phenylephrine-hydrochloride and Pituitary-ACTH-Hypersecretion

Knosp grade 4 adrenocorticotropic hormone (ACTH)-secreting pituitary adenoma is a rare cause of Cushing disease. After the 1st surgery, the remission rate among these patients is extremely low.. We presented a case of a 33-year-old female with classical Cushingoid symptoms. Further investigations revealed ATCH-dependent hypercortisolemia, as well as a Knosp grade 4 pituitary macroadenoma.. Cushing disease, caused by a Knosp grade 4 pituitary macroadenoma.. The patient underwent endoscopic endonasal transsphenoidal surgery with the assistance of intraoperative transsphenoidal Doppler and image-guidance devices.. Pathologic examinations confirmed that the lesion was an ACTH-secreting pituitary adenoma. The patient was in biochemical remission after surgery. Her postoperative magnetic resonance imaging showed gross-total tumor resection. There was no evidence of recurrence during the 1-year follow-up.. With intraoperative Doppler and image-guidance, gross-total resection and biochemical remission can be achieved in Cushing disease when the internal carotid artery is completely encased by the pituitary adenoma.

Topics: ACTH-Secreting Pituitary Adenoma; Adenoma; Adult; Female; Humans; Magnetic Resonance Imaging; Natural Orifice Endoscopic Surgery; Neoplasm Recurrence, Local; Nose; Pituitary ACTH Hypersecretion; Pituitary Gland; Pituitary Neoplasms; Sphenoid Bone; Treatment Outcome; Ultrasonography, Interventional

Surgery is currently the first-line treatment of Cushing's disease. Surgery for Cushing's patients requires technical specificity, especially if no adenoma is identified on dedicated preoperative pituitary MRI.. From 2006 to 2020, 683 patients with Cushing's disease were operated on with a mononostril endoscopic endonasal approach by the same two senior neurosurgeons. Here, we report the particularities of this challenging surgery.. A rigorous and planned surgical strategy avoids the pitfalls of Cushing's disease surgery and leads to a high rate of endocrine remission.

Topics: Adenoma; Humans; Magnetic Resonance Imaging; Nose; Pituitary ACTH Hypersecretion; Pituitary Gland; Pituitary Neoplasms

To assess outcomes and predictors of early and long-term remission in patients with Cushing's disease (CD) due to ACTH-secreting adenomas treated via endoscopic endonasal approach (EEA).. This is a retrospective study. Consecutive patients operated for CD from 1998 to 2017 in an Italian referral Pituitary Center were enrolled. Clinical, radiological, and histological data at enrollment and follow-up were collected.. 151 patients (107 F) were included; 88.7% were naïve for treatment, 11.3% had been treated surgically and 11.2% medically. At pre-operative magnetic resonance imaging (MRI), 35 had a macroadenoma and 80 a microadenoma, while tumor was undetectable in 36 patients. Mean age at surgery was 41.1 ± 16.6 years. Diagnosis was confirmed histologically in 82.4% of the cases. Patients with disease persistence underwent second surgery and/or medical and/or radiation therapy. Mean follow-up was 92.3 ± 12.0 (range 12-237.4) and median 88.2 months. Remission rate was 88.1% after the first surgery and 90.7% at last follow-up. One patient died of pituitary carcinoma. Post-surgical cortisol drop (p = 0.004), tumor detection at MRI (p = 0.03) and size < 1 cm (p = 0.045) increased the chance of disease remission; cavernous sinus invasion was a negative predictor of outcome (p = 0.002). Twenty-seven patients developed diabetes insipidus and 18 hypopituitarism. Surgery repetition increased the risk of hypopituitarism (p = 0.03), but not of other complications, which included epistaxis (N = 2), cerebrospinal fluid leakage (1), pneumonia (3), myocardial infarction (1), and pulmonary embolisms (2).. Selective adenomectomy via EEA performed by experienced surgeons, supported by a multidisciplinary dedicated team, allows long-term remission in the vast majority of CD patients with low complication rate.

