Petrosal Sinus Catheterization Research Articles

THU037 Diagnostic Accuracy Of Bilateral Inferior Petrosal Sinus Sampling In Adrenocorticotropin-dependent Cushing’s Syndrome, And The Utility Of Maximum-stimulated Petrosal Sinus ACTH To Baseline Petrosal Sinus ACTH Ratio

Abstract Disclosure: A.N. Maldar: None. P.H. Chauhan: None. A. Kulkarni: None. N.F. Shah: None. B.K. Misra: None. M. Chadha: None. Methods: The diagnosis of Cushing’s Syndrome (CS) was made as per the prevalent endocrine society guidelines. CS patients with serum ACTH>15 pg/mL were considered as ACTH-dependent CS. Baseline ACTH (both inferior petrosal sinuses and peripheral) values were obtained, and since 2014, stimulated ACTH values (5 and 10 minutes post one unit iv vasopressin) were obtained as standard protocol. Prolactin corrected ACTH ratios were additionally being calculated since 2016. The highest central to peripheral ACTH gradient >2 in baseline samples (bC:P), >3 in samples after vasopressin stimulation (sC:P), and >0.8 in PRL-corrected ratios (pC:P) was used as reference value indicative of Cushing’s Disease (CD). An inter-petrosal gradient >1.4 was considered to be suggestive of an adenoma located on the side of the petrosal sinus with the higher ACTH concentration. Results: Of the 51 patients with ACTH-dependent CS who underwent BIPSS, four patients with incomplete data, one patient with unilateral petrosal sinus catheterization, one patient with Cortisol Resistance Syndrome were excluded from the analysis. Mean age of our cohort was 37.5 years, with 77.8% females. 39 patients had CD (positive histopathology-immunohistochemistry in 34, and remission post-surgery in additional five with hyperplasia), while six had ectopic CS (one each of bronchial carcinoid, lung NET, thymic NET, pancreatic NET, and two with undiagnosed ectopic source). bC:P>2 (N=45) demonstrated 92.3% sensitivity, 83.3% specificity, 97.3% PPV and 62.5% NPV, while sC:P>3 (N=31) demonstrated 96.3% sensitivity, 100% specificity, 100% PPV and 80% NPV to diagnose CD. pC:P>0.8 (N=20) improved the sensitivity and NPV to 100%, with a 75% specificity and 94.1% PPV. On ROC analysis, cut-point ratio of 2.55 for bC:P value gave a sensitivity of 89.7% and specificity of 100% (AUC=0.964); sC:P ratio of 2.95 had 96.3% sensitivity and 100% specificity (AUC=0.991); 0.93 for pC:P ratio provided a sensitivity of 93.8% and specificity of 100% for diagnosis of CD (AUC=0.984). Maximum-stimulated petrosal sinus ACTH to baseline petrosal sinus ACTH ratio of <1.11 provided a 100% sensitivity, while a ratio >1.75 provided 100% specificity towards diagnosis of CD. 71.4% (10/14) patients with left sided adenoma, and 85% (17/20) patients with right sided adenoma were correctly lateralised on BIPSS. In patients whom adenoma was detected on pituitary MRI, 66.7% (6/9) were correctly lateralised to the left side, and 94.1% (16/17) to the right side. Conclusion: BIPSS provides high sensitivity and specificity to diagnose CD. Vasopressin stimulation improves the sensitivity and specificity, while prolactin correction improves the sensitivity of BIPSS, as compared to baseline ratios. Maximum-stimulated petrosal sinus ACTH to baseline petrosal sinus ACTH ratio can be additionally used, especially in CD patients suspected of false negative localization. Presentation: Thursday, June 15, 2023

Papillary Thyroid Carcinoma and Cushing’s Disease: A Rare Association With Multiple Endocrine Neoplasia Type 1

