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NCBI: db=pubmed; Term=adrenal tumor
Updated: 6 days 22 hours ago

Current Management of Pheochromocytoma/Paraganglioma: A Guide for the Practicing Clinician in the Era of Precision Medicine.

Fri, 10/11/2019 - 07:45
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Current Management of Pheochromocytoma/Paraganglioma: A Guide for the Practicing Clinician in the Era of Precision Medicine.

Cancers (Basel). 2019 Oct 08;11(10):

Authors: Nölting S, Ullrich M, Pietzsch J, Ziegler CG, Eisenhofer G, Grossman A, Pacak K

Abstract
Pheochromocytomas and paragangliomas (PCC/PGLs) are rare, mostly catecholamine-producing neuroendocrine tumors of the adrenal gland (PCCs) or the extra-adrenal paraganglia (PGL). They can be separated into three different molecular clusters depending on their underlying gene mutations in any of the at least 20 known susceptibility genes: The pseudohypoxia-associated cluster 1, the kinase signaling-associated cluster 2, and the Wnt signaling-associated cluster 3. In addition to tumor size, location (adrenal vs. extra-adrenal), multiplicity, age of first diagnosis, and presence of metastatic disease (including tumor burden), other decisive factors for best clinical management of PCC/PGL include the underlying germline mutation. The above factors can impact the choice of different biomarkers and imaging modalities for PCC/PGL diagnosis, as well as screening for other neoplasms, staging, follow-up, and therapy options. This review provides a guide for practicing clinicians summarizing current management of PCC/PGL according to tumor size, location, age of first diagnosis, presence of metastases, and especially underlying mutations in the era of precision medicine.

PMID: 31597347 [PubMed]

A Micellar Mitotane Formulation with High Drug-Loading and Solubility: Physico-Chemical Characterization and Cytotoxicity Studies in 2D and 3D In Vitro Tumor Models.

Fri, 10/11/2019 - 07:45
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A Micellar Mitotane Formulation with High Drug-Loading and Solubility: Physico-Chemical Characterization and Cytotoxicity Studies in 2D and 3D In Vitro Tumor Models.

Macromol Biosci. 2019 Oct 09;:e1900178

Authors: Haider MS, Schreiner J, Kendl S, Kroiss M, Luxenhofer R

Abstract
Adrenocortical carcinoma (ACC) is a rare tumor and prognosis is overall poor but heterogeneous. Mitotane (MT) has been used for treatment of ACC for decades, either alone or in combination with cytotoxic chemotherapy. Even at doses up to 6 g per day, more than half of the patients do not achieve targeted plasma concentration (14-20 mg L-1 ) even after many months of treatment due to low water solubility, bioavailability, and unfavorable pharmacokinetic profile. Here a novel MT nanoformulation with very high MT concentrations in physiological aqueous media is reported. The MT-loaded nanoformulations are characterized by Fourier transform infrared spectroscopy, differential scanning calorimetry, and powder X-ray diffraction which confirms the amorphous nature of the drug. The polymer itself does not show any cytotoxicity in adrenal and liver cell lines. By using the ACC model cell line NCI-H295 both in monolayers and tumor cell spheroids, micellar MT is demonstrated to exhibit comparable efficacy to its ethanol solution. It is postulated that this formulation will be suitable for i.v. application and rapid attainment of therapeutic plasma concentrations. In conclusion, the micellar formulation is considered a promising tool to alleviate major drawbacks of current MT treatment while retaining bioactivity toward ACC in vitro.

PMID: 31596553 [PubMed - as supplied by publisher]

Timeline of Advances in Genetics of Primary Aldosteronism.

Fri, 10/11/2019 - 07:45
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Timeline of Advances in Genetics of Primary Aldosteronism.

