scholarly journals Cushing’s Disease due to ACTH Secreting Pituitary Microadenoma: A Single Institutional Experience of Four Cases

2020 ◽  
Author(s):  
Ashok Gandhi ◽  
Mayur V. Barhate ◽  
Shashikant K. Jain ◽  
Sapna A. Gandhi
Keyword(s):  
Postoperative Period ◽  
Cushing’S Disease ◽  
Signs And Symptoms ◽  
Cushing's Disease ◽  
Pathological Examination ◽  
Endoscopic Endonasal ◽  
Pituitary Microadenoma ◽  
Institutional Experience ◽  
Endoscopic Endonasal Transsphenoidal Surgery ◽  
Acth Secreting

AbstractACTH secreting pituitary adenomas are rare occurrences in autoptical, surgical and neuroradiological series. We herewith describe a series of four patients of Cushing’s disease due to ACTH secreting pituitary microadenoma. All patients presented with signs and symptoms of hypercortisolism. Hormonal testing and bilateral inferior petrosal sinus sampling were indicative of pituitary-dependent Cushing`s syndrome. Dynamic sellar MRI was suggestive of asymmetric pituitary, with suspect pituitary microadenoma in all cases. Three patients were operated for endoscopic endonasal transsphenoidal surgery, while one patient refused surgery and was managed with ketoconazole. During postoperative period, remission of hypercortisolism was ensured in two patients, while one patient continued to have raised levels of cortisol. One patient with raised levels of cortisol during postoperative period was prescribed Ketoconazole for 6 months. Histopathology confirmed the presence of pituitary adenoma. Adenoma displayed ACTH immunoreactivity. Our cases indicate that ACTH secreting pituitary microadenoma may occur in patients with Cushing̓s disease. Careful radiological, surgical and pathological examination is necessary to recognize this condition and avoid surgical failure.

Efficacy of endoscopic endonasal transsphenoidal surgery for Cushing’s disease in 230 patients with positive and negative MRI

2017 ◽  
Vol 159 (7) ◽  
pp. 1227-1236 ◽  
Author(s):  
Hélène Cebula ◽  
Bertrand Baussart ◽  
Chiara Villa ◽  
Guillaume Assié ◽  
Anne Boulin ◽  
...  
Keyword(s):  
Cushing’S Disease ◽  
Transsphenoidal Surgery ◽  
Cushing's Disease ◽  
Endoscopic Endonasal ◽  
Endoscopic Endonasal Transsphenoidal Surgery

scholarly journals Value of pituitary gland MRI at 7 T in Cushing’s disease and relationship to inferior petrosal sinus sampling: case report

2019 ◽  
Vol 130 (2) ◽  
pp. 347-351 ◽  
Author(s):  
Meng Law ◽  
Regina Wang ◽  
Chia-Shang J. Liu ◽  
Mark S. Shiroishi ◽  
John D. Carmichael ◽  
...  
Keyword(s):  
Cushing’S Disease ◽  
Clinical Symptoms ◽  
Cushing's Disease ◽  
Inferior Petrosal Sinus ◽  
Contrast Imaging ◽  
Pituitary Mass ◽  
Endoscopic Endonasal ◽  
Inferior Petrosal Sinus Sampling ◽  
Pituitary Microadenoma ◽  
Pituitary Lesion

Cushing’s disease is caused by adrenocorticotrophic hormone (ACTH)–secreting pituitary adenomas, which are often difficult to identify on standard 1.5-T or 3-T MRI, including dynamic contrast imaging. Inferior petrosal and cavernous sinus sampling remains the gold standard for MRI-negative Cushing’s disease.The authors report on a 27-year-old woman with Cushing’s disease in whom the results of standard 1.5-T and 3-T MRI, including 1.5-T dynamic contrast imaging, were negative. Inferior petrosal sinus sampling showed a high central-to-peripheral ACTH ratio (148:1) as well as a right-to-left ACTH gradient (19:1), suggesting a right-sided pituitary microadenoma. The patient underwent 7-T MRI, which showed evidence of a right-sided pituitary lesion with focal hypoenhancement not visualized on 1.5-T or 3-T MRI. The patient underwent an endoscopic endonasal transsphenoidal operation, with resection of a right-sided pituitary mass. Postoperatively, she developed clinical symptoms suggestive of adrenal insufficiency and a nadir cortisol level of 1.6 μg/dl on postoperative day 3, and hydrocortisone therapy was initiated. Permanent histopathology specimens showed Crooke’s hyaline change and ACTH-positive cells suggestive of an adenoma.MRI at 7 T may be beneficial in identifying pituitary microadenoma location in cases of standard 1.5-T and 3-T MRI-negative Cushing’s disease. In the future, 7-T MRI may preempt inferior petrosal sinus sampling and help in cases of standard and dynamic contrast 1.5-T and 3-T MRI-negative Cushing’s disease.

