scholarly journals Recovery of Adrenal Function after Pituitary Surgery in Patients with Cushing Disease: Persistent Remission or Recurrence?

2019 ◽  
Vol 108 (3) ◽  
pp. 211-218 ◽  
Author(s):  
Andreea Liliana Serban ◽  
Elisa Sala ◽  
Giulia Carosi ◽  
Giulia Del Sindaco ◽  
Claudia Giavoli ◽  
...  
Keyword(s):  
Surgical Intervention ◽  
Pituitary Surgery ◽  
Disease Recurrence ◽  
Adrenal Function ◽  
Surgical Removal ◽  
Cushing Disease ◽  
Adult Age ◽  
First Line Therapy ◽  
Acth Secreting

Background: Cushing disease (CD) represents the principal cause of endogenous hypercortisolism. The first-line therapy of CD is surgical removal of the ACTH-secreting pituitary adenoma, which is generally followed by adrenal insufficiency (AI). Objective: To analyze the recovery of AI in patients with CD after pituitary surgery in relation with recurrence and persistent remission of CD. Patients and Methods: We performed a retrospective analysis of patients with CD who met the following inclusion criteria: adult age, presence of AI 2 months after the surgical intervention, and a minimum follow-up of 3 years after the surgical intervention. Results: Sixty-one patients were followed for a median of 6 years. Ten (16.4%) patients recurred during follow-up. The patients who restored adrenal function did so after a median time of 19 months, with a significantly shorter time in the recurrence group (12.5 vs. 25 months, p = 0.008). All 10 patients who recurred recovered their adrenal function within 22 months. The recovery rate of AI in the persistent remission group was 37.3% (19/51) at 3 years and 55.8% (24/43) at 5 years. In all patients the duration of AI was negatively associated with disease recurrence. Conclusion: The duration of postsurgical AI in patients with recurrent CD is significantly shorter than that in patients with persistently remitted CD, and this parameter may be a useful predictor of recurrence. Patients showing a normal pituitary-adrenal axis within 2 years after surgery should be strictly monitored as they are at higher risk of disease relapse.

Recovery of the adrenal function after pituitary surgery in patients with Cushing Disease: remission or recurrence?

2018 ◽  
Author(s):  
Andreea Serban ◽  
Elisa Verrua ◽  
Elisa Sala ◽  
Marco Locatelli ◽  
Maura Arosio ◽  
...  
Keyword(s):  
Pituitary Surgery ◽  
Adrenal Function ◽  
Cushing Disease ◽  
Disease Remission

Treatment of recurrent Langerhans cell histiocytosis of the vulva with lenalidomide.

2013 ◽  
Vol 31 (15_suppl) ◽  
pp. e16555-e16555 ◽  
Author(s):  
Ibrahim Fuad Ibrahim ◽  
Harris V. K. Naina
Keyword(s):  
Rare Disease ◽  
Langerhans Cell Histiocytosis ◽  
Symptom Control ◽  
Female Genital Tract ◽  
Disease Recurrence ◽  
Langerhans Cell ◽  
Effective Treatment Option ◽  
Distant Disease ◽  
First Line Therapy

e16555 Background: Langerhans cell histiocytosis (LCH) of the female genital tract is an extremely rare disease. We report a 14 year follow up of a patient with LCH successfully treated with lenalidomide in the relapsed setting. Methods: N/A. Results: A 43 year old Ethiopian woman initially presented to another institution with a nodular lesion on her left vulva with biopsy consistent with LCH. Metastatic workup at the time did not reveal evidence of distant disease. The patient was initially treated with radiotherapy to the vulva and 2 years later she was diagnosed with recurrent disease in the vulva and underwent a wide local excision. Six months afterwards she developed a lesion on her right labium majus consistent with disease recurrence. She was treated with radiotherapy again and underwent a wide radical vulvar excision. She recurred only 3 months later and was started on salvage therapy with thalidomide, 3 years after the initial diagnosis. Within 2 months of starting thalidomide therapy, the patient experienced resolution of vulvar lesions and symptoms. She remained symptom free for 8 years while on thalidomide but then presented to our institution with a new central vulvar lesion after being off therapy for 4 months. Vulvar biopsy confirmed the presence of LCH. She was subsequently restarted on thalidomide and achieved symptom control for an additional 18 months before developing worsening pain and neuropathy associated with therapy. Imaging revealed no evidence of metastatic disease. We then started lenalidomide therapy at dose of 10 mg daily for 21 days in a 28 day cycle and subsequently increased the dose to 25 mg daily continuously. She achieved marked improvement in vulvar symptoms within a month of initiation of lenalidomide. She has tolerated therapy well with no neuropathy or cytopenias. Conclusions: Primary vulvar LCH is a rare disease with no standard therapies. Thalidomide has been described in the literature as an effective treatment option. We describe the use of lenalidomide as an alternative and well tolerated therapy in the relapsed setting. Further investigation is required to determine whether lenalidomide is a viable first line therapy for this rare disease.