Exp Suppl. 2019;111:213-243

Authors: Meyer LS, Reincke M, Williams TA

Abstract
The overwhelming majority of cases of primary aldosteronism (PA) occur sporadically due to a unilateral aldosterone-producing adenoma (APA) or bilateral idiopathic adrenal hyperplasia. Familial forms of PA are rare with four subtypes defined to date (familial hyperaldosteronism types I-IV). The molecular basis of familial hyperaldosteronism type I (FH type I or glucocorticoid-remediable aldosteronism) was established in 1992; two decades later the genetic variant causing FH type III was identified and germline mutations causing FH type IV and FH type II were determined soon after. Effective diagnostic protocols and methods to detect the overactive gland in unilateral PA by adrenal venous sampling followed by laparoscopic adrenalectomy have made available APAs for scientific studies. In rapid succession, following the widespread use of next-generation sequencing, recurrent somatic driver mutations in APAs were identified in genes encoding ion channels and transporters. The development of highly specific monoclonal antibodies against key enzymes in adrenal steroidogenesis has unveiled the heterogeneous features of the diseased adrenal in PA and helped reveal the high proportion of APAs with driver mutations. We discuss what is known about the genetics of PA that has led to a clearer understanding of the disease pathophysiology.

PMID: 31588534 [PubMed - indexed for MEDLINE]

Diseases Predisposing to Adrenocortical Malignancy (Li-Fraumeni Syndrome, Beckwith-Wiedemann Syndrome, and Carney Complex).

Fri, 10/11/2019 - 07:45
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Diseases Predisposing to Adrenocortical Malignancy (Li-Fraumeni Syndrome, Beckwith-Wiedemann Syndrome, and Carney Complex).

Exp Suppl. 2019;111:149-169

Authors: Jouinot A, Bertherat J

Abstract
Adrenocortical malignancies can occur in the context of several tumor predisposition syndromes.The Carney complex (CNC) is responsible for the majority of primary pigmented nodular adrenal diseases and is more rarely associated with adrenocortical carcinoma (ACC). Other core manifestations of CNC include cardiac and cutaneous myxomas, lentiginosis, somatotroph pituitary adenomas, Sertoli tumors, melanocytic schwannoma, and thyroid, breast, and bone tumors. CNC is mostly due to germline inactivating mutations of PRKAR1A.The majority of childhood ACC are related to genetic predisposition. The Beckwith-Wiedemann syndrome (BWS) is an overgrowth and tumor predisposition syndrome due to genetic or epigenetic alterations at the 11p15 locus. Classical tumor spectrum of BWS includes embryonal tumors and childhood ACC. The Li-Fraumeni syndrome (LFS) is a devastating tumor predisposition syndrome, due to germline inactivating mutations of TP53, and characterized by a high, various, and early-onset cancer risk. LFS spectrum includes premenopausal breast cancer, soft-tissue sarcoma, osteosarcoma, central nervous system tumor, and ACC, accounting for 50-80% of pediatric cases. Finally, germline predisposition affects up to 10% of adult ACC patients, mostly in part of LFS and Lynch syndrome.This chapter focuses on the diagnosis, screening, and management of adrenal tumors in part of these tumor predisposition syndromes.

PMID: 31588532 [PubMed - indexed for MEDLINE]

Hereditary Diseases Predisposing to Pheochromocytoma (VHL, NF-1, Paraganglioma Syndromes, and Novel Genes).

Fri, 10/11/2019 - 07:45
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Hereditary Diseases Predisposing to Pheochromocytoma (VHL, NF-1, Paraganglioma Syndromes, and Novel Genes).

Exp Suppl. 2019;111:129-147

Authors: Sarkadi B, Patócs A

Abstract
Pheochromocytomas (Pheo) and paragangliomas (PGL) are rare tumors originating from catecholamine-producing chromaffin cells. They occur approximately in 0.1% of patients affected with hypertonia. Pheo/PGL may manifest itself at any age; in 10% of the patients, the disease is bilateral, and also in 10% it occurs outside of the adrenal medulla. From a genetic aspect, a considerable proportion of these tumors represents a prototype for an autosomal dominantly inherited syndrome with incomplete penetrance. In addition, to date more than 15 genes have been identified representing genetic susceptibility for Pheo/PGL and accounting for 40% of all cases. In general, in familiar cases, the tumor manifests at younger age, and they are often occurring as multiplex tumors. Permanent recovery can be achieved with an early diagnosis and with a successful surgical removal of the tumor tissue. On the other hand, undiagnosed, hormonally active Pheos may lead to severe, or even lethal, consequences. This chapter will summarize our recent knowledge about the genetics of Pheo/PGL, focusing on tumor syndromes where Pheo/PGLs are among the main manifestations.

