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[Nelson's syndrome concomitant with Cushing's syndrome]
A Vicente Delgado1, T Lucas Morante, C Páramo Fernández
1Servicio de Endocrinología, Hospital Puerta de Hierro, Madrid.
Revista Clinica Espanola
|June 1, 1991
Summary
Nelson's Syndrome occurred with Cushing's Syndrome after bilateral adrenalectomy. Treatment involved surgery and radiotherapy, leading to remission but persistent ACTH elevation and hypopituitarism.
Area of Science:
- Endocrinology
- Oncology
- Neurosurgery
Background:
- Cushing's Disease, a condition causing excess cortisol, is often treated with bilateral adrenalectomy.
- Nelson's Syndrome can develop post-adrenalectomy due to persistent stimulation of a pituitary adenoma by elevated ACTH.
- This case highlights the complex interplay between these endocrine disorders.
Observation:
- A female patient presented with hyperpigmentation, elevated ACTH, and an intrasellar mass five years after adrenalectomy for Cushing's Disease.
- Diagnostic findings included non-rhythmic, non-suppressible plasma cortisol and increased urinary cortisol.
- A pituitary macroadenoma was identified via CT scan.
Findings:
- Surgical resection of the macroadenoma followed by pituitary radiotherapy resulted in clinical remission and biochemical improvement.
- Post-treatment, the patient exhibited persistent slightly elevated ACTH levels, panhypopituitarism, and an empty sella turcica.
- The case underscores the challenges in managing Nelson's Syndrome after treatment for Cushing's Disease.
Implications:
- This case demonstrates a rare presentation of concomitant Nelson's and Cushing's Syndromes.
- Effective management requires a multidisciplinary approach involving endocrinologists, neurosurgeons, and radiation oncologists.
- Long-term monitoring is crucial for patients with a history of pituitary adenomas post-adrenalectomy.