scholarly journals Presentation, Treatment, Histology, and Outcomes in Adrenal Medullary Hyperplasia Compared With Pheochromocytoma

2019 ◽  
Vol 3 (8) ◽  
pp. 1518-1530 ◽  
Author(s):  
Henrik Falhammar ◽  
Adam Stenman ◽  
Jan Calissendorff ◽  
Carl Christofer Juhlin
Keyword(s):  
Blood Pressure ◽  
Surgical Management ◽  
Cushing Syndrome ◽  
Adrenocortical Adenoma ◽  
Cushing Disease ◽  
Genetic Syndrome ◽  
Alpha Blockers ◽  
Adrenal Medullary Hyperplasia ◽  
Symptoms And Signs

Abstract Context Information about adrenal medullary hyperplasia (AMH) is scarce. Objective To study a large cohort of AMHs. Design, Setting, and Participants Nineteen AMH cases were compared with 95 pheochromocytomas (PCCs) without AMH. AMH without (n = 7) and with PCC (n = 12) were analyzed separately. Results Of 936 adrenalectomies, 2.1% had AMH. Mean age was 47.2 ± 15.1 years. Only two (11%) AMHs had no concurrent PCC or adrenocortical adenoma. In AMHs, a genetic syndrome was present in 58% vs 4% in PCCs (P < 0.001). The noradrenaline/metanephrine levels were lower in AMHs, whereas suppression of dexamethasone was less than in PCCs. Cushing syndrome was found in 11% of AMHs. More AMHs were found during screening and less as incidentalomas. PCC symptoms were less prevalent in AMHs. Surgical management was similar; however, fewer of the AMHs were pretreated with alpha-blockers. Adrenalectomy improved blood pressure slightly less in AMHs. The disappearance of glycemic disturbances was similar to the PPCs. During a period of 11.2 ± 9.4 years, a new PCC developed in 32% of patients with AMH, 11% died, but no PCC metastasis occurred (PCCs: 4%, P < 0.001; 14% and 5%). AMHs without PCC had milder symptoms but more often Cushing disease than patients with PCC, whereas AMH with PCC more often displayed a familiar syndrome with more PCC recurrences. Conclusion A total of 2.1% of all adrenalectomies displayed AMH. AMH seemed to be a PCC precursor. The symptoms and signs were milder than PCCs. AMHs were mainly found due to screening. Outcomes seemed favorable, but new PCCs developed in many during follow-up.

Image-Guided Radiofrequency Ablation for Adrenocortical Adenoma with Cushing Syndrome: Outcomes After Mean Follow-up of 33 Months

Urology ◽  
2007 ◽  
Vol 70 (3) ◽  
pp. 407-411 ◽  
Author(s):  
Kiminobu Arima ◽  
Kouichirou Yamakado ◽  
Ryuichi Suzuki ◽  
Hiroshi Matsuura ◽  
Atsuhiro Nakatsuka ◽  
...  
Keyword(s):  
Radiofrequency Ablation ◽  
Cushing Syndrome ◽  
Adrenocortical Adenoma ◽  
Image Guided

scholarly journals ACTH adenomas transforming their clinical expression: report of 5 cases

2015 ◽  
Vol 38 (2) ◽  
pp. E15 ◽  
Author(s):  
Matteo Zoli ◽  
Marco Faustini-Fustini ◽  
Diego Mazzatenta ◽  
Gianluca Marucci ◽  
Eugenio De Carlo ◽  
...  
Keyword(s):  
Pituitary Adenomas ◽  
Cushing Syndrome ◽  
Brain Mri ◽  
Gamma Knife Radiosurgery ◽  
Spontaneous Resolution ◽  
Ophthalmological Examination ◽  
Clinical Expression ◽  
Cell Adenoma ◽  
Cushing Disease

