The role of bilateral adrenalectomy in the treatment of refractory Cushing's disease

2015 ◽  
Vol 38 (2) ◽  
pp. E9 ◽  
Author(s):  
Anni Wong ◽  
Jean Anderson Eloy ◽  
James K. Liu
Keyword(s):  
Cushing’S Disease ◽  
High Risk Patient ◽  
Laparoscopic Approach ◽  
Bilateral Adrenalectomy ◽  
Long Term Results ◽  
Cushing's Disease ◽  
Open Approach ◽  
Effective Treatment Modality

Cushing's syndrome (CS) results from sustained exposure to excessive levels of free glucocorticoids. One of the main causes of CS is excessive adrenocorticotropic hormone (ACTH) secretion by tumors in the pituitary gland (Cushing's disease [CD]). Cushing's disease and its associated hypercortisolism have a breadth of debilitating symptoms associated with an increased mortality rate, warranting urgent treatment. Currently, the first line of treatment for CD is transsphenoidal surgery (TSS), with excellent long-term results. Transsphenoidal resections performed by experienced surgeons have shown remission rates ranging from 70% to 90%. However, some patients do not achieve normalization of their hypercortisolemic state after TSS and continue to have persistent or recurrent CD. For these patients, various therapeutic options after failed TSS include repeat TSS, radiotherapy, medical therapy, and bilateral adrenalectomy (BLA). Bilateral adrenalectomy has been shown to be a safe and effective treatment modality for persistent or recurrent CD with an immediate and definitive cure of the hypercortisolemic state. BLA was traditionally performed through an open approach, but since the advent of laparoscopic adrenalectomy, the laparoscopic approach has become the surgical method of choice. Advances in technology, refinement in surgical skills, competency in adrenopathology, and emphasis on multidisciplinary collaborations have greatly reduced morbidity and mortality associated with adrenalectomy surgery in a high-risk patient population. In this article, the authors review the role of BLA in the treatment of refractory CD. The clinical indications, current surgical and endocrinological results reported in the literature, surgical technique (open vs laparoscopic), drawbacks, and complications of BLA are discussed.

Favorable long-term outcome of bilateral adrenalectomy in Cushing's disease

2014 ◽  
Author(s):  
Andrea Osswald ◽  
Eva Plomer ◽  
Christina Dimopoulou ◽  
Monika Milian ◽  
Rainer Blaser ◽  
...  
Keyword(s):  
Cushing’S Disease ◽  
Bilateral Adrenalectomy ◽  
Cushing's Disease ◽  
Term Outcome ◽  
Long Term Outcome

scholarly journals Bilateral adrenalectomy in Cushing's disease: Altered long-term quality of life compared to other treatment options

2019 ◽  
Vol 80 (1) ◽  
pp. 32-37 ◽  
Author(s):  
Pauline Sarkis ◽  
Muriel Rabilloud ◽  
Jean-Christophe Lifante ◽  
Anna Siamand ◽  
Emmanuel Jouanneau ◽  
...  
Keyword(s):  
Quality Of Life ◽  
Cushing’S Disease ◽  
Treatment Options ◽  
Bilateral Adrenalectomy ◽  
Cushing's Disease

scholarly journals Update on the management of recurrent Cushing's disease

2015 ◽  
Vol 38 (2) ◽  
pp. E16 ◽  
Author(s):  
Martin J. Rutkowski ◽  
Patrick M. Flanigan ◽  
Manish K. Aghi
Keyword(s):  
Patient Outcomes ◽  
Cushing’S Disease ◽  
Adrenocorticotropic Hormone ◽  
Bilateral Adrenalectomy ◽  
Cushing's Disease ◽  
Tumor Control ◽  
Repeat Resection ◽  
Risks And Benefits ◽  
Acth Secreting

After transsphenoidal surgery, Cushing's disease (CD) shows excellent long-term remission rates, but it may recur and pose a therapeutic challenge. Findings in recent published reports on the treatment of recurrent adrenocorticotropic hormone (ACTH)–secreting tumors suggest that repeat resection, radiation-based therapies such as Gamma Knife surgery and proton-beam radiosurgery, pharmacotherapy, and bilateral adrenalectomy all have important roles in the treatment of recurrent CD. Each of these interventions has inherent risks and benefits that should be presented to the patient during counseling on retreatment options. Radiation-based therapies increasingly appear to have efficacies similar to those of repeat resection in achieving biochemical remission and tumor control. In addition, an expanding retinue of medication-based therapies, several of which are currently being evaluated in clinical trials, has shown some promise as tertiary adjunctive therapies. Lastly, bilateral adrenalectomy may offer durable control of refractory recurrent CD. An increasing number of published studies with long-term patient outcomes highlight the evolving treatment patterns in the management of recurrent CD.