scholarly journals Current state of the art in the diagnosis and surgical treatment of Cushing disease: early experience with a purely endoscopic endonasal technique

2007 ◽  
Vol 23 (3) ◽  
pp. 1-8 ◽  
Author(s):  
Amir R. Dehdashti ◽  
Fred Gentili
Keyword(s):  
Surgical Treatment ◽  
Cavernous Sinus ◽  
Pituitary Surgery ◽  
Magnetic Resonance Images ◽  
Primary Therapy ◽  
Cushing Disease ◽  
Endoscopic Endonasal ◽  
Number Of Patients ◽  
Free Cortisol

Object Transsphenoidal pituitary surgery is the primary therapy for Cushing disease because of its potential to produce lasting remission without the need for long-term drug or hormone replacement therapy. The authors evaluated the current role of pure endoscopic endonasal pituitary surgery in the treatment of Cushing disease. Methods Twenty-five patients underwent pure endoscopic surgery for confirmed Cushing disease. Thirteen patients had microadenomas and seven had macroadenomas; magnetic resonance images obtained in five patients were only suspicious or nondiagnostic, and thus they underwent inferior petrosal sinus sampling. Two patients had evidence of cavernous sinus involvement. Final histological results were consistent with adrenocorticotropin hormone (ACTH)–secreting adenoma in 20 patients. Results Twenty patients (80%) had clinical remission and laboratory confirmation of hypocortisolemia (serum cortisol < 100 nmol/L requiring substitution therapy), suppression to low-dose dexamethasone, and normal 24-hour urinary free cortisol. The median follow-up period was 17 months (range 3–32 months). There was no recurrence at the time of the last follow-up. Three patients presented with new anterior pituitary deficiency, but no one had permanent diabetes insipidus. In one patient a cerebrospinal fluid leak developed but later resolved following lumbar drainage. Treatment failure was attributable to involvement of the cavernous sinus in two patients, incomplete tumor removal in one, negative exploration in one, and nodular corticotroph hyperplasia of the pituitary gland in one. Conclusions Early results indicated that endoscopic endonasal surgery is a safe and effective treatment for ACTH-producing adenomas. The rate of remission in this study is comparable to those in previous series, and the rate of major postoperative complications is extremely low. Further studies with a larger number of patients and longer follow-ups are required to determine whether this more minimally invasive pure endoscopic approach should become the standard of care for the surgical treatment of Cushing disease.

scholarly journals Outcome of using the histological pseudocapsule as a surgical capsule in Cushing disease

2009 ◽  
Vol 111 (3) ◽  
pp. 531-539 ◽  
Author(s):  
Jay Jagannathan ◽  
Rene Smith ◽  
Hetty L. DeVroom ◽  
Alexander O. Vortmeyer ◽  
Constantine A. Stratakis ◽  
...  
Keyword(s):  
Mr Imaging ◽  
Remission Rate ◽  
Pituitary Surgery ◽  
Anterior Lobe ◽  
Accurate Identification ◽  
Cushing Disease ◽  
Complete Excision ◽  
Imaging Results ◽  
Prospective Database