OBJECT Adrenocorticotropic hormone (ACTH) adenomas have been recognized as a more aggressive and invasive subtype of pituitary adenomas. An additional and clinically relevant peculiarity of these tumors is their ability to modify their clinical expression from a silent form to Cushing disease or vice versa. The aim of this study was to review a series of patients with pituitary adenomas and analyze the clinical implications of the transformation of clinical expression in 5 cases that showed this phenomenon. METHODS The authors retrospectively reviewed a series of patients with pituitary adenoma and collected clinical, biohumoral, and neuroradiological data of those who presented with a transformation from silent ACTH adenomas to functioning tumors or vice versa. In all the cases, preoperative assessment consisted of brain MRI, ophthalmological examination, and complete baseline endocrinological investigation. In patients with clinical and/or biochemical findings suspicious for Cushing syndrome, a low-dose dexamethasone suppression test was performed to rule in or out this diagnosis. Endocrinological evaluations were repeated 1 month after surgery, 3 months after surgery, and every 6 months or annually thereafter. Ophthalmological evaluations and brain MRIs were repeated after 3 months and then every 6 or 12 months thereafter. RESULTS Five patients (2 men and 3 women) included in this series had corticotropic tumors that showed transformation from an endocrinologically silent form to manifest Cushing disease and vice versa. The mean age at presentation was 40 years (range 18–51 years). In 3 of these patients, a transformation from silent to functioning ACTH adenoma with manifest Cushing disease occurred. In 1 patient, the authors observed the transition from a functioning to a silent adenoma with spontaneous resolution of hypercortisolism. Another patient's silent adenoma “shifted” to a functioning adenoma and then regressed back to a silent form with spontaneous resolution of Cushing disease. This patient again developed hypercortisolism, which finally resolved spontaneously. In this series, the transformation occurred after a mean of 3.5 years (range 6 months to 7 years). The shift from an ACTH-silent to a functioning adenoma was observed in 9% of the ACTH-silent adenomas in this series (4 of 44 cases), and the spontaneous remission of Cushing disease to a silent corticotroph cell adenoma occurred in 1.5% of cases of this series (2 of 132 functioning ACTH adenomas). At follow-up (mean 107 months; range 60–177 months), cortisol levels were within normal limits in all 5 cases. However, 1 patient required Gamma Knife radiosurgery and eventually adrenalectomy for disease control to be achieved. CONCLUSIONS The ability of silent ACTH adenomas to transform their secretion pattern poses a challenge for neurosurgeons and endocrinologists. Because the transformation is often unexpected, the clinical and biochemical data can be underestimated. Furthermore, this bizarre and unpredictable postoperative tumor behavior can lead to misinterpretation of clinical and endocrinological outcomes. Even if these cases are very rare, they are not anecdotal in large series. Thus, ACTH adenomas require careful biohumoral and neuroradiological follow-up to detect possible transformations.

scholarly journals SAT-142 Periodic Cushing Syndrome in a Patient with an Intestinal Neuroendocrine Tumor (NET). A Novel Case Report

2020 ◽  
Vol 4 (Supplement_1) ◽  
Author(s):  
Nektaria Papadopoulou-Marketou ◽  
Ern Typhoxylou ◽  
Th Kounadi ◽  
Piaditis George ◽  
George P Chrousos ◽  
...  
Keyword(s):  
Small Intestine ◽  
Cushing Syndrome ◽  
Neuroendocrine Tumour ◽  
Neuroendocrine Neoplasm ◽  
Adrenocortical Adenoma ◽  
Limb Weakness ◽  
Cushing Disease ◽  
Ectopic Acth ◽  
First Case ◽  
Free Cortisol

Abstract Introduction A major diagnostic enigma regarding ACTH-dependent Cushing syndrome is the distinction between the source of ACTH, which may have either a pituitary or ectopic origin. We present the first described patient with cyclic Cushing syndrome caused by an intestinal neuroendocrine tumour (NET) detected by 68GA-DOTATATE PET-CT, despite functional tests that were indicative of pituitary Cushing disease. Clinical Case A 53-year old man was admitted to outpatient clinic because of muscle weakness. His phenotype and clinical findings (progressively worsening upper and lower limb weakness, emotional disturbances, easy bruising, a Buffalo hump, prediabetes and leucocytosis) led to the diagnosis of Cushing syndrome. Initial laboratory tests established the diagnosis with an abnormal diurnal cortisol and ACTH secretion (night cortisol F: 22.1μg / dl), absence of suppression with dexamethasone 1mg and increased free urinary free cortisol 24h (243.5μg /24h). Abdominal CT scanning revealed a left-sided adrenal adrenocortical adenoma 1.5 mm in max diameter. Pituitary MRI and somatostatin scintigraphy were normal. Low dexamethasone suppression test was indicative of Cushing (F: 14μg / dl) followed by a combined CRH stimulation test during bilateral inferior petrosal sinus sampling. Pituitary / peripheral ACTH ratio pre-infusion of CRH and 3 min after CRH infusion was compatible with right-sided pituitary origin of ACTH hypersecretion. Pending the results of the laboratory, the patient showed a remission of his symptoms along with a laboratory-confirmed recession of active hypercortisolaemia (LDDST test), and this led to the suspicion of periodic Cushing syndrome. The patient was followed with clinical and laboratory examinations weekly, with recurrence of symptoms 2 months later followed by a new remission 3 months later. A PETGA CT SCAN with 68GA-HA-DOTATATE was performed, which showed an increased uptake of the radioisotope in the small intestine. A surgical excision of the affected small bowel region was performed according to the guidelines for intestinal NETs. Histology confirmed the existence of a well-differentiated neuroendocrine neoplasm of the small intestine of 1.1 cm diameter, grade 1 (WHO 2010). Immunophenotype was positive for serotonin and ACTH. Postoperatively, the patient showed a complete remission of symptomatology and regression of hypercortisolaemia over a 18-month period. Follow-up abdominal MRI and 68GA-HA-DOTATATE revealed no pathological findings. Conclusion: Our patient is the first case of ectopic Cushing disease caused by intestinal NET. The differential diagnosis between pituitary and ectopic Cushing syndrome due to ACTH or CRH hypersecretion is not easy and frequently complicated by the periodicity of the disease. In patients with no visible pituitary lesions on MRI we suggest further investigation for ectopic ACTH- driven Cushing syndrome.