Introduction: Multiple Endocrine Neoplasia type 1 (MEN1) is characterized by tumours of the parathyroid glands, neuroendocrine pancreatic tumour (PNET), and anterior pituitary. Studies suggest that MEN1 gen is not etiologically related to the oncogenesis of the papillary thyroid carcinoma (PTC). Expression of menin is preserved in human normal thyroid tissue and THc. Papillary thyroid carcinoma (PTC) combined with multiple endocrine neoplasia type 1 is a rare association. Cushing Syndrome (CS) is another rare associated condition with MEN-1. In patients with MEN1, CS can result from pituitary, adrenal, or other endocrine tumours. In large series of MEN-1 CS has low frequency (0.5%) or not described. Clinical Case: A.M.F.A, 49 years, female. In 2007, cytological study of thyroid nodules resulted in PTC, underwent total thyroidectomy. Anatomopathological (AP) confirmed PTC. She was followed with an excellent response to therapy. In 2018, abdominal ultrasound due to epigastric pain showed pancreatic lesion. MRI confirmed lesion in the head and body of the pancreas of 8.2cm. She underwent total gastroduodenopacreatectomy (TGDP), AP showed a well-differentiated PNET (KI67: 2%). Before TGDP, tests for MEN-1 were collected: IGF-1 of 450 ng/ml (RV for age: 90-249 ng/ml); calcium: 1,46 nmol/l (RV: 1,12-1,40), PTH: 91,7 pg/ml (RV: 15-65), prolactin 13,3 ng/ml (RV: 5-23), gastrin 22pg/ml (RV 13-115), and late night salivary cortisol (LNSC): 8,3 nmol/l (RV <7,6). Pituitary MRI showed a 0.5cm intrasellar nodule. Genetic study revealed c654 + 1G>T mutation in heterozygosity. O3 family members had the same mutation. To investigate hypercortisolism, it was made basal ACTH (56,1 pg/ml (VR 46 pg/ml), urine free cortisol (UFC) of 261,0 mcg/24h, 1mg DST of 15,2 mcg/dl (RV <1,8), basal cortisol of 16,5 mcg/dl and 8mg DST of 13,7 mcg/dl (not 50% suppressing of basal value). Another measurement of IGF-1 (after the PNET surgery) was normal. A petrosal venous sinus catheterization (PVSC) was made, which was compatible with Cushing’s disease (CD). She had brittle hair, facial plethora, proximal muscle weakness and easily bruising, without other clinical CS aspects. Treatment with ketoconazole was initiated and she was referred to transesphenoidal surgery. Conclusion: Patient had two rare presentation of MEN1, first manifestation was PTC. PNET and CD was diagnosed 11 years late during clinical follow up. In this patient, MEN1 syndrome may have predisposed the patient to developing PTC. Currently, little is known about the prevalence of THc in MEN1 and it is unclear whether tumorigenesis is related. Further studies and additional case reports are required to clarify this connection. Also, we observed IGF-1 value normalized after TGDP, so it could be caused by a functional PNET. Rare causes of acromegaly are excess secretion of GH-releasing hormone (GHRH) by hypothalamic tumours, and ectopic GHRH or GH secretion by NET.

Efficiency of Endovascular Therapy for Bilateral Cavernous Sinus Dural Arteriovenous Fistula

The principles of endovascular treatment of bilateral cavernous sinus dural arteriovenous fistula (CS-dAVF) are not well established because of the complexity in the number of fistula tracts and their behavior, which seems to be more aggressive. We aimed to determine an efficient technique for endovascular treatment of bilateral CS-dAVF and the associated factors to achieve good clinical and angiographic outcomes. The data were analyzed from 165 consecutive patients diagnosed with CS-dAVF from January 2005 to September 2018. The demographic data included approaching route, embolization times, embolization material, sequence of embolization, number of embolization sessions, and angiographic and clinical outcomes. Interrater agreement of bilateral CS-dAVF diagnosis was performed using the κ coefficient. The factors associated with treatment outcome were analyzed using a Pearson χ2 test. Bilateral CS-dAVF was detected in 43 patients (26%). Angiographic presentations that showed evidence of sinus thrombosis, dangerous venous drainage, and higher Satomi classification were more commonly found in bilateral CS-dAVF than in unilateral CS-dAVF. Good clinical outcome and cure from angiography were obtained in 90% and 74%, respectively. Ipsilateral inferior petrosal sinus-intercavernous sinus-contralateral cavernous sinus catheterization was the major approach route of treatment. The factors associated with improved clinical outcome were transvenous approach, shunt closure, coil embolization, and sequencing the embolization (P < 0.001). Dangerous venous drainage tends to increase in bilateral CS-dAVF. Retrograde ipsilateral inferior petrosal sinus catheterization using coil embolization and sequencing the embolization are the major concerns for treatment.