Object Many patients with Cushing disease still have active or recurrent disease after pituitary surgery. The histological pseudocapsule of a pituitary adenoma is a layer of compressed normal anterior lobe that surrounds the adenoma and can be used during surgery to identify and guide removal of the tumor. In this study the authors examined the results of using the pseudocapsule as a surgical capsule in the resection of adenomas in patients with Cushing disease. Methods The authors reviewed a prospective database of data obtained in patients with Cushing disease who underwent surgery. The analysis included all cases in which a lesion was identified during surgery and in which the lesion was believed to be confined to the pituitary gland in patients with Cushing disease between January 1990 and March 2007. Since the objective was to determine the success of using the pseudocapsule as a surgical capsule, patients with invasive tumors and patients in whom no lesion was identified during surgery—challenging cases for surgical success—were excluded from analysis. Results In 261 patients an encapsulated adenoma was identified at surgery. Tumor was visible on MR imaging in 135 patients (52%); in 126 patients (48%) MR imaging detected no tumor. The range of tumor size overlapped considerably in the groups with positive and negative MR imaging results, indicating that in addition to size other features of the adenoma influence the results of MR imaging. In 252 patients hypercortisolism resolved after the first operation, whereas in 9 patients (3 with positive MR imaging and 6 with negative MR imaging) early reoperation was required. Hypercortisolism resolved in all 261 patients (256 with hypocortisolism and 5 with eucortisolism) before hospital discharge. Forty-six patients (18%) had postoperative electrolyte abnormalities (30 with hyponatremia and 16 with diabetes insipidus), but only 2 patients required treatment at discharge. The mean clinical follow-up duration was 84 months (range 12–215 months). Six patients (2%) had recurrence of hypercortisolism, all of whom were treated successfully with reoperation. Conclusions Because of their small size, adenomas can be challenging to identify in patients with Cushing disease. Use of the histological pseudocapsule of an adenoma allows accurate identification of the tumor and helps guide its complete excision. With this approach the overall remission rate is high and the rate of complications is low.

scholarly journals Surgical treatment of retroperitoneal neuroblastoma in a 14-month-old child

2021 ◽  
Vol 25 (1) ◽  
pp. 61-67
Author(s):  
M. N. Sukhov ◽  
A. G. Narbutov ◽  
I. P. Lyvina ◽  
I. A. Bryzzheva ◽  
M. V. Isaeva ◽  
...  
Keyword(s):  
Surgical Treatment ◽  
Nursery School ◽  
Disease Recurrence ◽  
Surgical Removal ◽  
Anatomical Location ◽  
Good State ◽  
Staging Systems ◽  
Tumor In Childhood ◽  
The Right

Introduction. Neuroblastoma is the most common malignant embryonic extracranial solid tumor in childhood having a high mortality rate. While treating neuroblastoma, it is extremely important to follow international protocols with their staging systems and groups of risk. Surgical treatment is a compulsory approach, but in some cases it is extremely difficult, and it involves significant risks. However, in some cases, radical removal of the tumor can cure patients without chemotherapy.Material and methods. The article presents a clinical case of 14-month-old child with neuroblastoma of stage 3 and difficult anatomical location after 2 courses of polychemotherapy by NB-2004 protocol. The child had subradical resection of an extensive tumor in the retroperitoneal space and nephrectomy on the right. The authors also describe specific moments during surgery, problems which their faced during child’s treatment as well as follow-up examinations in the postoperative period.Results. Follow-up examinations did not reveal any signs of disease recurrence. Catamnesis lasted for 56 months. The child is in a good state; she is active, attends a nursery school.Conclusion. The discussed case demonstrates that a successful radical surgical removal of neuroblastoma of difficult anatomic location may have good outcomes without adjuvant postoperative therapy.

scholarly journals Plasma microRNA Levels Combined with CEA and CA19-9 in the Follow-Up of Colorectal Cancer Patients

Cancers ◽  
2019 ◽  
Vol 11 (6) ◽  
pp. 864 ◽  
Author(s):  
Martin Pesta ◽  
Radek Kucera ◽  
Ondrej Topolcan ◽  
Marie Karlikova ◽  
Katerina Houfkova ◽  
...  
Keyword(s):  
Colorectal Cancer ◽  
Plasma Levels ◽  
Quantitative Estimation ◽  
High Specificity ◽  
Disease Recurrence ◽  
Palliative Surgery ◽  
Surgical Removal ◽  
Plasma Microrna ◽  
Low Sensitivity

