Paediatric patients with Cushing disease and negative pituitary MRI have a higher risk of nonremission after transsphenoidal surgery

2021 ◽  
Author(s):  
Christina Tatsi ◽  
Maria E. Bompou ◽  
Chelsi Flippo ◽  
Meg Keil ◽  
Prashant Chittiboina ◽  
...  
Keyword(s):  
Transsphenoidal Surgery ◽  
Cushing Disease ◽  
Paediatric Patients

OUTCOMES OF TRANSSPHENOIDAL SURGERY IN CUSHING DISEASE PATIENTS WITH NEGATIVE PITUITARY MAGNETIC RESONANCE IMAGING FINDINGS: A SINGLE-CENTER EXPERIENCE

2020 ◽  
Vol 26 (11) ◽  
pp. 1320-1330
Author(s):  
Congxin Dai ◽  
Siyu Liang ◽  
Xiaohai Liu ◽  
Yanghua Fan ◽  
Xinjie Bao ◽  
...  
Keyword(s):  
Magnetic Resonance Imaging ◽  
Magnetic Resonance ◽  
Surgical Outcomes ◽  
Transsphenoidal Surgery ◽  
Remission Rate ◽  
Resonance Imaging ◽  
Mri Findings ◽  
College Hospital ◽  
Cushing Disease ◽  
Medical College

Objective: Transsphenoidal surgery (TSS) is a first-line treatment for Cushing disease (CD). However, a subset of patients with CD have no visible adenoma on magnetic resonance imaging (MRI), and whether MRI results affect surgical outcomes is controversial. The aim of this study was to compare the surgical outcomes of CD patients with negative MRI findings to those of patients with positive MRI findings. Methods: The clinical features and outcomes of CD patients who underwent TSS between January 2000 and July 2019 at Peking Union Medical College Hospital were collected from medical records. The clinical, endocrinologic, histopathologic, surgical outcomes, and a minimum 12-month follow-up of 125 consecutive CD patients with negative MRI findings were compared with those of 1,031 consecutive CD patients with MRI-visible adenomas. Results: The total remission rate was 73.3% after TSS, and 11.8% of patients experienced recurrence. Of 1,031 patients with MRI-visible adenomas, postoperative remission was achieved in 762 patients (73.9%), and the recurrence of CD was observed in 94 (12.3%) patients. Of the 125 patients with negative MRI findings, postoperative remission was achieved in 85 (68%) patients, and recurrence was observed in 6 (7.1%) patients. The remission rate and recurrence rate were not significantly different between patients with negative MRI findings and those with positive MRI findings (all P>.05). The remission rate was not significantly different between patients who did or did not undergo bilateral inferior petrosal sinus sampling (BIPSS) in patients with negative MRI findings ( P>.05). In the patients with negative MRI findings who underwent BIPSS, the remission rate of patients with positive BIPSS results was not different from that in patients with negative BIPSS results ( P>.05). The lack of prior TSS, the detection of a tumor during operation, and pathologic confirmation of adenoma were associated with a higher surgical remission rate in patients with negative MRI findings (all P<.05). Similar results were observed in the patients with positive MRI findings (all P<.05). In addition, the major perioperative complications, including intraoperative cerebrospinal fluid leakage, hypopituitarism, and transient diabetes insipidus, were not related to the MRI results (all P>.05). Conclusion: The remission rate and recurrence rate were not different between patients with negative MRI findings and those with positive MRI findings. If CD is clearly diagnosed according to biochemical tests, radiologic examinations, and BIPSS, we recommend TSS as the first-line treatment for patients, even if the MRI results are negative. Abbreviations: ACTH = adrenocorticotropic hormone; BIPSS = bilateral inferior petrosal sinus sampling; CD = Cushing disease; 18F-FDG = 18F-fluorodeoxyglucose; IQR = interquartile range; MRI = magnetic resonance imaging; PET = positron emission tomography; PUMCH = Peking Union Medical College Hospital; TSS = transsphenoidal surgery; UFC = urine free cortisol

Transsphenoidal surgery in Cushing disease: 10 years of experience in 34 consecutive cases

2004 ◽  
Vol 100 (4) ◽  
pp. 634-638 ◽  
Author(s):  
Charlotte Höybye ◽  
Eva GrenbäcK ◽  
Marja Thorén ◽  
Anna-Lena Hulting ◽  
Lars Lundblad ◽  
...  
Keyword(s):  
Mr Imaging ◽  
Transsphenoidal Surgery ◽  
Cushing Syndrome ◽  
Preoperative Evaluation ◽  
Clinical Signs ◽  
Pituitary Hormone ◽  
Years Of Experience ◽  
Cushing Disease ◽  
Content Type ◽  
Fine Print