PMID: 31588531 [PubMed - indexed for MEDLINE]

Retroperitoneal extraosseous peripheral primitive neuroectodermal tumor in a Formosan serow: case report and literature review.

Fri, 10/11/2019 - 07:45
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Retroperitoneal extraosseous peripheral primitive neuroectodermal tumor in a Formosan serow: case report and literature review.

J Vet Diagn Invest. 2019 Oct 04;:1040638719879198

Authors: Hsieh YH, Hsu YH, Lien CY, Liu CH, Li WT

Abstract
A 10-y-old female captive Formosan serow (Capricornis swinhoei) was inactive and was azotemic. An autopsy was performed following her death, and multiple irregularly shaped, white-to-gray masses of 0.5-2 cm diameter were noted on both ureters, the left adrenal gland, urinary bladder, and uterus. Microscopically, organs were effaced by a poorly demarcated, highly infiltrative neoplasm, composed of neoplastic round cells arranged in islands, sheets, or nests with occasional rosette formation. The neoplastic cells were small: ≤2 red blood cell (≤ 15 μm) diameter. The neoplastic cells were positive for CD56, CK, FLI-1, and NSE, but negative for desmin, GFAP, melan A, NF, PAX-8, S100, synaptophysin, and vimentin. Therefore, the diagnosis of retroperitoneal extraosseous peripheral primitive neuroectodermal tumor (pPNET) was made. pPNET with FLI-1 expression has not been reported previously in animals, to our knowledge.

PMID: 31585511 [PubMed - as supplied by publisher]

Coincidental Central Precocious Puberty and Wilms Tumor in a 5-Year-Old Girl.

Fri, 10/11/2019 - 07:45
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Coincidental Central Precocious Puberty and Wilms Tumor in a 5-Year-Old Girl.

Case Rep Pediatr. 2019;2019:5427207

Authors: Kasongo L, Forget P, Nicolescu RC

Abstract
Wilms tumor is the most frequent pediatric renal malignancy, and its usual presentation is an abdominal mass or hematuria. Unusual presentations have also been reported, such as paraneoplastic syndromes (acquired von Willebrand disease, sudden death due to pulmonary embolism, and Cushing syndrome). These conditions can precede, occur concomitantly, or present in a later phase of tumor development. Precocious puberty, as paraneoplastic endocrine syndrome, has already been described in children with malignant tumors (brain, gonadal, adrenal tumors, and hepatoblastoma). However, little is known about central precocious puberty, as paraneoplastic manifestation of nephroblastoma or secondary to its specific chemotherapy. Here, we report a case of Wilms tumor and simultaneous precocious puberty in a 5-year-old girl. The initial diagnosis was premature telarche, but the clinical and biological pubertal progression changed our diagnosis to idiopathic central precocious puberty. Chemotherapy and nephrectomy were well tolerated, and we began treatment with a gonadotropin-releasing hormone agonist which showed favorable outcomes over the short term. We highlight the need for early diagnosis and work-up in all patients of precocious puberty, in order to institute timely management.

PMID: 31583153 [PubMed]

A 45-year-old woman with episodic anxiety, hypertension and diabetes mellitus.

Fri, 10/11/2019 - 07:45
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A 45-year-old woman with episodic anxiety, hypertension and diabetes mellitus.

Natl Med J India. 2018 May-Jun;31(3):160-163

Authors:

PMID: 31044765 [PubMed - indexed for MEDLINE]

[Two case reports on resistant hypertension].

Fri, 10/11/2019 - 07:45
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[Two case reports on resistant hypertension].