Surgical management of adrenocorticotropic hormone—secreting macroadenomas: outcome and challenges in patients with Cushing's disease or Nelson's syndrome

2005 ◽  
Vol 103 (5) ◽  
pp. 825-830 ◽  
Author(s):  
Claudio De Tommasi ◽  
Mary Lee Vance ◽  
David O. Okonkwo ◽  
Alfa Diallo ◽  
Edward R. Laws
Keyword(s):  
Cushing’S Disease ◽  
Adrenocorticotropic Hormone ◽  
Signs And Symptoms ◽  
Disease Recurrence ◽  
Cushing's Disease ◽  
Nelson’S Syndrome ◽  
Content Type ◽  
Nelson's Syndrome ◽  
Acth Secreting ◽  
Fine Print

Object. Adrenocorticotropic hormone (ACTH)—secreting pituitary macroadenomas are an uncommon cause of Cushing's disease (CD) and, subsequently, Nelson's syndrome (NS). They have been associated with low postoperative remission rates. The outcome of modern surgical treatment is unclear and thus was assessed in a series of 43 patients, with the goal of improving therapeutic results in patients with ACTH-secreting macroadenomas. Methods. Thirty-seven patients presented with CD and six with NS. They represented 15% of the patients surgically treated at the authors' institution for ACTH-secreting adenomas. The median patient age was 38 years (range 14–71 years), and the mean duration of follow up was 37 months (range 1–108 months). Remission occurred in 25 (67.6%) of 37 patients with CD, whereas the disease persisted in 12 (32.4%) of 37 patients. After an initial remission, three (12%) of 25 patients demonstrated signs and symptoms indicative of disease recurrence. Among patients with NS, only one (16.6%) displayed remission. Invasion of the dura mater by tumor was histologically demonstrated in 10 patients with CD and in two patients with NS. Conclusions. Comprehensive management of CD caused by ACTH-secreting macroadenomas through the appropriate use of combination therapy, including surgery, radiotherapy, radiosurgery, and adrenalectomy, can lead to outcomes similar to those for microadenomas. Disease recurrence and persistence rates are higher, often because of the invasiveness associated with macroadenomas. Remission of NS-associated macroadenomas is difficult to achieve. Strategies for the characterization and treatment of invasive macroadenomas are needed.

scholarly journals ICA Occlusion by an ACTH- secreting pituitary adenoma post-TSS and irradiation

2020 ◽  
Vol 13 (1) ◽  
Author(s):  
Diala El-Zammar ◽  
Ryojo Akagami
Keyword(s):  
Pituitary Adenoma ◽  
Cushing’S Disease ◽  
Pituitary Tumour ◽  
Cushing's Disease ◽  
Suppressive Therapy ◽  
Pathological Examination ◽  
Pituitary Macroadenoma ◽  
The Right ◽  
Acth Secreting

Occlusion of intracranial arteries by a pituitary adenoma with ensuing infarction is a rare occurrence. In this case study, we show the instance of a pituitary  macroadenoma and apoplexy causing mechanical obstruction of the internal carot- id artery with consequent infarction following transphenoidal surgery (TSS) and ra- diation therapy in a patient with Cushing’s disease. we report a 44-year-old woman  presented with amenorrhea and headaches. Necessary investigations, resection by  TSS, and microscopic examination revealed an adenocorticotropin (ACTH)-secret- ing pituitary macroadenoma. The pituitary tumour recurred in subsequent years,  resulting in the development of Cushing’s disease and syndrome. despite two more  transphenoidal surgeries, radiotherapy, and medical suppressive therapy, the pi- tuitary adenoma continued to enlarge, and the hypercortisolemia and Cushingoid  symptoms persisted. A craniotomy was arranged as the next step in the treatment strategy. Only hours prior to the scheduled surgery, the patient developed left-sided hemiplegia, was diagnosed with acute occlusion of the right ICA and underwent an emergency bifrontal craniotomy with evacuation of the tumour and decompression.  Pathological examination revealed evidence of apoplexy in the ACTH-secreting pi- tuitary adenoma. This case demonstrates the vast scope of complications that can  arise from pituitary adenomas despite combination therapy and forewarns clinicians to be prepared to manage these infrequent but conceivable occurrences.