Muestreo de senos petrosos

Objetivos: analizar, evaluar y describir la utilidad del muestreo de senos petrosos para diagnóstico del síndrome de Cushing de origen central.&#x0D; Material y métodos: se analizaron los aspectos técnicos y resultados del muestreo bilateral venoso de senos petrosos, desde el 1º de enero de 2012 a 31 de diciembre de 2018 en el Hospital Universitario San Vicente de Paúl en Medellín, Colombia.&#x0D; Resultados: el promedio de edad fue 43.3 años con un rango de edad desde los 19 hasta los 69 años. La lateralidad pudo ser demostrada en 68.2% de los casos con una tendencia a la localización en el lado izquierdo en 53.3%. El origen central de producción de ACTH logró ser demostrado en 95.4% de los casos, con una relación central/periferia basal promedio de 21.7 y postestimulación de 70.8. Todas las muestras a los 3, 5 y 10 minutos fueron confirmatorias tras la estimulación.&#x0D; Conclusión: en nuestro estudio retrospectivo el cateterismo de senos petrosos confirmó la fuente central de producción de ACTH por laboratorio en un alto porcentaje de pacientes sin ninguna complicación inmediata

SUN-462 Petrous Sinus Catheterization, Baseline and after Stimulation with Desmopressin, for the Diagnosis of Cushing's Disease, Experience of a National Health Institute in Mexico

Introduction: Cushing's disease (CD) is, in some cases, difficult to diagnose and the petrosal sinus catheterization (PSC) is indicated to rule out other etiologies of hypercortisolism, mainly in patients without a visible adenoma, that have an adenoma less than 6 mm or when the dynamic tests are discordant, and the suspicion of CD remains. Objective: To evaluate basal PSC and PSC after stimulation with desmopressin as a diagnostic tool for CD. As a secondary outcome we described the clinical and characteristics of the enrolled patients. Methods: We included all patients who underwent petrosal sinus catheterization since 1980 at our Institution and were >18 years old. We exclude all patients that underwent the procedure after surgical resection or radiotherapy of the adenoma for any reason. We eliminated patients that had incomplete information regarding the procedure. The gold standard for diagnosing CD was the histological evidence of an adenoma and/or remission of the disease with surgery. Results: We included 26 patients, 84.6% women, with an average age at diagnosis of 31 (23-40) years. The most common comorbidities were dyslipidemia (61.5%), followed by glucose abnormalities (50%). The main indication to perform the PSC was to have an adenoma smaller than 6 mm (38.4%). The final diagnosis was CD in 84.6% (n = 22) and adrenal hyperplasia in the rest (n = 4), there were no patients with ectopic Cushing's syndrome in our series. We couldn’t evaluate the usefulness of the procedure regarding the laterality of the adenoma because not all the patients had a complete report of the MRI. The central to peripheral gradient of ACTH (CPGA) > 1.2 (baseline), showed a sensitivity 95%, specificity 75%, positive predictive value (PPV) 95%, negative predictive value (NPV) 75%, positive likelihood ratio (LR+) 3.81 and negative likelihood ratio (LR-) 0.06. The CPGA post-stimulation with desmopressin > 2 had a sensitivity 86%, specificity 75%, PPV 95%, NPV 50%, LR+ 3.45, LR- 0.18. Conclusions: The PSC is a useful tool to make the differential diagnosis of CD with adrenal Cushing, when is indicated. The stimulation with desmopressin shows an adequate performance compared to that reported for CRH. A gradient > 1.2 baseline and > 2 post-stimulation can be used as diagnostic criteria.

Effectiveness of Bilateral Inferior Petrosal Sinuses Sampling in Tumor Lateralization: Intraoperative Findings and Postoperative Results.

Object Diagnosis and treatment of Cushing's disease (CD) is a demanding endeavor. We evaluated the effectiveness of inferior petrosal sinus sampling (IPSS) in preoperative tumor lateralization and surgical outcomes. Methods Fifty-two consecutive patients with CD and normal magnetic resonance imaging (MRI) were enrolled between 2009 and 2015. Selective catheterization of petrosal sinuses for IPSS was performed. All patients underwent microsurgical dissection, and if a lesion was found, underwent lesion resection. Demographic, biochemical, and intraoperative findings were prospectively collected and reviewed 6 and 12 months postoperatively. Results Forty-five patients (86.4%) had clear lateralization while seven patients had bilateral elevation. Twenty-two patients (42%) were found to have a macroscopic lesion, defined as an abnormal tissue during microsurgical dissection, which was thus resected. No patient had a lesion larger than 4 mm. Eighty-one percent of patients had the lesion where IPSS had been predicted. Hemi-hypophysectomy was performed on twenty-eight patients based on IPSS findings, since no lesion was found. Eighty-six percent of patients with lesion resection had biochemical remission by 12 months. Biochemical remission was documented in 78 and 71% of patients who underwent hemi-hypophysectomy by 6 and 12 months, respectively. Conclusion With careful microsurgical dissection, a macroscopic lesion could be found in a significant number of patients with normal MRI. When no lesion was found, we recommend performing a hemi-hypophysectomy based on IPSS findings, since prediction accuracy was high and remission rates were significant.