Colorectal cancer (CRC) ranks among the most common cancers worldwide. Surgical removal remains the best strategy for treatment of resectable tumors. An important part of caring for patients after surgery is monitoring for early detection of a possible relapse of the disease. Efforts are being made to improve the sensitivity and specificity of routinely used carcinoembryonic antigen (CEA) with the use of additional biomarkers such as microRNAs. The aim of our study was to evaluate the prognostic potential of microRNAs and their use as markers of disease recurrence. The quantitative estimation of CEA, CA19-9, and 22 selected microRNAs (TaqMan Advanced miRNA Assays) was performed in 85 paired (preoperative and postoperative) blood plasma samples of CRC patients and in samples taken during the follow-up period. We have revealed a statistically significant decrease in plasma levels for miR-20a, miR-23a, miR-210, and miR-223a (p = 0.0093, p = 0.0013, p = 0.0392, and p = 0.0214, respectively) after surgical removal of the tumor tissue. A statistically significant relation to prognosis (overall survival; OS) was recorded for preoperative plasma levels of miR-20a, miR-21, and miR-23a (p = 0.0236, p = 0.0316, and p =0.0271, respectively) in a subgroup of patients who underwent palliative surgery. The best discrimination between patients with favorable and unfavorable outcomes was achieved by a combination of CEA, CA19-9 with miR-21, miR-20a, and miR-23a (p < 0.0001). The use of these microRNAs for early disease recurrence detection was affected by a low specificity in comparison with CEA and CA19-9. CEA and CA19-9 had high specificity but low sensitivity. Our results show the benefit of combining currently used standard biomarkers and microRNAs for precise prognosis estimation.

Cushing disease: use of perioperative serum cortisol measurements in early determination of success following pituitary surgery

2007 ◽  
Vol 23 (3) ◽  
pp. 1-8 ◽  
Author(s):  
Armand Krikorian ◽  
Dima Abdelmannan ◽  
Warren R Selman ◽  
Baha M Arafah
Keyword(s):  
Sella Turcica ◽  
Pituitary Surgery ◽  
Serum Cortisol ◽  
Serum Cortisol Level ◽  
Current Recommendation ◽  
Cushing Disease ◽  
Acth Deficiency ◽  
Cortisol Levels ◽  
Acth Secreting

✓Despite many recent advances, management of cases of Cushing disease continues to be challenging. After complete resection of ACTH-secreting adenomas, patients develop transient ACTH deficiency requiring glucocorticoid replacement for several months. The current recommendation by many centers, including ours, for patients with ACTH-secreting adenomas is to withhold glucocorticoid therapy during and immediately after adenomectomy until there is clinical or biochemical evidence of ACTH deficiency. A serum cortisol level of less than 2 μg/dl within the first 48 hours after adenomectomy is a reliable biochemical marker of ACTH deficiency and is associated with clinical remission of Cushing disease. Higher serum cortisol levels in the immediate postoperative period should be interpreted with caution. The decision to immediately reexplore the sella turcica should be individualized, taking into account the findings at surgery, the histopathological findings, and the changes in serum cortisol levels as well as the patient's wishes and concerns. Optimal diagnosis and therapy for patients with Cushing disease require thorough and close coordination and involvement of all members of the management team.

scholarly journals Rapidly Progressive Myelopathy Caused by Aggressive Vertebral Hemangioma

2019 ◽  
Vol 2019 ◽  
pp. 1-5
Author(s):  
Kevin A. Moattari ◽  
Rojeh Melikian ◽  
Sanjay K. Khurana
Keyword(s):  
Incidental Findings ◽  
Surgical Intervention ◽  
Rapid Development ◽  
Disease Recurrence ◽  
Benign Tumors ◽  
Vertebral Hemangioma ◽  
Radiological Findings ◽  
Progressive Myelopathy ◽  
Vertebral Hemangiomas