scholarly journals Clinical, Biological, Radiological Pathological and Immediate Post-Operative Remission of Sparsely and Densely Granulated Corticotroph Pituitary Tumors: A Retrospective Study of a Cohort of 277 Patients With Cushing’s Disease

2021 ◽  
Vol 12 ◽  
Author(s):  
Beata Rak ◽  
Maria Maksymowicz ◽  
Monika Pękul ◽  
Grzegorz Zieliński
Keyword(s):  
Cushing’S Disease ◽  
Pituitary Tumors ◽  
Signs And Symptoms ◽  
Cortisol Concentration ◽  
Cushing's Disease ◽  
Ki 67 ◽  
Remission Status ◽  
Multiple Data ◽  
The Mean ◽  
Corticotroph Adenomas

PurposeCushing’s disease is the most common cause of endogenous hypercortisolemia due to a corticotroph pituitary tumor. Up-to-date there is no reliable biomarker of invasiveness among corticotroph tumors, while it is well established in the literature that sparsely granulated somatotroph tumors are characterized by poorer prognosis. The aim of the study was to correlate multiple data including clinical, biochemical, radiological, and pathological findings (including granulation pattern) as well as immediate post-operative remission status among patients operated on due to corticotroph tumors.MethodsWe enrolled all patients consecutively operated on for planned transsphenoidal neurosurgery due to corticotroph PitNETs in years 2010–2018. We excluded from analysis silent corticotroph tumors, plurihormonal PitNETs, and the Crooke’s cell adenomas.ResultsWe recorded 348 hormonally active corticotroph PitNETs. The results of the analysis showed the female predominance 79.88% (n = 278), with the mean age of Cushing’s disease occurrence 43.27 years of age. The mean time from the first signs and symptoms to the operation was 2 years. The women were diagnosed earlier (20–40 years of age vs. 50–60 years of age among men). We performed a detailed analysis of 277 cases classified by granularity pattern as DG or SG corticotroph PitNETs. Densely granulated tumors (DG) occurred four times more frequently than sparsely granulated (SG) (n = 225 vs. n = 52), at similar age (mean 42.94; median 40 vs. mean 45.46; median 45.5; p = 0.3896), but were characterized by lower Knosp’s scale grades (p = 0.0147*), smaller preoperative tumors’ volumes measured at MRI, and more commonly exhibited lower Ki-67 labeling index (<3%) (p = 0.0168*). What is more, DG adenomas more frequently achieved an immediate remission status (measured as postoperative cortisol concentration <2 µg/dl; p = 0.0180*), and the mean postoperative cortisol concentration in DG group was lower than in SG group (mean 5.375 µg/dl vs. 10.47 µg/dl; median 2.49 µg/dl vs. 6.52 µg/dl; p = 0.0028**).ConclusionsOur study indicates that DG corticotroph adenomas occurred at younger age, more commonly were microadenomas as compared to SG tumors, less frequently had invasive features in comparison to SG corticotroph adenomas (p = 0.0019**), and more commonly achieved an immediate postsurgical hormonal remission (p = 0.0180*). We highlight the need for an accurate differentiation of DG and SG subtypes in the pathomorphological diagnosis of corticotropic tumors, especially in invasive PitNETs.

scholarly journals MANAGEMENT OF ENDOCRINE DISEASE: Paediatric Cushing's disease

2015 ◽  
Vol 173 (1) ◽  
pp. R35-R45 ◽  
Author(s):  
Helen L Storr ◽  
Martin O Savage
Keyword(s):  
Early Diagnosis ◽  
Clinical Diagnosis ◽  
Cushing’S Disease ◽  
Therapeutic Strategy ◽  
Cushing's Disease ◽  
Significant Morbidity ◽  
Endocrine Disease ◽  
Definition Of ◽  
Acth Secreting ◽  
Biochemical Features