Long Term Results of Transsphenoidal Adenomectomy in Patients with Cushing's Disease

Neurosurgery ◽  
1987 ◽  
Vol 21 (2) ◽  
pp. 218-222 ◽  
Author(s):  
Toshichi Nakane ◽  
Akio Kuwayama ◽  
Masao Watanabe ◽  
Tatsuo Takahashi ◽  
Tetsuo Kato ◽  
...  
Keyword(s):  
Cushing’S Disease ◽  
Pituitary Adenomas ◽  
Remission Rate ◽  
Hormone Secretion ◽  
Late Recurrence ◽  
Long Term Results ◽  
Cushing's Disease ◽  
Cell Hyperplasia ◽  
Transsphenoidal Adenomectomy

Abstract As part of an ongoing series, 100 patients with Cushing's disease underwent transsphenoidal operations. Pituitary adenomas were confirmed in 93 patients, and initial remission was achieved in 86 (92%) of them. Hypercortisolemia was not corrected in 7 patients, and in 4 this was due to invasive adenomas. These patients were subjected to irradiation, medical treatment, or both after operation. Only 7 of the 100 patients had no pituitary adenoma found at operation, and they obtained no clinical remission even after partial or subtotal hypophysectomy. Follow-up review, with an emphasis on endocrinological studies, was performed on these patients for a mean period of 38 months. Seventy-eight patients were in long term remission after operation and had restoration of noncorticotropic hormone secretion as well as pituitary-adrenal function. Recurrence was noted in 8 patients after 19 to 82 months in remission. In all of these patients, pituitary adenomas were verified by reoperation and no case of corticotrophic cell hyperplasia was noted. We conclude that late recurrence of Cushing's disease may occur after adenoma removal and is due to the regrowth of adenoma cells left behind in the peritumoral tissue at the first operation. In view of the overall remission rate, transsphenoidal adenomectomy is considered a highly effective treatment for Cushing's disease.

scholarly journals Long-term results of treatment in patients with ACTH-secreting pituitary macroadenomas

2003 ◽  
pp. 195-200 ◽  
Author(s):  
S Cannavo ◽  
B Almoto ◽  
C Dall'Asta ◽  
S Corsello ◽  
RM Lovicu ◽  
...  
Keyword(s):  
Cavernous Sinus ◽  
Cushing’S Disease ◽  
Long Term Results ◽  
Cushing's Disease ◽  
Tumor Diameter ◽  
Pituitary Macroadenoma ◽  
Second Surgery ◽  
Acth Secreting

OBJECTIVE: Since Cushing's disease due to large pituitary tumors is rare, we evaluated biochemical characteristics at entry and the results of first surgical approach and of adjuvant therapeutic strategies during a long-term follow-up period. DESIGN: We studied 26 patients (nine male, 17 female; 42.5+/-12.7 years, mean+/-s.e.) with ACTH-secreting pituitary macroadenoma (tumor diameter: 11-40 mm). METHODS: At entry, plasma ACTH, serum cortisol and 24-h urinary free cortisol (UFC) levels were measured in all patients, a high-dose dexamethasone (dexa) suppression test was evaluated in 22 cases and a corticotrophin releasing hormone (CRH) test in 20 cases. Patients were re-evaluated after operation and, when not cured, they underwent second surgery, radiotherapy and/or ketoconazole treatment. The follow-up period was 78+/-10 months. RESULTS: Before surgery, dexa decreased ACTH (>50% of baseline) in only 14/22 patients. The CRH-stimulated ACTH/cortisol response was normal in six patients, impaired in six patients and exaggerated in eight patients. After operation eight patients were cured, nine had normalized cortisol levels and nine were not cured. Pre-surgery, mean ACTH values were significantly higher in the not cured patients than in those normalized (P<0.05) and cured (P<0.01); the ACTH response to CRH was impaired in only six patients of the not cured group. The tumour diameter was significantly less in cured patients (P<0.02) and in normalized patients (P<0.05) than in the not cured ones. Magnetic resonance imaging (MRI) showed invasion of the cavernous sinus in 2/9 normalized, and in 6/9 not cured patients. After surgery, ACTH, cortisol and UFC were significantly lower than at entry in cured and in normalized patients, but not in not cured patients. In the cured group, the disease recurred in one patient who was unsuccessfully treated with ketoconazole. In the normalized group, a relapse occurred in eight patients: radiotherapy and ketoconazole induced cortisol normalization in one case, hypoadrenalism in one case and were ineffective in another one, while five patients were lost at follow-up. In the not cured group, eight patients underwent second surgery, radiotherapy and/or ketoconazole, while one patient was lost at follow-up. These therapies induced cortisol normalization in two patients and hypoadrenalism in one. CONCLUSIONS: (i) A sub-set of patients with ACTH-secreting pituitary macroadenoma showed low sensitivity to high doses of dexamethasone and to CRH, (ii) pituitary surgery cured Cushing's disease in a minority of patients, (iii) high baseline ACTH levels, impaired ACTH response to CRH, increased tumor size or invasion of the cavernous sinus were unfavourable prognostic factors for surgical therapy, and (iv) second surgery, radiotherapy and/or ketaconazole cured or normalized hypercortisolism in half of the patients with recurrence or not cured.

