; Case 2 suggests that irradiation should be given as soon as possible, but the occurrence of gonadotrophin deficiency after radiotherapy has led other centres to advocate that therapy should be delayed in young women, such as Case 1, until near the time of any planned
conception7. REFERENCES I Wass JAH, Wiliiams J, Charlesworth M, et a]. Bromocriptine in 2 3 4
5 6
7
management of large pituitary tumours. B MJ 1982;284:1908-11 Molitch ME, Elton RL, Blackwell RE, et al. Bromocriptine as primary therapy for prolactin secreting macroadenomas, results of a prospective multicentre study. J Clin Endocrinol Metab 1985;60:698-705 Bevan JS, Webster J, Burke CW, Scanlon MF. Dopamine agonists and pituitary tumor shrinkage. Endocr Rev 1992;13:220-40 Seri 0, Rasio E, Bearegard H, Hardy J, Somma M. Recurrence of hyperprolactinoma after sdective transphenoidal adenectomy in women with prolactinomas. N EnglJ Med 1983;309:280-3 Feria MA, Tindall GT. Transsphenoidal microsurgery for prolactin secreting pituitary adenomas. J Neurosurg 1982;56:33-43 Jones A. Radiation oncogenesis in relation to treatment of pituitary tumours. Clin Endocrnol 1991;35:379-97 Tsagarais S, Gromman A, Plowman PN, et al. Megavoltage pituitary irradiation in management of prolacdnomas; long term follow-up. Cin Endocinol 1991;34:399-406
(Accepted 7 June 1994)