Cushing's disease (CD) is the commonest form of ACTH-dependent Cushing's syndrome and is a rare clinical diagnosis in paediatric and adolescent patients. CD is caused by an ACTH-secreting pituitary corticotroph adenoma and is associated with significant morbidity in children; therefore, early diagnosis and treatment are critical for optimal therapeutic outcome. This review highlights the key clinical and biochemical features of paediatric CD and appraises current practices in diagnosis and management. A close liaison with adult endocrinology colleagues, particularly, for interpretation of investigations and definition of therapeutic strategy is strongly advised.

scholarly journals Treatment effectiveness of pasireotide on health-related quality of life in patients with Cushing's disease

2014 ◽  
Vol 171 (1) ◽  
pp. 89-98 ◽  
Author(s):  
Susan M Webb ◽  
John E Ware ◽  
Anna Forsythe ◽  
Min Yang ◽  
Xavier Badia ◽  
...  
Keyword(s):  
Quality Of Life ◽  
Cushing’S Disease ◽  
Treatment Effectiveness ◽  
Clinical Signs ◽  
Signs And Symptoms ◽  
Cushing's Disease ◽  
Health Related Quality ◽  
Related Quality ◽  
Health Related

ObjectiveCushing's disease (CD) can significantly impair patients' health-related quality of life (HRQOL). This study investigated the treatment effectiveness of pasireotide on HRQOL of CD patients, and assessed the relationships between HRQOL and urinary free cortisol (UFC) and CD-related signs and symptoms.DesignIn this phase III, randomized, double-blind study, patients with UFC ≥1.5×upper limit of normal (ULN) received s.c. pasireotide 600 or 900 μg twice daily. The trial primary endpoint was UFC at or below ULN at month 6 without dose titration. Open-label treatment continued through month 12. HRQOL was measured using the Cushing's Quality of Life Questionnaire (CushingQoL) instrument at baseline and follow-up visits until month 12 during which clinical signs and features of CD, and the Beck Depression Inventory II (BDI-II), were also collected.MethodsPearson's/Spearman's correlations between changes in CushingQoL and changes in clinical signs and symptoms were assessed. Changes in CushingQoL and the proportion of patients achieving a clinically meaningful improvement in CushingQoL were also compared among patients stratified by mean UFC (mUFC) control status (controlled, partially controlled, and uncontrolled) at month 6. Analyses were also conducted at month 12, with multivariable adjustment for baseline characteristics and CushingQoL.ResultsChange in CushingQoL was significantly correlated with changes in mUFC (r=−0.40), BMI (r=−0.39), weight (r=−0.41), and BDI-II (r=−0.54) at month 12 but not at month 6. The percentage of CushingQoL responders at month 12 based on month 6 mUFC control status were as follows: 63, 58.8, and 37.9% in the controlled, partially controlled, and uncontrolled groups respectively. Adjusted CushingQoL scores at month 12 were 58.3 for controlled patients (Δ=11.5 vs uncontrolled,P=0.012) and 54.5 for partially controlled patients (Δ=7.7 vs uncontrolled,P=0.170).ConclusionsPasireotide treatment can result in a meaningful HRQOL improvement among those who complete a 12-month treatment period, most often among patients achieving biochemical control.

Immunochistochemical characteristics of blood vessels in non-visualized and visualized on MRI pituitary adenoma in patients with Cushing’s disease

2016 ◽  
Vol 62 (5) ◽  
pp. 65-66
Author(s):  
Patimat M. Khandaeva ◽  
Iya A. Voronkova ◽  
Zhanna E. Belaya ◽  
Lyudmila Y. Rozhinskaya ◽  
Aleksandr V. Vorontsov ◽  
...  
Keyword(s):  
Pituitary Adenoma ◽  
Blood Vessels ◽  
Cushing’S Disease ◽  
Pituitary Tumor ◽  
Pituitary Tumors ◽  
Average Diameter ◽  
Cushing's Disease ◽  
Retrospective Evaluation ◽  
Acth Secreting ◽  
Microvessels Density

