Exceptional Response of Nelson’s Syndrome to Pasireotide LAR in the Long-Term Follow-up of 9 Years

Author(s):  
Sandra M. Fill ◽  
Kathrin H. Popp ◽  
Günter K. Stalla ◽  
Anastasia P. Athanasoulia-Kaspar
1989 ◽  
Vol 31 (3) ◽  
pp. 309-323 ◽  
Author(s):  
T. A. HOWLETT ◽  
P. N. PLOWMAN ◽  
J. A. H. WASS ◽  
L. H. REES ◽  
A. E. JONES ◽  
...  

2019 ◽  
Vol 105 (5) ◽  
pp. 1527-1537 ◽  
Author(s):  
Athanasios Fountas ◽  
Eugenie S Lim ◽  
William M Drake ◽  
Andrew S Powlson ◽  
Mark Gurnell ◽  
...  

Abstract Context Long-term outcomes of patients with Nelson’s syndrome (NS) have been poorly explored, especially in the modern era. Objective To elucidate tumor control rates, effectiveness of various treatments, and markers of prognostic relevance in patients with NS. Patients, design, and setting Retrospective cohort study of 68 patients from 13 UK pituitary centers with median imaging follow-up of 13 years (range 1–45) since NS diagnosis. Results Management of Cushing’s disease (CD) prior to NS diagnosis included surgery+adrenalectomy (n = 30; eight patients had 2 and one had 3 pituitary operations), surgery+radiotherapy+adrenalectomy (n = 17; two received >1 courses of irradiation, two had ≥2 pituitary surgeries), radiotherapy+adrenalectomy (n = 2), and adrenalectomy (n = 19). Primary management of NS mainly included surgery, radiotherapy, surgery+radiotherapy, and observation; 10-year tumor progression-free survival was 62% (surgery 80%, radiotherapy 52%, surgery+radiotherapy 81%, observation 51%). Sex, age at CD or NS diagnosis, size of adenoma (micro-/macroadenoma) at CD diagnosis, presence of pituitary tumor on imaging prior adrenalectomy, and mode of NS primary management were not predictors of tumor progression. Mode of management of CD before NS diagnosis was a significant factor predicting progression, with the group treated by surgery+radiotherapy+adrenalectomy for their CD showing the highest risk (hazard ratio 4.6; 95% confidence interval, 1.6–13.5). During follow-up, 3% of patients had malignant transformation with spinal metastases and 4% died of aggressively enlarging tumor. Conclusions At 10 years follow-up, 38% of the patients diagnosed with NS showed progression of their corticotroph tumor. Complexity of treatments for the CD prior to NS diagnosis, possibly reflecting corticotroph adenoma aggressiveness, predicts long-term tumor prognosis.


2002 ◽  
Vol 87 (12) ◽  
pp. 5465-5469 ◽  
Author(s):  
P. A. Kelly ◽  
G. Samandouras ◽  
A. B. Grossman ◽  
F. Afshar ◽  
G. M. Besser ◽  
...  

Abstract Total bilateral adrenalectomy remains the definitive procedure for cure in Cushing’s disease. It is complicated by the development of Nelson’s syndrome, the treatment of which remains troublesome. We report the long-term follow-up, median 17 yr (range, 8–22 yr), of 13 patients (3 males and 10 females) treated with pituitary surgery for Nelson’s syndrome at a median age of 35 yr (range, 21–67 yr). The presence of a pituitary mass lesion necessitated neurosurgery in all. Preoperatively, the median plasma ACTH level was 664 pmol/liter (range, 92–3665 pmol/liter); this fell to 29 pmol/liter (range, <2 to 1124 pmol/liter) postoperatively (P < 0.0005). Cutaneous hyperpigmentation was reduced in all and resolved in 11 patients. The pituitary tumor bulk was clearly reduced in 12 patients. There was no perioperative mortality. No patient developed a visual field defect attributable to surgery. New anterior pituitary hormone deficiency occurred in seven patients, and permanent diabetes insipidus occurred in five patients. At latest follow-up, the median plasma ACTH is 73 pmol/liter (range, <2 to 7759 pmol/liter); six patients have levels of less than 44 pmol/liter and also have a resolution of their pigmentation and no evidence of a recurrence of a pituitary mass lesion. We conclude that pituitary surgery is an efficacious treatment for mass lesions associated with Nelson’s syndrome, has long-term benefit with minimal side effects, and must be considered in the management of this distressing complication.


2019 ◽  
Vol 42 ◽  
Author(s):  
John P. A. Ioannidis

AbstractNeurobiology-based interventions for mental diseases and searches for useful biomarkers of treatment response have largely failed. Clinical trials should assess interventions related to environmental and social stressors, with long-term follow-up; social rather than biological endpoints; personalized outcomes; and suitable cluster, adaptive, and n-of-1 designs. Labor, education, financial, and other social/political decisions should be evaluated for their impacts on mental disease.


2001 ◽  
Vol 120 (5) ◽  
pp. A397-A397
Author(s):  
M SAMERAMMAR ◽  
J CROFFIE ◽  
M PFEFFERKORN ◽  
S GUPTA ◽  
M CORKINS ◽  
...  

2001 ◽  
Vol 120 (5) ◽  
pp. A204-A204
Author(s):  
B GONZALEZCONDE ◽  
J VAZQUEZIGLESIAS ◽  
L LOPEZROSES ◽  
P ALONSOAGUIRRE ◽  
A LANCHO ◽  
...  

2001 ◽  
Vol 120 (5) ◽  
pp. A754-A755 ◽  
Author(s):  
H ALLESCHER ◽  
P ENCK ◽  
G ADLER ◽  
R DIETL ◽  
J HARTUNG ◽  
...  

2007 ◽  
Vol 177 (4S) ◽  
pp. 3-4
Author(s):  
George J. Huang ◽  
Natalia Sadetsky ◽  
Peter R. Carroll ◽  
David F. Penson

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