Size reduction of an ACTH-secreting pituitary macroadenoma in Nelson's syndrome by sodium valproate: effect of withdrawal and re-institution of treatment

1988 ◽  
Vol 119 (3) ◽  
pp. 435-442 ◽  
Author(s):  
Paola Loli ◽  
Maria Elisa Berselli ◽  
Federico Vignati ◽  
Carlo De Grandi ◽  
Mariantonella Tagliaferri

Abstract. We describe herein the reduction in the size of an ACTH-secreting pituitary macroadenoma in a patient with Nelson's syndrome during chronic administration of sodium valproate, and the changes in tumour volume after withdrawal and re-institution of treatment. The patient had elevated plasma ACTH levels (1123–1255 pmol/l), which increased markedly after CRH stimulation. A first 4-month course of sodium valproate administration (600 mg/day, orally) was started. Plasma ACTH fell to 550–726 pmol/l with persistence of responsiveness to CRH; brain computed tomography showed a clearcut reduction of tumour size. One month after drug withdrawal, the tumour volume appeared unchanged and plasma ACTH values ranged between 374 and 440 pmol/l. One and a half year after drug withdrawal, a brain computed tomography showed re-expansion of the pituitary adenoma with evidence of suprasellar extension, which had never been seen previously. Plasma ACTH ranged between 113 and 199 pmol/l. A second course of sodium valproate was started; after three months, a brain computed tomography documented clearcut reduction of tumour volume from a suprasellar extension to a partially empty pituitary fossa. Plasma ACTH ranged from 396 to 542 pmol/l with persistence of responsiveness to CRH. The present report documents for the first time the reduction of tumour size in a patient with an ACTH-secreting macroadenoma by chronic administration of sodium valproate.

2014 ◽  
Vol 65 (1) ◽  
pp. 86-90 ◽  
Author(s):  
Ryan K.L. Lee ◽  
Shirley Y.W. Liu ◽  
Cina S.L. Tong ◽  
Paul S.F. Lee ◽  
Enders K.W. Ng ◽  
...  

Objective To evaluate the morphologic changes of aldosterone-producing adenoma (APA) on computed tomography (CT) before and after radiofrequency ablation (RFA) and to assess the factors that are important in determining successful complete ablation of these tumours. Method Between August 2004 and August 2011, 24 consecutive patients with APA undergoing CT-guided percutaneous RFA were identified from our prospective database. The pre-RFA and post-RFA CT appearances of these APAs that showed positive biochemical response were reviewed retrospectively for their 3-dimensional size, tumour volume, and CT attenuation in terms of Hounsfield units (HU). A comparison of these parameters before and after RFA was performed. Results In this study, there were 23 APAs in these 24 patients that showed biochemical cure of primary aldosteronism after RFA. When comparing post-RFA to pre-RFA CTs, there was no significant change in tumour size (14.5 mm vs 14.6 mm: P = .83) and tumour volume (1.55 cm3 vs 1.59 cm3; P = .41) after RFA. In nonenhanced CT images, there was no significant reduction in HU from pre-RFA to post-RFA measurements (4.4 HU vs 7.9 HU; P = .52). In contrast-enhanced CTs, there was a significant drop in HU after RFA (from 48.3 HU to 14.7 HU; P = .03). None of the included cases showed a focal region of contrast enhancement to suggest residual tumour. Conclusion A change in tumour size, tumour volume, and HU in nonenhanced CT were unreliable in defining radiologic treatment success. Only changes in HU in contrast-enhanced CT was useful in confirming a positive treatment response after RFA for APA.


1984 ◽  
Vol 105 (4) ◽  
pp. 449-454 ◽  
Author(s):  
Grant Gwinup ◽  
Alan N. Elias ◽  
Benjamin Choi

Abstract. Di-n-propylacetic acid (valproic acid) has been reported to lower plasma ACTH concentration after both acute and chronic administration of the drug. To date, there is no report on the efficacy of the drug in inducing regression of ACTH-secreting pituitary adenomas. We report herein our findings in a patient with Nelson's syndrome who was treated with valproic acid for over a year. Despite a lowering of her plasma ACTH concentration during therapy with valproic acid, the patient's tumour showed no evidence of regression while she was taking the drug. In view of these findings, valproic acid therapy for ACTH-secreting pituitary tumours needs to be re-evaluated.