Internist (Berl). 2019 05;60(5):529-532

Authors: Beger C, Haller H, Limbourg FP

Abstract
Primary aldosteronism (PA) is a frequent cause of resistant hypertension. The clinical presentation is heterogeneous, but a suppressed or low normal renin (especially with ACE inhibitors or sartans) should raise suspicion for primary aldosteronism, even when aldosterone levels are in the normal range. Diagnosis of unilateral hormone production from an adrenal adenoma (Conn syndrome), which is curable by surgery, requires adrenal vein sampling, which should be performed in experienced centers.

PMID: 30707244 [PubMed - indexed for MEDLINE]

Type A Aortic Dissection Complicated by Pheochromocytoma.

Fri, 10/11/2019 - 07:45
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Type A Aortic Dissection Complicated by Pheochromocytoma.

Ann Thorac Surg. 2019 01;107(1):e13-e14

Authors: Runyan B, Hanak CR, Mahendiran S, Allamaneni S, Vester S

Abstract
This report presents a case of aortic dissection as the patient's initial presentation of an undiagnosed pheochromocytoma. A 36-year-old man presented with substernal chest pressure and abdominal pain. Computed tomography revealed type A aortic dissection with a 3.6-cm left adrenal mass. Elevated catecholamine levels were diagnostic of pheochromocytoma. Type A aortic dissection caused by uncontrolled hypertension secondary to pheochromocytoma is a rare entity. This can complicate surgical planning. Although this situation is rare, it is important to consider pheochromocytoma in the differential diagnosis of uncontrolled hypertension in the setting of type A aortic dissection.

PMID: 30558735 [PubMed - indexed for MEDLINE]

Discussion.

Fri, 10/11/2019 - 07:45
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Discussion.

Surgery. 2019 01;165(1):210

Authors:

PMID: 30527005 [PubMed - indexed for MEDLINE]

Discussion.

Fri, 10/11/2019 - 07:45
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Discussion.

Surgery. 2019 01;165(1):200-201

Authors:

PMID: 30527004 [PubMed - indexed for MEDLINE]

Discussion.

Fri, 10/11/2019 - 07:45
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Discussion.

Surgery. 2019 01;165(1):194-195

Authors:

PMID: 30527003 [PubMed - indexed for MEDLINE]

Association of Patient Frailty With Increased Risk of Complications After Adrenalectomy.

Fri, 10/11/2019 - 07:45
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Association of Patient Frailty With Increased Risk of Complications After Adrenalectomy.

JAMA Surg. 2018 10 01;153(10):966-967

Authors: Anderson JE, Seib CD, Campbell MJ

PMID: 29971357 [PubMed - indexed for MEDLINE]

Corticosteroid use endpoints in neuro-oncology: Response Assessment in Neuro-Oncology Working Group.

Fri, 10/11/2019 - 07:45
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Corticosteroid use endpoints in neuro-oncology: Response Assessment in Neuro-Oncology Working Group.

Neuro Oncol. 2018 06 18;20(7):897-906

Authors: Arvold ND, Armstrong TS, Warren KE, Chang SM, DeAngelis LM, Blakeley J, Chamberlain MC, Dunbar E, Loong HH, Macdonald DR, Reardon DA, Vogelbaum MA, Yuan Y, Weller M, van den Bent M, Wen PY

Abstract
Background: Corticosteroids are the mainstay of treatment for peritumor edema but are often associated with significant side effects. Therapies that can reduce corticosteroid use would potentially be of significant benefit to patients. However, currently there are no standardized endpoints evaluating corticosteroid use in neuro-oncology clinical trials.
Methods: The Response Assessment in Neuro-Oncology (RANO) Working Group has developed consensus recommendations for endpoints evaluating corticosteroid use in clinical trials in both adults and children with brain tumors.
Results: Responders are defined as patients with a 50% reduction in total daily corticosteroid dose compared with baseline or reduction of the total daily dose to ≤2 mg of dexamethasone (or equivalent dose of other corticosteroid); baseline dose must be at least 4 mg of dexamethasone daily (or equivalent dose of other corticosteroids) for at least one week. Patients must have stable or improved Neurologic Assessment in Neuro-Oncology (NANO) score or Karnofsky performance status score or Eastern Cooperative Oncology Group (ECOG) (Lansky score for children age <16 y), and an improved score on a relevant clinical outcome assessment tool. These criteria must be sustained for at least 4 weeks after baseline assessment to be considered a response, and are confirmed 4 weeks after that (ie, 8 wk after baseline assessment) to be considered a sustained response.
Conclusions: This RANO proposal for corticosteroid use endpoints in neuro-oncology clinical trials may need to be refined and will require prospective validation in clinical studies.