Anatomic variations of internal jugular vein, inferior petrosal sinus and its confluence pattern: Implications in inferior petrosal sinus catheterization.

The purpose of this study is to describe anatomic variations of the internal jugular vein (IJV), inferior petrosal sinus (IPS) and their confluence pattern and implications in IPS catheterization. The anatomic route of IPS after going out of the cranium and its confluence patterns with IJV and will supply knowledge about typing of IPS-IJV junction. A review of the literature was performed. There might be different routes for entering the intracranial segment of the IPS and multislice spiral computed tomography (MSCT) is effective in identifying the confluences of the IPS with the IJV and their courses. It is important to find the confluence of IPS with IJV for diagnosis and treatment of intracranial lesions via venous route. Meanwhile, IPS diameter at the confluence can significantly affect success of catheterization. The classification and the theory of the development of the caudal end of the IPS may be useful in establishing treatment strategies that involve endovascular manipulation via the IPS.

Maximizing the accuracy of Inferior petrosal sinus sampling: validation of the use of Prolactin as a marker of pituitary venous effluent in the diagnosis of Cushing’s disease

This study aimed to increase the accuracy of the inferior petrosal sinus sampling (IPSS) procedure and reduce the false-negative rate through the addition of prolactin as a marker of pituitary venous outflow as well as validate this adjunct to the test process. Inferior petrosal sinus sampling (IPSS) for ACTH is the current gold standard test for the differentiation of pituitary Cushing's disease from the ectopic ACTH syndrome. Although early studies with IPSS reported a diagnostic sensitivity and specificity approaching 100%, additional experience has revealed a false-negative rate of 1-10%. This has been attributed to either technical problems with unsuccessful petrosal sinus catheterization or anomalous venous drainage of the pituitary. Previous studies have suggested that the measurement of other anterior pituitary hormones may be useful during IPSS as a guide to the effectiveness of cannulation and to improve the diagnostic accuracy of the procedure. We reviewed the data, in this retrospective cohort study, for all patients who had undergone IPSS for the investigation of ACTH-dependent hypercortisolism. The study included 83 patients who underwent IPSS at St. Thomas's hospital between 2005 and 2010. Plasma ACTH and prolactin levels were measured both centrally and peripherally. The normalized ACTH/Prolactin inferior petrosal sinus/peripheral ratio was then calculated to assess the accuracy of the sampling procedure. A total of 83 patients with confirmed ACTH-dependent cortisol excess underwent investigation with IPSS during the study period. Sixty-seven patients initially had a positive IPSS result (i.e. a basal central/peripheral ACTH ratio >2·0 and >3·0 post-CRH). However, when the concurrent prolactin data were used, six patients were additionally found to have positive results suggestive of pituitary Cushing's. The Prolactin normalized ACTH IPS/Peripheral ratios were all >0·8 in patients with proven Cushing's disease, whereas they were all <0·6 in proven ectopic ACTH syndrome. The diagnosis was subsequently confirmed histologically in 72 of the patients. Using Prolactin as a concurrent index of pituitary venous effluent helps us recognize whether pituitary venous blood has been accurately sampled. Normalizing the IPS/peripheral ratios with Prolactin helps to improve the accuracy of the result and reduces the false-negative rate. With regards to the usefulness/validity of this test in clinical practice, it is relevant, reproducible and is easily adaptable from the existing diagnostic sequence.