Backgraund. Regardless of improvements in MRI, up to 20% of ACTH-secreting pituitary tumors are only identified at surgical exploration.Aim: to estimate whether there is any difference in blood vessels and the subsequent ability to uptake contrast agent in visualized microadenoma as compared to non-visualized on MRI ACTH-secreting pituitary tumors.Materials and methods. retrospective evaluation of ACTH-positive pituitary tumors from patients with Cushing’s disease (n=39) with either non-visualized pituitary tumor on MRI (n=17) or pituitary tumor less then 25 mm (n=22). MRI was performed using Siemens Magnetom Harmony 1.0T with gadolinium. Selected tumors were stained with anty-СD34 antibody (clone QBEnd/10, RTU, Leica) and anty-D2-40 antibody (clone D2-40, RTU, Dako). We evaluated the microvessels density and measured the diameter of larger and smaller vessel.Results. The microvessels density were not different in subject with visualized (123 [77;136]) and non-visualized (112 [110,0;126,5]) pituitary adenomas as well as number of slit-shaped vessels (32 [5;50] in visualized vs 25 [5;50] in non-visualized pituitary adenoma). The diameter of these vessels also did not differ: the diameter of the largest vessels in patients without visualization 53 µm [32,5;63,5] vs 33 µm [30,0;51,5], the average diameter of the blood vessels 15 µm [14,5-26,0] against 13 µm [12;14].Conclusions. The diameter and microvessels density in ACTH-producing pituitary adenoma does not affect the visualization of adenoma on MRI in patients with Cushing 's disease.

Cushing's Disease in Childhood: Benign Intracranial Hypertension after Trans-sphenoidal Adenomectomy

Neurosurgery ◽  
1983 ◽  
Vol 13 (2) ◽  
pp. 195-198 ◽  
Author(s):  
Mark N. Weissman ◽  
Larry K. Page ◽  
Raphael L. Bejar
Keyword(s):  
Intracranial Hypertension ◽  
Steroid Therapy ◽  
Cushing’S Disease ◽  
Adrenocorticotropic Hormone ◽  
Serum Cortisol ◽  
Cushing's Disease ◽  
Benign Intracranial Hypertension ◽  
Pituitary Microadenoma

Abstract A 7-year-old girl presented with the physical and endocrinological stigmata of Cushing's disease. An adrenocorticotropic hormone (ACTH)-producing pituitary microadenoma was excised. Three weeks after trans-sphenoidal adenomectomy, the patient developed benign intracranial hypertension. Although ACTH levels had decreased to normal, the serum cortisol had fallen to subnormal levels. The child responded to exogenous steroid therapy, which was gradually tapered and discontinued after 5 months. Normal pituitary and adrenal functions persist 2 years later.

scholarly journals Cyclic Cushing's disease with misleading inferior petrosal sinus sampling results during a trough phase

2015 ◽  
Vol 38 (2) ◽  
pp. E7 ◽  
Author(s):  
Vivien Bonert ◽  
Namrata Bose ◽  
John D. Carmichael
Keyword(s):  
Cushing’S Syndrome ◽  
Cushing’S Disease ◽  
Adrenocorticotropic Hormone ◽  
Diagnostic Testing ◽  
Cushing's Syndrome ◽  
Cushing's Disease ◽  
Inferior Petrosal Sinus ◽  
Inferior Petrosal Sinus Sampling ◽  
Acth Secreting ◽  
Transsphenoidal Resection

Diagnosing Cushing's syndrome is challenging and is further hampered when investigations are performed in a patient with cyclic Cushing's syndrome. A subset of patients with Cushing's syndrome exhibit periods of abnormal cortisol secretion with interspersed normal secretion. Patients can have periods of clinical improvement during these quiescent phases or remain symptomatic. Initial diagnostic testing can be challenging because of the unpredictable durations of the peak and trough phases, and it is especially challenging when the diagnosis of cyclic Cushing's syndrome has not yet been determined. Here, the authors present the case of a patient with Cushing's disease with a pathology-proven adrenocorticotropic hormone (ACTH)–secreting pituitary adenoma and whose initial inferior petrosal sinus sampling (IPSS) results were deemed indeterminate; further studies elucidated the diagnosis of cyclic Cushing's syndrome. Repeat IPSS was diagnostic of a central source for ACTH secretion, and the patient was treated successfully with transsphenoidal resection. Literature concerning the diagnosis and management of cyclic Cushing's syndrome is also reviewed.

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