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Published on: January 17, 2018
Cushing's disease: management outcome in a tertiary care centre
Nalini S Shah1, A H Goel, R D Nagpal
1Department of Endocarinology, Seth GS Medical College and KEM Hospital, Parel, Mumbai.
Insights
Accurate lesion localization is crucial for managing Cushing's syndrome (CS). Combining high-dose dexamethasone suppression testing and imaging improves localization rates, aiding successful treatment of Cushing's disease (CD).
Area of Science:
- Endocrinology
- Neurosurgery
- Oncology
Background:
- Cushing's syndrome (CS) presents diagnostic and management challenges.
- Accurate localization of the causative lesion is essential for successful CS treatment.
- This article details a single-center experience with CS diagnosis and management.
Purpose of the Study:
- To evaluate diagnostic test yields for Cushing's disease (CD) lesion localization.
- To highlight challenges in CS diagnosis and management.
- To report outcomes of surgical and other treatment modalities.
Main Methods:
- Retrospective study of 100 CS patients, with 69 diagnosed with CD.
- Diagnosis confirmed by a 2-day low-dose dexamethasone suppression test.
- Lesion localization using high-dose dexamethasone suppression (HDDS) and imaging; IPSS used when needed.
Main Results:
- HDDS sensitivity 70%, specificity 99% for localization. Imaging localized 68%; combined HDDS and imaging 90%.
- IPSS localized lesions in 10% of cases. Transsphenoidal surgery (TSS) performed in 65 patients.
- Cure rates: 77% for microadenoma, 36% for macroadenoma. Mortality/morbidity 7% each.
Conclusions:
- Multidisciplinary management by experienced endocrinologists and neurosurgeons is optimal for CS.
- Further interventions like repeat TSS, radiotherapy, or adrenalectomy are options for treatment failures.
Unlabelled:
Cushing's syndrome (CS) can pose a challenge in diagnosis and management. Successful management of CS needs accurate localization of the site of lesion. Present article narrates experience of a single center dealing with large number of patients with CS and highlights difficulties in diagnosis as well as management of Cushing's disease (CD).
Methods:
All patients with CD, where histopathological lesion was documented were studied to evaluate yield from different diagnostic tests. Diagnosis was established by standard 2 days low dose dexamethasone suppression test. Localization of the lesion was achieved with high dose dexamethasone suppression (HDDS) and imaging. Inferior petrosal sinus sampling (IPSS) was used whenever diagnosis was not arrived at with the standard tests.
Results:
Out of 100 consecutive patients of CS seen, 69 had CD. HDDS had sensitivity of 70% and specificity of 99% in localizing the lesion. Imaging localized the lesion in 68% of patients. Combination of HDDS and imaging localized the lesion in 90% of patients. IPSS helped to localise the lesion in remaining 10% of patients. Transsphenoidal surgery was carried out in 65/69 patients. Cure rate for microadenoma was 77% and for macroadenoma was 36%. Mortality and morbidity rate was 7% and 7% respectively. 2nd TSS, radiotherapy and bilateral adrenalectomy were the additional modalities used in that order, in patients who were not cured or who had recurrence.
Conclusion:
Management of CS is best carried in a center where a team of experienced endocrinologist and neurosurgeon is available.
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