Cushing Syndrome due to Ectopic Adrenocorticotropin Secretion by Oncocytic Thyroid Nodule

A 60-yr-old woman presented with osteoporosis and atraumatic fractures of the spine and ribs in our neurological hospital in Linz, Upper Austria. She had a clinical history of bipolar affective disorder and cognitive decline, but the actual medical problem was lumbar pain. Treatment for osteoporosis already included bisphosphonates, vitamin D, and calcium. Endocrine evaluation for secondary osteoporosis revealed low TSH (0.17 U/ml) under prophylactic levothyroxine treatment (75 g/d) for multinodular goiter and ACTH-dependent hypercortisolism. Clinical evaluation presented obesity (weight, 75 kg, for 150 cm height), arterial hypertension, and diabetes mellitus. ACTH was elevated at 106 pg/ml, whereas morning serum cortisol (22 g/dl) was still in the normal range. Cortisol measured in 24-h urine was highly elevated at 502 g/d (normal range, 40–158 g/d). Dexamethasone suppression test with 1, 4, and 8 mg failed to suppress either plasma ACTH or cortisol in serum and/or 24-h urine (ACTH, 45 pg/ml; and cortisol, 20 g/dl and 400 g/d in all tests). Brain magnetic resonance imaging showed mild cortical atrophy and did not present any alterations of the pituitary. Because petrosal sinus catheterization cannot be practiced in the federal state of Upper Austria, it was postponed. Whole body fluorodeoxyglucose (FDG) positron emission tomography (PET)/computed tomography (CT) was done in May 2009 for tumor screening to detect a possible ectopic source of ACTH. A single nodule of the right thyroid lobe proved to be FDG avid (Fig. 1). Additionally, CT detected a lesion of the right kidney with a diameter of 2 cm that did not accumulate FDG. Because a former thyroid evaluation in March 2009— practiced in a regional institute in the “Salzkammergut” in Upper Austria—had evaluated this FDG-avid lesion as a cold nodule by Technetium-scintiscan and fine-needle-aspiration had shown oncocytic transformation of thyrocytes, we recommended thyroid surgery as the next step. Calcitonin screening had been negative in the patient. Near-total resection of the thyroid took place in June 2009, histology revealed regressive goiter, and a malignant lesion was not detected. The suspicious nodule was

Superficial Temporal Vein: Route to Embolization of Cavernous Dural Arteriovenous Fistula

Superficial temporal vein catheterization was used to embolize a dural arteriovenous malformation of the cavernous sinus in a 44-year-old woman. The pertinent venous anatomy is discussed. This route may provide crucial access to the cavernous sinus when conventional approaches such as the Inferior petrosal sinus catheterization are difficult.

Cateterização dos seios petrosos inferiores: aspectos técnicos

Inferior petrosal sinus catheterization and sampling for corticotropin dosage helps to differentiate hypophisary and ectopic forms of Cushing syndrome. The aim of this paper is to describe the technique used in inferior petrosal sinus catheterization in our service, emphasizing the solution found for frequent difficulties, and verify the success rate achieved. Between September/2000 and September/2005, forty-two (eighty-four sinuses) patients were submitted to inferior petrosal sinus sampling. The difficulties for correct catheter positioning were identified and correlated with their solutions. Anatomical variations, similarity between IPS and emissary vein of the basilar plexus and unfavorable flow to the contrastation of the structures (retrograde catheterization) were the main problems. Using pre-shaped catheters, curved, steerable guide-wires, road-mapping and venography by contalateral injection, besides criteria to differentiate IPS from the emissary vein. Of the 84 sinuses approached, one was thrombosed, and 80 (96.4%) of 83 possible were selectively catheterized. No clinical complication occurred. IPSC can be safe and successfully performed in most cases. The identification of the emissary vein of the basilar plexus and use of venography by contralateral injection, improved the method performance.

Diagnosis, treatment, and outcome of pituitary tumors and other abnormal intrasellar masses. Retrospective analysis of 353 patients.