PMID: 29788429 [PubMed - indexed for MEDLINE]

Composite Pheochromocytoma: A Rare Form of Tumor.

Fri, 10/04/2019 - 06:38
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Composite Pheochromocytoma: A Rare Form of Tumor.

Indian J Nephrol. 2019 Sep-Oct;29(5):307-308

Authors: Lakshminarayana GR

Abstract
The pheochromocytomas are one of the rare and curable causes of secondary hypertension arising from adrenal medulla, commonly presenting with hypertension; either paroxysmal or persistent. Very rarely they may show cells belonging to more than one line of differentiation and are called as mixed or composite pheochromocytoma.

PMID: 31571735 [PubMed]

The Prognostic Role of Pretreatment Neutrophil to Lymphocyte Ratio (NLR) in Malignant Adrenal Lesions Treated With Stereotactic Body Radiation Therapy (SBRT).

Fri, 10/04/2019 - 06:38
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The Prognostic Role of Pretreatment Neutrophil to Lymphocyte Ratio (NLR) in Malignant Adrenal Lesions Treated With Stereotactic Body Radiation Therapy (SBRT).

Am J Clin Oncol. 2019 Sep 27;:

Authors: Mills MN, Reddy AV, Richardson L, Richardson KM, Kersh CR

Abstract
OBJECTIVE: The objective of this study was to evaluate a single institution's experience with stereotactic body radiotherapy (SBRT) in treating malignant adrenal lesions, as well as the prognostic value of systemic inflammation biomarkers.
MATERIALS AND METHODS: From November 2007 to February 2018, 27 patients with malignant adrenal lesions received 31 SBRT treatments. Outcomes, measured from the date of SBRT, included overall survival (OS), local control (LC), and freedom from progression. Cox proportional hazard model was utilized to identify potential prognostic factors. Tumor response was assessed with PET Response Evaluation Criteria In Solid Tumors (PERCIST)/Response Evaluation Criteria in Solid Tumors (RECIST) criteria. Acute toxicity was evaluated with the National Cancer Institute Common Terminology Criteria for Adverse Events, version 4.03 criteria.
RESULTS: Median follow-up for all patients was 8 months. The complete response, partial response, stable disease, and progressive disease rates were 59%, 9%, 32%, and 0%, respectively. One-year LC, OS, and freedom from progression were 77.7%, 38.0%, and 10.0%, respectively. There was a trend toward significance upon multivariate analysis for pretreatment neutrophil to lymphocyte ratio >4.1 to predict inferior OS (adjusted hazard ratio=3.29, P=0.09, 1-year OS: 11% vs. 80%). There were 3 cases (10%) complicated by grade 2 acute toxicity, including nausea and fatigue. There was 1 grade 5 toxicity, as 1 case was complicated by a fatal gastric ulcer occurring 3 months after SBRT to the left adrenal gland (112.5 BED10).
CONCLUSIONS: These results support the limited existing literature, demonstrating that SBRT provides adequate LC for adrenal lesions with minimal toxicity. Pretreatment neutrophil to lymphocyte ratio may serve as a prognostic factor in these patients.

PMID: 31569166 [PubMed - as supplied by publisher]

KRAS rs7973450 A>G increases neuroblastoma risk in Chinese children: a four-center case-control study.

Fri, 10/04/2019 - 06:38
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KRAS rs7973450 A>G increases neuroblastoma risk in Chinese children: a four-center case-control study.