1984 ◽  
Vol 107 (4) ◽  
pp. 471-475 ◽  
Author(s):  
Hans P. F. Koppeschaar ◽  
Ronald J. M. Croughs ◽  
Johanna W. van 't Verlaat ◽  
Martin J. Hendriks ◽  
Cor J. M. Arts ◽  
...  

Abstract. Transfrontal hypophysectomy was performed in a patient with Cushing's disease and gross enlargement of the pituitary. Despite some reduction of cortisol production active Cushing's syndrome remained due to the presence of a tumour remnant. Medical treatment with the GABA-transaminase inhibitor sodium valproate induced hypocorticism necessitating corticosteroid substitution therapy. Nine months after sodium valproate withdrawal hypercorticism was documented. Re-institution of sodium valproate treatment induced hypocorticism again. As sodium valproate is known to induce a decrease of plasma ACTH in Nelson's syndrome, it is proposed that large tumours present at the time of diagnosis and those appearing after adrenalectomy may represent the spectrum of a single disorder. A prospective trial to study the effects of sodium valproate and other neurotransmitter modulating agents on the size and endocrine function of ACTH secreting macroadenomas is urgently needed.


1992 ◽  
Vol 50 (4) ◽  
pp. 539-542 ◽  
Author(s):  
Nélida S. Garretto ◽  
David Monteverde ◽  
Héctor Giócoli ◽  
Blanca I. Ravera ◽  
Hugo A. Molina ◽  
...  

Schwannomatosis is a rare disorder, still not quite well defined, seldom described in the literature. In this paper we report the case of male. Patient, 52 years old, who in the last 30 years developed five subcutaneous tumors within his limbs peripheral nerves, which histologically proved to be schwannomas. A brain computed tomography showed a partially calcified tumor in the left temporal lobe which most likely was a meningioma. A thorough clinical examination was unable to find signs of type I or type II neurofibromatosis. The present condition, probably a form of phacomatosis, has to be distinguished from neurofibromatosis and is considered as an independent clinical entity whose origin still awaits further detailed investigations.


2021 ◽  
Author(s):  
Maria Fleseriu ◽  
Dagmer Führer-Sakel ◽  
Aart J van der Lely ◽  
Laura De Marinis ◽  
Thierry Brue ◽  
...  

Objective: To report the final long-term safety and efficacy analyses of patients with acromegaly treated with pegvisomant from the ACROSTUDY. Design: Global (15 countries), multicentre, non-interventional study (2004-2017). Methods: The complete ACROSTUDY cohort comprised patients with acromegaly, who were being treated with pegvisomant (PEGV) prior to the study or at enrolment. Main endpoints were long-term safety (comorbidities, adverse events [AEs], pituitary tumour volumes, liver tests) and efficacy (IGF-1 changes). Results: Patients (n = 2221) were treated with PEGV for a median of 9.3 years (range, 0-20.8 years) and followed up for a median of 7.4 years (range, 0-13.9 years). Before PEGV, 96.3% had received other acromegaly treatments (surgery/radiotherapy/medications). Before PEGV treatment, 87.2% of patients reported comorbidities. During ACROSTUDY, 5567 AEs were reported in 56.5% of patients and of these 613 were considered treatment-related (in 16.5% of patients) and led to drug withdrawal in 1.3%. Pituitary imaging showed a tumour size increase in 7.1% of patients; the majority (71.1%) reported no changes. Abnormal AST or ALT liver tests occurred in 3.2% of patients. IGF-1 normalization rate improved over time, increasing from 11.4% at PEGV start to 53.7% at year 1, and reaching 75.4% at year 10 with use of ≥30 mg PEGV/day in an increasing proportion of patients. Conclusion: This comprehensive review of the complete cohort in ACROSTUDY confirmed the overall favourable benefit-to-risk profile and high efficacy of PEGV as mono- and combination therapy in patients with an aggressive course/uncontrolled/active acromegaly requiring long-term medical therapy for control.


2001 ◽  
Vol 33 (2) ◽  
pp. 334-339 ◽  
Author(s):  
Thomas J. Tegos ◽  
Michael M. Sabetai ◽  
Andrew N. Nicolaides ◽  
Tarek S. Elatrozy ◽  
Surinder Dhanjil ◽  
...  

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