We reviewed the clinical features, essential laboratory data, pituitary imaging findings (computerized tomography and magnetic resonance imaging), management, and outcome of 353 consecutive patients with the presumptive diagnosis of pituitary tumor investigated from January 1984 through December 1997 at University Hospital, Lausanne, Switzerland. In 18 cases primary empty sella turcica was diagnosed, and in 13 cases of pseudacromegaly there were no endocrine abnormalities. The remaining 322 patients disclosed abnormal pituitary masses, including 275 pituitary adenomas, 18 craniopharyngiomas, 6 cases of primary pituitary hyperplasia, 6 intrasellar meningiomas, 6 cases of distant metastases, 4 intrasellar cysts, 2 chordomas, 1 primary lymphoma, and 1 astrocytoma. Biologic data and immunohistochemical analysis of the excised tissues demonstrated that prolactinomas and nonsecreting adenomas (NSAs) were the most frequent pituitary tumors (40% and 39%, respectively), followed by somatotropic adenomas with acromegaly (11%) and Cushing disease (6%). In contrast with the vast majority of NSAs, which significantly expressed glycoprotein hormones in tissue without secreting them, there was a small group of glycoprotein hormone-secreting adenomas (2%), which had a more severe clinical course after surgery. Thirty-eight pituitary masses were incidentally discovered, most of them NSAs. The expansion of pituitary adenomas into the right cavernous sinus was twice as frequent as to the left cavernous sinus. For the differential diagnosis of hyperprolactinemia, basal prolactin (PRL) levels above 85 micrograms/L, in the absence of renal failure and PRL-enhancing drugs, and a PRL increment of less than 30% after thyrotropin-releasing hormone (TRH) accurately ruled out functional hyperprolactinemia due to NSA, and were typical of prolactinomas. For screening and follow-up of acromegaly, basal growth hormone (GH) and insulin-like growth factor 1 (IGF-1) levels, as well as the paradoxical GH response to TRH (present in 2/3 acromegalic patients), could be used as convenient tools, but the most accurate test for diagnosis and prediction of outcome after therapy was GH (lack of) suppression during oral glucose tolerance test. In Cushing disease, single evening plasma cortisol was as good as the overnight dexamethasone suppression test for screening, and a combined dexamethasoneovine corticotropin-releasing hormone (oCRH) test was as accurate as the long dexamethasone suppression test to confirm the diagnosis. Bilateral inferior petrosal sinus catheterization coupled with oCRH test confirmed the pituitary origin of excess adrenocorticotropic hormone (ACTH) in all patients, including those with normal pituitary on magnetic resonance imaging (50% of the cases). However, this procedure failed to predict tumor localization correctly within the pituitary in 21% of patients. Pituitary cysts, meningiomas, and craniopharyngiomas with an intrasellar component were correctly diagnosed based on pituitary imaging in 75%, 67%, and 44% of cases, respectively. The remainder, as well as the cases of pituitary hyperplasia, metastases, and other less frequent pathologies, were initially diagnosed as NSAs or as masses of unknown nature. When surgery was indicated, pituitary adenomas and other intrasellar masses were operated on by the transsphenoidal route, with the exception of 100% of meningiomas, 83% of craniopharyngiomas, and 10% of NSAs, which were operated on by the transcranial route. Favorable late surgical outcome of prolactinomas could be predicted by a restored PRL response to TRH. However, dopamine agonist (DA) therapy, usually resulting in satisfactory control of PRL levels and in tumor shrinkage, progressively displaced surgery as primary treatment for prolactinomas throughout the study period. After full-term pregnancy, the size of prolactinoma decreased in 7 of 9 patients, and PRL was normal in 2. Surgery was the first treatment for NSAs, with a tumor rela

Bronchopulmonary carcinoid tumors associated with cushing's syndrome: A more aggressive variant of typical carcinoid

Objectives: Our objectives were to delineate the clinicopathologic characteristics of adrenocorticotropin-secreting bronchopulmonary carcinoid tumors causing Cushing's syndrome and to derive from these findings a rational approach to diagnosis and surgical management of this unusual condition. Methods: We conducted a retrospective, chart-review analysis of seven consecutive patients treated at the Massachusetts General Hospital over a 16-year period. Results: The patients uniformly had symptoms of marked hypercortisolism, and the underlying lung lesions remained clinically occult for a mean of 24 months. Standard endocrine testing was misleading in 83% of patients, reinforcing the need for an alternative diagnostic strategy based on petrosal sinus catheterization and computed tomography of the chest. Although 72% of the tumors were typical carcinoids by standard criteria, 57% demonstrated microscopic evidence of local invasiveness, and 43% were associated with mediastinal lymph node metastases. Eighty-six percent of patients have been cured by pulmonary resection a mean of 59 months after the operation, but 50% of these required a second operation for resection of involved lymph nodes after an initial relapse. Conclusions: These data suggest that adrenocorticotropin-secreting bronchopulmonary carcinoid tumors represent a distinct, more aggressive subtype of the usual, typical carcinoid. The high rate of lymphatic and local spread demands a surgical approach consisting of anatomic resection and routine mediastinal lymph node dissection. (J Thorac Cardiovasc Surg 1997;114:367-75)

Hormonal studies in Cushing's syndrome during petrosal sinus catheterization. Part II: Absence of relationship between intestinal vasoactive peptide and prolactin.