Topics: ACTH-Secreting Pituitary Adenoma; Adenoma; Adult; Endoscopy; Female; Humans; Italy; Magnetic Resonance Imaging; Male; Middle Aged; Neurosurgical Procedures; Nose; Pituitary ACTH Hypersecretion; Prognosis; Referral and Consultation; Retrospective Studies; Treatment Outcome; Young Adult

This study investigated the outcome of transsphenoidal surgery (TSS) for Cushing disease (CD) and the influence of our surgical strategy on remission rates and postoperative pituitary function.. We retrospectively reviewed data from 71 patients with CD who underwent microscope navigation TSS (MN-TSS) in Saint-Luc Hospital between 1996 and 2017. True remission was defined as normal fasting cortisol level, normal 24-hour urinary free cortisol, or continued need for hydrocortisone replacement for 1 year after surgery.. Overall remission rate after 1 or repeated MN-TSS was 83%. Highest remission rate was found in patients with macroadenomas (92%). Successful first MN-TSS was correlated with a high final remission rate (95%), whereas failed first MN-TSS was correlated with a low final remission rate (36%). Although day 1 cortisol levels were significantly lower in patients with long-term remission, high levels were still observed in a few patients, especially those who had had CD for many years. We found a low rate of postoperative pituitary long-term hypofunction (9.7%).. MN-TSS is a safe and effective procedure to treat CD, allowing remission rates of 83%. One-year remission period after first surgery is correlated with a final remission rate of 95%. Although day 1 morning cortisol value is the most significant predictor for long-term remission, some patients with CD for many years may keep high postoperative cortisol levels and be in later remission, likely because of secondary adrenal hyperplasia. Our focused approach with microscope navigation resulted in low rates of postoperative pituitary hypofunction and kept a recurrence rate comparable to that in the literature.

Topics: Adolescent; Adult; Aged; Aged, 80 and over; Female; Humans; Longitudinal Studies; Magnetic Resonance Imaging; Male; Middle Aged; Neurosurgical Procedures; Nose; Pituitary ACTH Hypersecretion; Pituitary Hormones; Retrospective Studies; Sphenoid Bone; Sphenoid Sinus; Treatment Outcome; Young Adult

Cushing disease is a relatively rare cause of Cushing syndrome secondary to a hyperfunctioning pituitary adenoma. In addition to signs and symptoms of hypercortisolism, Cushing disease may present with diverse otolaryngic manifestations, which may guide diagnosis and management. We performed a retrospective chart review of patients who were found to have Cushing disease and who underwent transnasal transsphenoidal surgery for pituitary adenomas between January 1, 2007, and July 1, 2014, at a tertiary academic medical center. There were 37 consecutive patients in this series with Cushing disease caused by a pituitary adenoma. Fifteen (41%) patients complained of visual changes. Five (14%) patients suffered from obstructive sleep apnea. Four (11%) patients had thyroid disease. Other symptoms included hearing loss, vertigo, tinnitus, epistaxis, dysphagia, and salivary gland swelling. Although Cushing disease traditionally presents with classic "Cushingoid" systemic features, it also may present with various otolaryngic manifestations. A thorough workup by otolaryngologists is critical in the comprehensive management of these patients.

Topics: Adolescent; Adult; Aged; Child; Female; Humans; Male; Middle Aged; Natural Orifice Endoscopic Surgery; Nose; Otorhinolaryngologic Diseases; Pituitary ACTH Hypersecretion; Pituitary Neoplasms; Postoperative Complications; Retrospective Studies; Young Adult

The primary objective was to assess the remission rate, and the secondary objectives were to evaluate the early complications and recurrence rate and to define the predictive factors for the remission and recurrence rates.. This prospective single-center study included 230 consecutive patients, operated on by a single surgeon for Cushing's disease via a transsphenoidal endoscopic endonasal approach, over a 6-year period (2008-2013). The patients included in this series were all adults (>18 years of age), who presented with clinical and biological characteristics of Cushing's disease confirmed based on dedicated MRI pituitary imaging. Biochemical remission was defined as a postoperative serum cortisol level <5 μg/dl on the 2nd day following surgery that required glucocorticoid replacement therapy.. The remission rate for the global population (n = 230) with a follow-up of 21 ± 19.2 months concerned 182 patients (79.1%) divided into 132 patients (82.5%) with positive MRI and 50 patients (71.4%) with negative MRI with no statistically significant difference (p = 0.077). Complications occurred in 77 patients with no deaths. A total of 22% of patients had transient diabetes insipidus and 6.4% long-term diabetes insipidus, and no postoperatively CSF leakage was observed. The recurrence rate was 9.8% with a mean time of 32.7 ± 15.2 months. The predictive factors for the remission rate were the presence of pituitary microadenoma and a positive histology. No risk factors were involved regarding the recurrence rate.. Whatever the MRI results, the transsphenoidal endonasal endoscopic approach remains the gold standard treatment for Cushing's disease. It was maximally effective with a remission rate of 79.1% and lower morbidity.

