Treatment of Nelson's syndrome by pituitary implantation of yttrium-90 or gold-198.

Cassar, J.; Doyle, F. H.; Pd, Lewis; Mashiter, Κ.; Noorden, Susan Van; Joplin, G. F.

doi:10.1136/bmj.2.6030.269

Cited by 20 publications

(5 citation statements)

References 14 publications

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“…Some investigators reported favourable results by conventional external pituitary irradiation [3,23,26,39], by intra-pituitary implantation of radioactive materials [3,4] or by heavy particle irradiation [24,27], however, other literature [32,55,57] pointed out that the response to radiotherapy was, on the whole, poor. Results of trans-sphenoidal microsurgical removal of Nelson's adenomas by several expert neurosurgeons have been unexpectedly poor with the cure rate of 20-30% [9,14,25,33,56,57].…”

Section: Treatmentmentioning

confidence: 97%

Trans-sphenoidal microsurgical treatment of Nelson's syndrome

Fukushima

1985

Neurosurg. Rev.

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Clinical features and the operative results in the author's personal series of ten cases with Nelson's syndrome are presented. There were nine females and one male patient with ages ranging from 20 to 44 years (mean 36 y). All patients presented with typical hyperpigmentation of the skin and had abnormally raised plasma ACTH levels. X-ray polytomography detected sellar abnormalities in 80% of the cases. Computerized tomography was positive for pituitary tumour in three patients. Trans-sphenoidal microsurgical removal of Nelson's ACTH adenomas were successful in seven of ten patients. The operative technique of radical-selective resection to achieve endocrinological cure is described. Pertinent literature is reviewed and the incidence of Nelson's syndrome, an issue of controversies regarding the pathogenesis, and the management of Nelson's adenomas are discussed.

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Section: Treatmentmentioning

confidence: 97%

Trans-sphenoidal microsurgical treatment of Nelson's syndrome

Fukushima

1985

Neurosurg. Rev.

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“…Early studies (1976,1977) reported outcomes in 28 patients treated by radiation with heavy particles (910 MeV alpha), leading to improvement of hyperpigmentation and decline of ACTH (85,86); one study from 1976 used the implantation of Yttrium-90 and Gold-198 seeds into the pituitary, by which also improvement and an ACTH decline could be achieved (87).…”

Section: Other Forms Of Radiationmentioning

confidence: 99%

Corticotroph tumor progression after bilateral adrenalectomy (Nelson’s syndrome): systematic review and expert consensus recommendations

Reincke

Albani

Assié

et al. 2021

European Journal of Endocrinology

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BACKGROUND: Corticotroph tumor progression (CTP) leading to Nelson’s syndrome (NS) is a severe and difficult-to-treat complication subsequent to bilateral adrenalectomy (BADX) for Cushing’s disease. Its characteristics are not well described, and consensus recommendations for diagnosis and treatment are missing. METHODS: A systematic literature search was performed focusing on clinical studies and case series (≥5 patients). Definition, incidence, treatment and long-term outcomes of CTP/NS after BADX were analyzed using descriptive statistics. The results were presented and discussed at an interdisciplinary consensus workshop attended by international pituitary experts in Munich on October 28th, 2018. RESULTS: Data covered definition and incidence (34 studies, 1275 patients), surgical outcome (12 studies, 187 patients), outcome of radiation therapy (21 studies, 273 patients), and medical therapy (15 studies, 72 patients). CONCLUSIONS: We endorse the definition of CTP-BADX/NS as radiological progression or new detection of a pituitary tumor on thin-section MRI. We recommend surveillance by MRI after 3 months and every 12 months for the first 3 years after BADX. Subsequently, we suggest clinical evaluation every 12 months and MRI at increasing intervals every 2-4 years (depending on ACTH and clinical parameters). We recommend pituitary surgery as first-line therapy in patients with CTP-BADX/NS. Surgery should be performed before extrasellar expansion of the tumor to obtain complete and long-term remission. Conventional radiotherapy or stereotactic radiosurgery should be utilized as second-line treatment for remnant tumor tissue showing extrasellar extension.

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“…Long-term outcomes of patients with NS, especially in the modern era, have been poorly explored. This is due to the limited number of published series, often from single centres, each with very small number of patients (given the rarity of NS), and commonly with short follow-up (14,23,26,(28)(29)(30)(31)(32)(33)(34)(35)(36)(37)(38).…”

Section: Consensus On What Defines Ns Is Lacking the Most Widely Accmentioning

confidence: 99%

Outcomes of Patients with Nelson’s Syndrome after Primary Treatment: A Multicenter Study from 13 UK Pituitary Centers

Fountas

Lim

Drake

et al. 2019

The Journal of Clinical Endocrinology &Amp; Metabolism

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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.

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Treatment of Nelson's syndrome by pituitary implantation of yttrium-90 or gold-198.

Cited by 20 publications

References 14 publications

Trans-sphenoidal microsurgical treatment of Nelson's syndrome

Trans-sphenoidal microsurgical treatment of Nelson's syndrome

Corticotroph tumor progression after bilateral adrenalectomy (Nelson’s syndrome): systematic review and expert consensus recommendations

Outcomes of Patients with Nelson’s Syndrome after Primary Treatment: A Multicenter Study from 13 UK Pituitary Centers

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