Hormonal studies in Cushing's syndrome during petrosal sinus catheterization. Part II: Absence of relationship between intestinal vasoactive peptide and prolactin.

Hormonal Studies in Cushing's Syndrome During Petrosal Sinus Catheterization: Part I: Existence of Multihormonal Gradients

Hormonal Studies in Cushing's Syndrome During Petrosal Sinus Catheterization: Part I: Existence of Multihormonal Gradients

Investigation, management and therapeutic outcome in 12 cases of childhood and adolescent Cushing's syndrome.

Cushing's syndrome in childhood and adolescence is rare. We analysed the clinical presentation, investigation, management and therapeutic outcome in 12 paediatric patients with Cushing's syndrome. Retrospective review of case notes. Twelve patients, 7 males and 5 females, aged 7.6-17.8 years with Cushing's syndrome who were admitted to St Bartholomew's Hospital between 1978 and 1993, were studied. Aetiologies of the Cushing's syndrome patients were: Cushing's disease (9), adrenal adenoma (1), nodular adrenocortical dysplasia (1) and ectopic ACTH syndrome (1). One further male patient, aged 17.8 years who presented with Nelson's syndrome after bilateral adrenalectomy for Cushing's disease in 1978, is described. Presenting symptoms, endocrine tests for hypercortisolism, imaging studies, simultaneous bilateral inferior petrosal sinus sampling and therapeutic strategies are discussed. The dominant clinical features were obesity, short stature, virilization, headaches, fatigue and emotional lability. Investigations confirmed Cushing's syndrome by demonstrating absent cortisol circadian rhythm and impaired suppression on low dose dexamethasone test and differentiated Cushing's disease from other aetiologies by high dose dexamethasone and hCRH tests. In Cushing's disease, pituitary CT scan identified a microadenoma in 4 out of 9 subjects. In 5 of the 9 patients (3 with a normal pituitary CT, 2 with a suggested microadenoma), a pituitary MRI scan was performed and confirmed the CT findings. Inferior petrosal sinus catheterization for ACTH in 4 patients confirmed excess pituitary ACTH secretion, correctly lateralizing the tumour in all cases. Cushing's disease was treated by transsphenoidal surgery alone in 6 patients and combined with pituitary irradiation in 3 patients. Of these 9 patients, 7 are cured and 2 are in remission. The patient with Nelson's syndrome is cured after total hypophysectomy. This series describes the clinical features, aetiologies and management of juvenile Cushing's syndrome. Investigation with low and high-dose dexamethasone suppression tests and hCRH test identified the aetiology in each case. Collaboration between paediatric and adult endocrine units together with an experienced neurosurgeon and a radiotherapist contributed to the successful therapeutic outcome of these patients.

Syndrome de Cushing paranéoplasique. Aspects cliniques. Stratégie diagnostique et thérapeutique

Objectives: Our objectives were to delineate the clinicopathologic characteristics of adrenocorticotropin-secreting bronchopulmonary carcinoid tumors causing Cushing's syndrome and to derive from these findings a rational approach to diagnosis and surgical management of this unusual condition. Methods: We conducted a retrospective, chart-review analysis of seven consecutive patients treated at the Massachusetts General Hospital over a 16-year period. Results: The patients uniformly had symptoms of marked hypercortisolism, and the underlying lung lesions remained clinically occult for a mean of 24 months. Standard endocrine testing was misleading in 83% of patients, reinforcing the need for an alternative diagnostic strategy based on petrosal sinus catheterization and computed tomography of the chest. Although 72% of the tumors were typical carcinoids by standard criteria, 57% demonstrated microscopic evidence of local invasiveness, and 43% were associated with mediastinal lymph node metastases. Eighty-six percent of patients have been cured by pulmonary resection a mean of 59 months after the operation, but 50% of these required a second operation for resection of involved lymph nodes after an initial relapse. Conclusions: These data suggest that adrenocorticotropin-secreting bronchopulmonary carcinoid tumors represent a distinct, more aggressive subtype of the usual, typical carcinoid. The high rate of lymphatic and local spread demands a surgical approach consisting of anatomic resection and routine mediastinal lymph node dissection. (J Thorac Cardiovasc Surg 1997;114:367-75)

