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Published on: January 17, 2018
Surgical strategies in the treatment of MR-negative Cushing's Disease: a systematic review and treatment algorithm
Andrew B Yang1, Fraser Henderson2, Theodore H Schwartz3
1Weill Cornell Medical School, New York, NY, USA.
Purpose:
Several surgical strategies have been proposed to treat MRI-negative Cushing's Disease. These include tumor removal, if identified, and if a tumor is not identified, resection of varying degrees of the pituitary gland, often guided by inferior petrosal sinus sampling (IPSS). The relative risks and benefits of each strategy have never been compared.
Methods:
This systematic review of the literature included only studies on the results of surgery for MRI-negative patients with Cushing's Disease in which the surgical strategy was clearly described and associated remission and/or hypopituitarism rates detailed for each strategy.
Results:
We identified 12 studies that met inclusion criteria for remission rates and 5 studies for hypopituitarism rates. We divided cases into 6 resection strategies. Remission and hypopituitarism rates for each strategy were: (1) tumor identified, resect tumor only (68%, 0%); (2) resect tumor and surrounding capsule (85%, 0%); and if the tumor was not identified (3) resect inferior 1/3 of gland (78%, no data); (4) resect 30-50% of gland based on IPSS (68%, 13%); (5) resect > 50% but < 100% of gland (65%, 9%); (6) resect entire gland (66%, 67%). Strategy 3 only contained 9 patients.
Conclusion:
Remission rates for MRI-negative Cushing's Disease support surgery as a reasonable approach. Results are best if a tumor is found. If a tumor is not identified, one can either remove one-third of the gland guided by IPSS lateralization, or remove both lateral portions along with the inferior portion leaving sufficient central gland to preserve function. Our recommendations are limited by the lack of rigorous and objective data.
Insights
Surgery for MRI-negative Cushing's Disease offers good remission rates. Best outcomes occur when a tumor is found; otherwise, gland resection guided by inferior petrosal sinus sampling (IPSS) is recommended.
Area of Science:
- Neurosurgery
- Endocrinology
- Oncology
Background:
- Cushing's Disease (CD) is a rare endocrine disorder caused by excess cortisol.
- Magnetic resonance imaging (MRI) negative CD presents a diagnostic and therapeutic challenge.
- Surgical management of MRI-negative CD involves pituitary gland resection, often guided by inferior petrosal sinus sampling (IPSS).
Purpose of the Study:
- To systematically review and compare surgical strategies for MRI-negative Cushing's Disease.
- To evaluate remission and hypopituitarism rates associated with different surgical approaches.
Main Methods:
- Systematic literature review of studies on surgical outcomes in MRI-negative CD.
- Inclusion criteria: clearly described surgical strategy, reported remission and/or hypopituitarism rates.
- Categorization of surgical strategies into 6 distinct resection approaches.
Main Results:
- 12 studies reported remission rates; 5 studies reported hypopituitarism rates.
- Tumor resection yielded 68% remission (0% hypopituitarism).
- If tumor unidentified, resection strategies showed variable outcomes: 78% remission (no data) for inferior 1/3 resection, 68% remission (13% hypopituitarism) for 30-50% gland resection guided by IPSS, and 66% remission (67% hypopituitarism) for total gland resection.
Conclusions:
- Surgery is a viable treatment for MRI-negative Cushing's Disease.
- Optimal outcomes are achieved when a tumor is identified and resected.
- If no tumor is found, gland resection guided by IPSS or preserving central gland function is suggested, though data limitations exist.
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