Introduction. Vertebral hemangiomas are the most common benign tumors of the spine, having an incidence of 10-12% in the general population. They are asymptomatic, incidental findings in the vast majority of patients; however, in rare cases, they can expand to cause neural compression. Aggressive lesions of this sort are most commonly found in the thoracic spine, and expansion leads to the subacute development of myelopathy. Case Report. The authors report a rare case of aggressive vertebral hemangioma at the T1 vertebral body which caused rapidly progressive myelopathy over the course of 7 days. Clinical and radiological findings are shown as well as surgical management of the lesion. The patient regained the ability to ambulate, and there was no evidence of disease recurrence at 2-year follow-up. Conclusions. Although aggressive vertebral hemangiomas are a rare cause of myelopathy, they must be kept in mind in the differential diagnosis of cord compressive lesions. In this case, contrary to most, the expansion of the hemangioma led to rapid development of neurological decline necessitating urgent surgical intervention.

scholarly journals MON-298 IGF-1 in the Early Post-operative Assessment of Acromegaly

2020 ◽  
Vol 4 (Supplement_1) ◽  
Author(s):  
Diane Donegan ◽  
Alicia Algeciras-Schimnich ◽  
Dana Z Erickson
Keyword(s):  
Cavernous Sinus ◽  
Surgical Intervention ◽  
Patient Anxiety ◽  
Cavernous Sinus Invasion ◽  
Delayed Treatment ◽  
First Line Therapy ◽  
Significant Difference ◽  
Suppression Test ◽  
Operative Assessment

Abstract Introduction: Transsphenoidal surgery is recommended as first line therapy in the vast majority of patients with acromegaly. Successful surgical intervention is determined by normalization of insulin like-growth factor (IGF) - 1 and adequate suppression of growth hormone (GH) during glucose GH suppression test or random GH levels &lt;1.0 ng/mL. Given the long half-life of IGF-1, evolution of IGF-1 and GH assays and limitations of GH suppression test, clinicians remain reliant on IGF-1 results 3–6 months following surgery to assess disease remission following surgical intervention. This can lead to delayed treatment of patients with persistent disease and significantly increase patient anxiety. Aim: To determine if IGF-1 levels at 6 weeks were equally predictive of surgical outcomes when compared to IGF-1 levels at 3–6 months postoperative. Methods: Retrospective review of patients with newly diagnosed acromegaly who had surgery between 2010–2019 and had post-operative IGF-1 level measured at 6 weeks and 3–6 months. IGF-1 was measured using the Siemens Immulite assay until 2016 when it was replaced by a LC-MS/MS assay. IGF-1 measurements at 6 weeks are obtained based on clinician discretion. Results: 69 patients (mean age 49 ± 14.8, female 31/69, 44.9%) with acromegaly had surgery and IGF-1 follow-up measurements at 6 weeks and 3–6 months. Persistent acromegaly was noted in 45/69 at 3–6 months. The median IGF-1 pre-operative was 701 ng/ mL (289–1600), 6 weeks postoperative was 286 ng/ mL (109–1038) and at 3–6 months was 267 ng/ mL (77–996). The median pre-operative IGF-1 was significantly higher than the median IGF-1 level at 6 weeks and 3–6 months (P=&lt;0.001). Although a statistically significant difference was seen between IGF-1 levels at 6 weeks and 3–6 months, the mean difference between these time points was small (20 ng/mL, P=&lt;0.001). There was a greater percent reduction in IGF-1 at 6 weeks following surgery in those who had a normal IGF-1 at 3–6 months compared to those who did not (63% vs 35%, P=&lt;0.01). Among patients who were deemed in remission at 3–6 months (24/69, 35%), 3/24 patients (all male with macroadenoma and no cavernous sinus invasion) had mildly elevated IGF-1 levels at 6 weeks (≤1.12 upper limit of normal) which subsequently normalized. In all of these patients, GH was &lt;1 ng/mL post-op. In 1 male (macroadenoma and cavernous sinus invasion), IGF-1 levels increased from normal at 6 weeks to abnormal when measured at 3–6 months. Conclusion: In the vast majority of patients, 6 weeks post-operative IGF-1 can be used to assess response to surgery avoiding a delay in treatment. However, we would recommend repeating IGF-1 levels at 3–6 months in those who have marginally elevated IGF-1 above normal (≤ 1.12), without cavernous sinus invasion and a post-operative GH of &lt;1 ng/mL prior to intervening if the IGF-1 is elevated 6 weeks as the IGF-1 may normalize.

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