Ask, and It Shall be Given You

On Being a Doctor1 September 1994Ask, and It Shall be Given YouTheodore B. Schwartz, MDTheodore B. Schwartz, MD4820 Roberts Road; Boise, ID 83705Search for more papers by this authorAuthor, Article, and Disclosure Informationhttps://doi.org/10.7326/0003-4819-121-5-199409010-00011 SectionsAboutFull TextPDF ToolsAdd to favoritesDownload CitationsTrack CitationsPermissions ShareFacebookTwitterLinkedInRedditEmail These days, with “managed competition” and “cost control” reverberating in our ears, we must remind ourselves that the modern practice of what I call restorative medicine must often rely heavily on laboratory procedures, frequently expensive, for diagnosis and treatment. For example, experts now insist that the specific cause of Cushing syndrome can no longer be firmly established without catheterization of both petrosal sinuses, usually accompanied by measurement of the response of plasma ACTH to corticotropin-releasing hormone. Big money.There is another less obvious but no less worrisome consequence of the emergence of laboratory data as the major determinant of the ... Author, Article, and Disclosure InformationAffiliations: 4820 Roberts Road; Boise, ID 83705Corresponding Author: Theodore B. Schwartz, MD, 4820 Roberts Road, Boise, ID 82705. PreviousarticleNextarticle Advertisement FiguresReferencesRelatedDetails Metrics Cited byA Revival of Paul Dudley WhiteBedside Science Reduces Laboratory Art 1 September 1994Volume 121, Issue 5Page: 376KeywordsAdrenocorticotropic hormoneBlood plasmaCatheterizationChartsHormonesResearch laboratoriesThoraxUrine Issue Published: 1 September 1994 Copyright & PermissionsCopyright © 1994 by American College of Physicians. All Rights Reserved.PDF downloadLoading ...

Ectopic cushing's syndrome caused by an 8 mm lung carcinoid localized by scintigram with the somatostatin analog111in-pentetreotide.

Small tumors producing adrenocorticophic hormone (ACTH) ectopically may be very difficult to locate. We describe a 57-year-old woman who presented with ectopic Cushing's syndrome as diagnosed by bilateral inferior petrosal sinus catheterization with corticotrophin-releasing hormone (CRH) test. Thoracic pentetreotide (a somatostatin analogue) revealed a small "hot spot" in the base of the left lung. This "hot spot" was constant throughout the procedure. A second thoracic CT scan with 3-mm cuts showed a small image in the area under suspicion, similar to vascular images found elsewhere in both lungs. At surgery, an 8-mm tumor was found and excised. Pathological examination revealed a carcinoid tumor immunoreactive for ACTH, beta-endorphin, bombesin, serotonin, and the α-subunit. One month after surgery, the patient was clinically well and had normal adrenal function. An111ln-pentetreotide scintiscan clearly identified a small ACTH-producing neuroendocrine tumor of the lung undetectable by plain chest radiography or CT scan.

Preliminary observations using endocrine markers of pituitary venous dilution during bilateral simultaneous inferior petrosal sinus catheterization in Cushing's syndrome: is combined CRF and TRH stimulation of value?

We determined whether the measurement of hormones in pituitary blood permits correction for dilution by non-pituitary blood during bilateral simultaneous inferior petrosal sinus blood sampling in Cushing's syndrome. Bilateral simultaneous inferior petrosal sinus blood sampling was performed after combined hCRF and TRH stimulation. Peak ACTH concentrations were corrected for the TSH and PRL inter-sinus ratio, assuming uniform secretion of both hormones into each inferior petrosal sinus. Eight patients with clinical and biochemical features of Cushing's syndrome. Basal and stimulated ACTH, TSH and PRL concentrations were measured after bilateral simultaneous inferior petrosal sinus blood sampling and simultaneously from a peripheral forearm vein. Basal central:peripheral ACTH ratio misdiagnosed four of eight patients as having non-pituitary disease. Peak uncorrected ACTH central:peripheral ratio erroneously suggested two of eight patients had non-pituitary disease. ACTH central:peripheral ratio corrected by TSH and PRL correctly predicted pituitary-dependent disease in all eight cases and provided correct lateralization data in four of five patients with a unilateral pituitary microadenoma. This study suggests that measuring other hormones in pituitary blood after TRH stimulation can offer a simple and reliable method for correcting for dilution by non-pituitary blood during bilateral simultaneous inferior petrosal sinus blood sampling in Cushing's syndrome.