Management of Nelson’s Syndrome
Nelson’s syndrome is a potentially severe condition that may develop in patients with Cushing’s disease treated with bilateral adrenalectomy. Its management can be challenging. Pituitary surgery followed or not by radiotherapy offers the most optimal tumour control, whilst pituitary irradiation alon...
| Main Authors: | , |
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| Format: | Article |
| Language: | English |
| Published: |
MDPI AG
2022-11-01
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| Series: | Medicina |
| Subjects: | |
| Online Access: | https://www.mdpi.com/1648-9144/58/11/1580 |
| _version_ | 1797467316552728576 |
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| author | Athanasios Fountas Niki Karavitaki |
| author_facet | Athanasios Fountas Niki Karavitaki |
| author_sort | Athanasios Fountas |
| collection | DOAJ |
| description | Nelson’s syndrome is a potentially severe condition that may develop in patients with Cushing’s disease treated with bilateral adrenalectomy. Its management can be challenging. Pituitary surgery followed or not by radiotherapy offers the most optimal tumour control, whilst pituitary irradiation alone needs to be considered in cases requiring intervention and are poor surgical candidates. Observation is an option for patients with small lesions, not causing mass effects to vital adjacent structures but close follow-up is required for a timely detection of corticotroph tumour progression and for further treatment if required. To date, no medical therapy has been consistently proven to be effective in Nelson’s syndrome. Pharmacotherapy, however, should be considered when other management approaches have failed. A subset of patients with Nelson’s syndrome may develop further tumour growth after primary treatment, and, in some cases, a truly aggressive tumour behaviour can be demonstrated. In the absence of evidence-based guidance, the management of these cases is individualized and tailored to previously offered treatments. Temozolomide has been used in patients with aggressive Nelson’s with no consistent results. Development of tumour-targeted therapeutic agents are an unmet need for the management of aggressive cases of Nelson’s syndrome. |
| first_indexed | 2024-03-09T18:51:54Z |
| format | Article |
| id | doaj.art-6dd7ce29179c4291954b58735ade7806 |
| institution | Directory Open Access Journal |
| issn | 1010-660X 1648-9144 |
| language | English |
| last_indexed | 2024-03-09T18:51:54Z |
| publishDate | 2022-11-01 |
| publisher | MDPI AG |
| record_format | Article |
| series | Medicina |
| spelling | doaj.art-6dd7ce29179c4291954b58735ade78062023-11-24T05:47:07ZengMDPI AGMedicina1010-660X1648-91442022-11-015811158010.3390/medicina58111580Management of Nelson’s SyndromeAthanasios Fountas0Niki Karavitaki1Institute of Metabolism and Systems Research, College of Medical and Dental Sciences, University of Birmingham, Birmingham B15 2TT, UKInstitute of Metabolism and Systems Research, College of Medical and Dental Sciences, University of Birmingham, Birmingham B15 2TT, UKNelson’s syndrome is a potentially severe condition that may develop in patients with Cushing’s disease treated with bilateral adrenalectomy. Its management can be challenging. Pituitary surgery followed or not by radiotherapy offers the most optimal tumour control, whilst pituitary irradiation alone needs to be considered in cases requiring intervention and are poor surgical candidates. Observation is an option for patients with small lesions, not causing mass effects to vital adjacent structures but close follow-up is required for a timely detection of corticotroph tumour progression and for further treatment if required. To date, no medical therapy has been consistently proven to be effective in Nelson’s syndrome. Pharmacotherapy, however, should be considered when other management approaches have failed. A subset of patients with Nelson’s syndrome may develop further tumour growth after primary treatment, and, in some cases, a truly aggressive tumour behaviour can be demonstrated. In the absence of evidence-based guidance, the management of these cases is individualized and tailored to previously offered treatments. Temozolomide has been used in patients with aggressive Nelson’s with no consistent results. Development of tumour-targeted therapeutic agents are an unmet need for the management of aggressive cases of Nelson’s syndrome.https://www.mdpi.com/1648-9144/58/11/1580Nelson’s syndromeCushing’scorticotroph tumour progressionbilateral adrenalectomytumour growthpituitary surgery |
| spellingShingle | Athanasios Fountas Niki Karavitaki Management of Nelson’s Syndrome Medicina Nelson’s syndrome Cushing’s corticotroph tumour progression bilateral adrenalectomy tumour growth pituitary surgery |
| title | Management of Nelson’s Syndrome |
| title_full | Management of Nelson’s Syndrome |
| title_fullStr | Management of Nelson’s Syndrome |
| title_full_unstemmed | Management of Nelson’s Syndrome |
| title_short | Management of Nelson’s Syndrome |
| title_sort | management of nelson s syndrome |
| topic | Nelson’s syndrome Cushing’s corticotroph tumour progression bilateral adrenalectomy tumour growth pituitary surgery |
| url | https://www.mdpi.com/1648-9144/58/11/1580 |
| work_keys_str_mv | AT athanasiosfountas managementofnelsonssyndrome AT nikikaravitaki managementofnelsonssyndrome |