scholarly journals Cushing’s disease: the desmopressin test as a compelling predictive marker of long-term remission after transsphenoidal surgery

2020 ◽  
Vol 182 (6) ◽  
pp. C17-C19
Author(s):  
D A Vassiliadi ◽  
S Tsagarakis
Keyword(s):  
Cushing’S Disease ◽  
Transsphenoidal Surgery ◽  
Predictive Marker ◽  
Positive Test ◽  
Cushing's Disease ◽  
Recent Issue ◽  
Recurrence Rates ◽  
Existing Data

Transsphenoidal surgery (TSS) is the treatment of choice in Cushing’s disease. However, recurrence rates are substantial and currently there are no robust predictors of late prognosis. As accumulating evidence challenge the accuracy of the traditionally used early postoperative cortisol values, alternative tests are required. The study of Cambos et al., published in a recent issue of the European Journal of Endocrinology, adds to the existing data that support a role of the desmopressin test as an early and reliable predictive marker in successfully TSS-treated patients. However, despite these promising data, the use of this test is hampered by the fact that it can be applied only in patients with a documented preoperative positive test. Moreover, the lack of robust criteria to define positive postoperative responses represents another major limitation.

scholarly journals MON-445 Long-Term Results of an Ongoing Non-Interventional, Real-World Observational Study of Pasireotide SC in Cushing's Disease

2019 ◽  
Vol 3 (Supplement_1) ◽  
Author(s):  
Kevin Yuen ◽  
Carla Giordano ◽  
Timo Deutschbein ◽  
Luca Manetti ◽  
Michael Roughton ◽  
...  
Keyword(s):  
Observational Study ◽  
Real World ◽  
Cushing’S Disease ◽  
Long Term Results ◽  
Cushing's Disease

scholarly journals Bilateral adrenalectomy: a review of 10 years’ experience

2017 ◽  
Vol 99 (2) ◽  
pp. 119-122 ◽  
Author(s):  
D Maccora ◽  
GV Walls ◽  
GP Sadler ◽  
R Mihai
Keyword(s):  
Cushing’S Disease ◽  
Operating Time ◽  
Laparoscopic Approach ◽  
Bilateral Adrenalectomy ◽  
Cushing's Disease ◽  
Adrenocortical Cancer ◽  
Infection Level ◽  
Tertiary Referral Centre ◽  
Postoperative Haemorrhage ◽  
Retroperitoneoscopic Adrenalectomy

INTRODUCTIONThe 2012 British Association of Endocrine and Thyroid Surgeons audit report showed that only 86 of 1359 patients who underwent adrenalectomy had a bilateral operation; thus the experience with this procedure remains limited.METHODSRetrospective review of patients undergoing bilateral adrenalectomy in a tertiary referral centre.RESULTSBetween November 2005 and January 2016, bilateral adrenalectomy was performed in 23 patients (6 male, 17 female, age 43 ± 4 years) diagnosed with Cushing’s disease (n = 13), hereditary phaeochromocytomas (n = 6), adrenocortical cancer (n = 2), colorectal metastatic disease (n = 1) and adrenocortical adenomas (n = 1). A laparoscopic transperitoneal approach was used in 17 patients, with one conversion to open. Three patients had open adrenalectomies for adrenocortical cancer and for simultaneous phaeochromocytomas and pancreatic neuroendocrine tumours in a patient with Von Hippel-Lindau syndrome. Three patients with Cushing’s had a bilateral retroperitoneoscopic operation. The mean operating time was 195 ± 16 minutes for laparoscopic operations (n = 16), 243 ± 44 minutes for open adrenalectomies (n = 4) and 151 ± 12 minutes for retroperitoneal operations. It was significantly shorter for Cushing’s disease than for phaeochromocytomas (162 ± 8 vs. 257 ± 39 minutes, P < 0.01). Median length of hospital stay was 5 days. Postoperative complications (Clavien-Dindo classification) included one chest infection (level 2), one postoperative haemorrhage and two chest drains for pneumothorax (level 3), two postoperative cardiac arrests (level 4) and one late cancer death from complications related to uncontrolled hypercortisolism (level 5).DISCUSSIONSynchronous bilateral adrenalectomy remains an infrequent operation. The laparoscopic approach is feasible in the majority of patients. It is likely that the retroperitoneoscopic adrenalectomy will become the standard approach for bilateral operations.

scholarly journals Transsphenoidal Microsurgery for Cushing’s Disease: Initial Outcome and Long-Term Results

2004 ◽  
Vol 89 (12) ◽  
pp. 6348-6357 ◽  
Author(s):  
Gary D. Hammer ◽  
J. Blake Tyrrell ◽  
Kathleen R. Lamborn ◽  
Carol B. Applebury ◽  
Elizabeth T. Hannegan ◽  
...  
Keyword(s):  
Cushing’S Disease ◽  
Long Term Results ◽  
Cushing's Disease ◽  
Transsphenoidal Microsurgery ◽  
Initial Outcome
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