Onco Targets Ther. 2019;12:7289-7295

Authors: Lin A, Hua RX, Tang J, Zhu J, Zhang R, Zhou H, Zhang J, Cheng J, Xia H, He J

Abstract
Background: Neuroblastoma is one of the most common extracranial solid pediatric tumors. KRAS plays an important role in regulating cell proliferation, differentiation, and apoptosis. Single nucleotide polymorphisms (SNPs) in KRAS have been shown to modify susceptibility to multiple tumors, but no specific molecular epidemiology study was reported regarding neuroblastoma.
Methods: We conducted a four-center case-control study to explore the association between KRAS gene polymorphisms (rs12587 G>T, rs7973450 A>G, rs7312175 G>A) and neuroblastoma susceptibility with 505 Chinese children and 1070 matched controls.
Results: We found that rs7973450 A>G was associated with significantly increased neuroblastoma risk [GG vs. AA: adjusted odds ratio (OR)=4.26, 95% confidence interval (CI)=1.28-14.19, P=0.018; GG vs. AA/AG: adjusted OR=4.27, 95% CI=1.28-14.24, P=0.018]. The stratified analysis further demonstrated that rs7973450 GG genotype carriers had a higher risk to develop neuroblastoma in the subgroups of males, tumor originated from the adrenal gland and clinical stages III+IV.
Conclusions: Overall, our results suggested that rs7973450 A>G was associated with increased neuroblastoma risk.

PMID: 31564912 [PubMed]

Adrenal Adenoma-Hemangioma Collision Tumor: Description of Two Cases.

Fri, 10/04/2019 - 06:38
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Adrenal Adenoma-Hemangioma Collision Tumor: Description of Two Cases.

J Radiol Case Rep. 2019 Jun;13(6):1-12

Authors: Foresti M, Parmiggiani A

Abstract
Adrenal collision tumors are rare clinical entities referring to separate coexisting adjacent tumors involving an adrenal gland with sharp demarcation between the two and without a substantial histologic admixture at the interface. Most of the adrenal collision tumors described are combinations of adenoma and metastasis or adenoma and myelolipoma. We report two cases of a 63-year-old male and a 76-year-old female patient with a presumable exceedingly rare adrenal hemangioma-adenoma collision tumor. To our knowledge, only two reports of a collision tumor comprising an adrenal hemangioma and an adenoma have been described in literature.

PMID: 31558958 [PubMed - in process]

Early postoperative HPA-axis testing after pituitary tumor surgery: reliability and safety of basal cortisol and CRH test.

Fri, 10/04/2019 - 06:38
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Early postoperative HPA-axis testing after pituitary tumor surgery: reliability and safety of basal cortisol and CRH test.

Endocrine. 2019 Sep 25;:

Authors: de Vries F, Lobatto DJ, Bakker LEH, van Furth WR, Biermasz NR, Pereira AM

Abstract
PURPOSE: To assess the reliability and safety of a postsurgical evaluation strategy of adrenal function using CRH stimulation and basal cortisol concentrations after transsphenoidal pituitary surgery.
METHODS: Retrospective cohort study of all patients undergoing endoscopic transsphenoidal surgery from 2010 to 2017, in whom early postoperative basal cortisol and/or CRH-stimulated cortisol secretion were available, including confirmation of adrenal function during follow-up. Patients with Cushing's disease were excluded. Optimal test performances were assessed using ROC analysis.
RESULTS: A total of 156 patients were included. Sensitivity and specificity of the CRH test were 78% and 90%, respectively, and 86% and 92% for basal cortisol, respectively, using an optimal cutoff of 220 nmol/L. Eight patients had false-negative test results with the CRH test (normal test but adrenal insufficient at follow-up), and six patients with basal cortisol, the majority of which had multiple pituitary hormone deficiencies and fluid imbalances. No clinical adverse events occurred in patients with false-negative test results. The diagnostic performance of a single basal cortisol measurement was superior to the CRH test.
CONCLUSIONS: The early postoperative basal cortisol is a safe and simple measurement to guide (dis)continuation of hydrocortisone replacement. However, disturbing factors, e.g., sodium balance disorders, contraceptives, untreated hypopituitarism, and illness impact the interpretation and in those cases this measure is unreliable. We propose an algorithm in which hydrocortisone replacement at discharge is based on basal cortisol <220 nmol/L on postoperative day 2 or 3 in a stable condition.

PMID: 31556005 [PubMed - as supplied by publisher]

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