Object. Cushing disease is a rare disorder. Because of their small size the adrenocorticotropic hormone (ACTH)—producing tumors are often not detectable on neuroimaging studies. To obtain a cure with transsphenoidal surgery (TSS) may therefore be difficult. In this report the authors present 10 years of experience in the treatment of patients with Cushing disease who were followed up with the same protocol and treated by the same surgeon. Methods. Thirty-four patients, 26 of them female and eight of them male (mean age 40 years, range 13–74 years) were studied. All had obvious clinical signs and symptoms of Cushing syndrome. Magnetic resonance (MR) imaging was performed in all patients, and inferior petrosal sinus (IPS) sampling was done in 14. In 12 patients MR imaging indicated a pituitary tumor; 10 were microadenomas and two were macroadenomas. In six patients with no visible tumor, the results of IPS sampling supported the diagnosis. All patients underwent TSS; the mean follow-up duration was 6 ± 0.5 years. Selective adenomectomy was performed in 32 and hemihypophysectomy in the other two patients. A cure was obtained in 31 patients (91%) after one TSS and in two more patients after further TSS; one patient was not cured despite two TSSs and one underwent bilateral adrenalectomy. Disease recurrence was seen in two patients after 3 years, and they were successfully treated with stereotactic gamma knife surgery. Half of the patients had an ACTH deficiency postoperatively, whereas one third had other pituitary hormone insufficiencies. There were no serious complications attributable to the surgical intervention. Conclusions. Transsphenoidal surgery with selective adenomectomy is an effective and safe treatment for Cushing disease. In the patients presented in this study, the surgical outcome seemed to depend on careful preoperative evaluation and the surgeon's experience. For optimal results in this rare disease the authors therefore suggest that the endocrinological, radiological, and surgical procedures be coordinated in a specialized center.

Calcific Periarthritis in a Patient With Previous Cushing Disease Treated With Transsphenoidal Surgery

2020 ◽  
Vol Publish Ahead of Print ◽  
Author(s):  
Sara Ganhão ◽  
Jacqueline Uson
Keyword(s):  
Transsphenoidal Surgery ◽  
Cushing Disease ◽  
Calcific Periarthritis

scholarly journals Cushing Disease: Are We Making Progress?

2017 ◽  
Vol 102 (7) ◽  
pp. 2133-2135 ◽  
Author(s):  
Laurence Kennedy ◽  
Pablo F. Recinos
Keyword(s):  
Transsphenoidal Surgery ◽  
Cushing Disease

This paper comments on how we can assess the success of transsphenoidal surgery for patients with Cushing disease.

scholarly journals Transsphenoidal Surgery for Cushing Disease After Nondiagnostic Inferior Petrosal Sinus Sampling

Neurosurgery ◽  
2012 ◽  
Vol 71 (1) ◽  
pp. 14-22 ◽  
Author(s):  
Sameer A. Sheth ◽  
Matthew K. Mian ◽  
Jonathan Neal ◽  
Nicholas A. Tritos ◽  
Lisa Nachtigall ◽  
...  
Keyword(s):  
Predictive Value ◽  
Transsphenoidal Surgery ◽  
Adrenocorticotropic Hormone ◽  
Remission Rate ◽  
Inferior Petrosal Sinus ◽  
Cushing Disease ◽  
Inferior Petrosal Sinus Sampling ◽  
Course Of Action ◽  
Anatomic Variants ◽  
Sensitivity Specificity

Abstract BACKGROUND: Inferior petrosal sinus sampling (IPSS) is a useful technique for confirming a pituitary source of adrenocorticotropic hormone (ACTH) overproduction in Cushing disease. Uncertainty remains regarding the appropriate course of therapy when an ectopic tumor is predicted by IPSS but none can be found and in circumstances when the procedure cannot be successfully completed owing to technical or anatomic limitations. OBJECTIVE: To determine an appropriate course of action after nondiagnostic IPSS. METHODS: We reviewed 288 IPSS procedures in 283 patients between 1986 and 2010 at our center. An IPS:peripheral ACTH ratio ≥ 2 at baseline or ≥ 3 after corticotrophin-releasing hormone was considered predictive of a pituitary source of ACTH. A procedure was considered nondiagnostic if the procedure was successfully performed and the results predicted an ectopic source but none could be found despite extensive imaging or if the IPS could not be bilaterally cannulated because of technical difficulties or anatomic variants. RESULTS: The sensitivity, specificity, positive predictive value, and negative predictive value of IPSS for detecting a pituitary source in Cushing disease were 94%, 50%, 98%, and 29%, respectively. We identified 3 categories of nondiagnostic IPSS comprising 44 of the total procedures. These patients underwent exploratory transsphenoidal surgery, and in 42 of these patients (95%), a pituitary source was surgically proven, with a remission rate of 83%. CONCLUSION: Transsphenoidal surgery should be considered in cases of ACTH-dependent Cushing disease and noncentralized or technically unsuccessful IPSS without evidence of ectopic tumor.

