Outcomes of patients with Nelson's syndrome after primary treatment: a multicenter study from 13 UK Pituitary centers.

Fountas, Athanasios and Lim, Eugenie S and Drake, William M and Powlson, Andrew S and Gurnell, Mark and Martin, Niamh M and Seejore, Khyatisha and Murray, Robert D and MacFarlane, James and Ahluwalia, Rupa and Swords, Francesca and Ashraf, Muhammad and Pal, Aparna and Chong, Zhuomin and Freel, Marie and Balafshan, Tala and Purewal, Tejpal S and Speak, Rowena G and Newell-Price, John and Higham, Claire E and Hussein, Ziad and Baldeweg, Stephanie E and Dales, Jolyon and Reddy, Narendra and Levy, Miles J and Karavitaki, Niki (2019) Outcomes of patients with Nelson's syndrome after primary treatment: a multicenter study from 13 UK Pituitary centers. The Journal of clinical endocrinology and metabolism. ISSN 1945-7197. This article is available to all UHB staff and students via ASK Discovery tool http://tinyurl.com/z795c8c by using their UHB Athens login IDs

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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 two and one had three 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, 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 (HR 4.6; 95% CI, 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.

Item Type: Article
Additional Information: This article is available to all UHB staff and students via ASK Discovery tool http://tinyurl.com/z795c8c by using their UHB Athens login IDs
Subjects: WK Endocrine system. Endocrinology
Divisions: Clinical Support > Pathology
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Depositing User: Mr Philip O'Reilly
Date Deposited: 27 Nov 2019 09:50
Last Modified: 27 Nov 2019 09:50
URI: http://www.repository.uhblibrary.co.uk/id/eprint/2626

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