Topics: Adolescent; Adult; Aged; Aged, 80 and over; Cerebrospinal Fluid Rhinorrhea; Diabetes Insipidus; Female; Humans; Magnetic Resonance Imaging; Male; Middle Aged; Natural Orifice Endoscopic Surgery; Nose; Pituitary ACTH Hypersecretion; Postoperative Complications

Transsphenoidal selective adenectomy is considered the treatment of choice in patients with Cushing's disease given the possibility for high remission and low complication rates. The endoscopic endonasal surgical nuances for Cushing's disease are not well illustrated in the neurosurgical technique and video collections.. We describe the technical nuances of microadenoma resection for two cases of Cushing's disease, one in which the adenoma is visualized on imaging and one in which the pre-operative MRI is negative.. Transsphenoidal endoscopic selective adenectomy is a safe and effective surgery for patients with Cushing's disease regardless of preoperative MRI findings.

Topics: Adenoma; Humans; Natural Orifice Endoscopic Surgery; Neurosurgical Procedures; Nose; Pituitary ACTH Hypersecretion; Pituitary Neoplasms; Postoperative Complications

Patients undergoing pituitary adenoma or Rathke cleft cyst (RCC) removal are often administered perioperative glucocorticoids regardless of lesion size and preoperative adrenocorticotropic hormone/cortisol levels. To minimize unnecessary glucocorticoid therapy, we describe a protocol in which patients with normal preoperative serum cortisol and adrenocorticotropic hormone levels are given glucocorticoids only if postoperative day 1 or 2 (POD1 or POD2) cortisol levels decrease below normal.. A total of 207 consecutive patients undergoing endonasal surgery for an adenoma or RCC were considered for study. Of these, 68 patients with preoperative adrenal insufficiency or Cushing disease were excluded. Glucocorticoids were withheld unless POD1 or POD2 morning cortisol values were below normal (≤4 μg/dL). Subsequent adrenal status was assessed through follow-up biochemical and clinical evaluations.. The 139 patients included 119 with macroadenomas, 14 microadenomas, and 6 RCCs (follow-up, 3-41 months; median, 10 months). Nine patients (6.5%), all with macroadenomas (mean diameter, 26 ± 10 mm) had low POD1 or POD2 cortisol values and received glucocorticoids; of these, five patients were weaned off within 3-28 weeks of surgery. Overall, 12 of 139 patients (8.6%) were treated for early (n = 9) or delayed (n = 3) adrenal insufficiency but only 5 patients (3.6%) remain on glucocorticoid replacement. No patient experienced an adrenal crisis. Using morning POD1 or POD2 cortisol values >4 μg/dL as a measure of adequate hypothalamic-pituitary-adrenal axis function, yields a sensitivity of 96%, a specificity of 57%, and a positive predictive value of 98%.. In patients with normal preoperative cortisol levels undergoing endonasal removal of a pituitary adenoma or RCC, normal morning cortisol values on POD1 and POD2 reliably predicts adequate and safe adrenal function in 98% of patients. This simple protocol of withholding postoperative glucocorticoids avoids unnecessary steroid exposure and poses minimal risk to the well-informed closely monitored patient.

Topics: Adenoma; Adrenocorticotropic Hormone; Adult; Aged; Biomarkers; Central Nervous System Cysts; Circadian Rhythm; Female; Follow-Up Studies; Glucocorticoids; Humans; Hydrocortisone; Male; Middle Aged; Nose; Pituitary ACTH Hypersecretion; Pituitary Neoplasms; Postoperative Period; Predictive Value of Tests; Sensitivity and Specificity; Sphenoid Bone

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.. To determine an appropriate course of action after nondiagnostic IPSS.. 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.. 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%.. Transsphenoidal surgery should be considered in cases of ACTH-dependent Cushing disease and noncentralized or technically unsuccessful IPSS without evidence of ectopic tumor.

Topics: Adolescent; Adrenocorticotropic Hormone; Adult; Aged; Aged, 80 and over; Child; Corticotropin-Releasing Hormone; Female; Humans; Hydrocortisone; Male; Middle Aged; Nose; Petrosal Sinus Sampling; Pituitary ACTH Hypersecretion; Pituitary Gland; Predictive Value of Tests; Retrospective Studies; Sensitivity and Specificity; Statistics, Nonparametric; Treatment Outcome; Young Adult