Complications after Transsphenoidal Surgery for Cushing Disease

2015 ◽  
Vol 76 (S 01) ◽  
Author(s):  
Timothy Smith ◽  
M. Hulou ◽  
Kevin Huang ◽  
Breno Nery ◽  
Edward Laws
Keyword(s):  
Transsphenoidal Surgery ◽  
Cushing Disease

Outcome of Transsphenoidal Surgery for Cushing Disease: A Single-Center Experience over 20 Years

2018 ◽  
Vol 119 ◽  
pp. e106-e117 ◽  
Author(s):  
Camille Brichard ◽  
Emmanuel Costa ◽  
Edward Fomekong ◽  
Dominique Maiter ◽  
Christian Raftopoulos
Keyword(s):  
Transsphenoidal Surgery ◽  
Single Center ◽  
Cushing Disease ◽  
Single Center Experience

Outcome of Microscopic Transsphenoidal Surgery in Cushing Disease: A Case Series of 96 Patients

2016 ◽  
Vol 87 ◽  
pp. 170-175 ◽  
Author(s):  
Manochehr Shirvani ◽  
Rouzbeh Motiei-Langroudi ◽  
Homa Sadeghian
Keyword(s):  
Transsphenoidal Surgery ◽  
Case Series ◽  
Cushing Disease

scholarly journals Treatment options for Cushing disease after unsuccessful transsphenoidal surgery

2007 ◽  
Vol 23 (3) ◽  
pp. 1-7 ◽  
Author(s):  
James K. Liu ◽  
Maria Fleseriu ◽  
Johnny B. Delashaw ◽  
Ivan S. Ciric ◽  
William T. Couldwell ◽  
...  
Keyword(s):  
Radiation Therapy ◽  
Transsphenoidal Surgery ◽  
Treatment Options ◽  
Pituitary Tumors ◽  
Premature Death ◽  
Treatment Modalities ◽  
Definitive Treatment ◽  
Sustained Remission ◽  
Cushing Disease ◽  
Acth Secreting

✓Cushing disease is considered an aggressive pituitary endocrinopathy because of the devastating effects from untreated hypercortisolemia. Although they are histologically benign, these adrenocorticotropic hormone (ACTH)-secreting pituitary tumors are associated with significant morbidity and premature death. Currently, transsphenoidal surgery is the primary treatment of Cushing disease associated with an ACTH-secreting pituitary tumor, resulting in remission rates ranging from about 50 to 90%. Some patients, however, will not achieve sustained remission after transsphenoidal surgery and can exhibit persistent or recurrent Cushing disease that requires multimodal treatment to achieve remission. In these patients, options for treatment include repeat transsphenoidal resection, radiation therapy (including conventional fractionated radiation therapy and stereotactic radiosurgery), and medical therapy. Despite undergoing multiple treatment modalities, some patients may ultimately require bilateral adrenalectomy for definitive treatment to eliminate hypercortisolemia associated with Cushing disease. In this article, the authors review the treatment options for patients who have persistent or recurrent Cushing disease after unsuccessful transsphenoidal surgery. The indications, current results reported in the literature, and complications of each treatment modality are discussed.

Repeated Transsphenoidal Surgery or Gamma Knife Radiosurgery in Recurrent Cushing Disease After Transsphenoidal Surgery

2013 ◽  
Vol 75 (02) ◽  
pp. 091-097 ◽  
Author(s):  
Mohammad Bodaghabadi ◽  
Hooman Riazi ◽  
Shima Aran ◽  
Mazdak Alikhani ◽  
Mahmud Alahverdi ◽  
...  
Keyword(s):  
Gamma Knife ◽  
Transsphenoidal Surgery ◽  
Gamma Knife Radiosurgery ◽  
Cushing Disease
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