scholarly journals Mifepristone Increases Thyroid Hormone Requirements in Patients With Central Hypothyroidism: A Multicenter Study

2019 ◽  
Vol 3 (9) ◽  
pp. 1707-1714 ◽  
Author(s):  
Francisco J Guarda ◽  
James Findling ◽  
Kevin C J Yuen ◽  
Maria Fleseriu ◽  
Lisa B Nachtigall
Keyword(s):  
Thyroid Hormone ◽  
Hormone Level ◽  
Cushing Syndrome ◽  
Case Series ◽  
The United States ◽  
Thyroid Status ◽  
Central Hypothyroidism ◽  
Cushing Disease ◽  
Retrospective Database

Abstract Purpose Mifepristone is a glucocorticoid and progesterone receptor blocker that can be used for patients with hyperglycemia and Cushing syndrome in whom surgery failed to achieve remission or who were ineligible for surgery. We report a case series of patients with Cushing disease (CD) and central hypothyroidism that presented with increased levothyroxine requirements during mifepristone therapy. Methods Retrospective longitudinal case series of patients with CD and central hypothyroidism treated with mifepristone in a retrospective database at four pituitary centers in the United States. Results Five patients with CD were found, all women, median age 50 (interquartile range 47 to 64.5). They received mifepristone because no adequate response or intolerance to other drugs was observed. Mifepristone initiation was associated with a decrease in free thyroxine levels, mandating a dose increase of a median 1.83 (1.71 to 3.5) times the initial dose of levothyroxine to achieve normal levels. Weight loss was seen in four of five patients, ranging from 3.2 to 42.6 kg in up to 54 months of follow-up. Conclusions Although the mechanism behind the decrease in thyroid hormone level is unknown, intestinal malabsorption, decreased residual thyroid function and increased inactivation of T4 via deiodinases are all potential causes. Whereas therapies for hypercortisolism aim to decrease features of hypercortisolemia such as weight gain and depression, hypothyroidism can hamper these goals. This case series raises awareness on the importance of assessment of thyroid status in patients receiving mifepristone to optimize clinical outcomes.

scholarly journals Telomere Length Changes in Children With Cushing Disease: A Pilot Study

2020 ◽  
Vol 4 (7) ◽  
Author(s):  
Christina Tatsi ◽  
Chelsi Flippo ◽  
Fabio R Faucz ◽  
Ninet Sinaii ◽  
Constantine A Stratakis
Keyword(s):  
Blood Pressure ◽  
Pilot Study ◽  
Telomere Length ◽  
Pediatric Patients ◽  
Inverse Correlation ◽  
Cushing Disease ◽  
Total Lymphocyte ◽  
Length Changes ◽  
Active Disease

Abstract Context Changes in telomere length (TL) have been linked to certain diseases. Studies on the effect of cortisol on TL have not led to conclusive results. Objective To determine whether TL is affected in pediatric patients with Cushing disease (CD) through an exploratory study. Design We studied 10 pediatric patients [mean age: 13.3 (2.6) years, 7 females], diagnosed and treated successfully for CD. TL was measured before and approximately 1 year after treatment. TL was compared with controls adjusting for age, and associations with disease characteristics were assessed. Results Adjusting for age, total lymphocyte TL of patients did not differ from controls during active disease (P = 0.13) but was shorter than controls at follow-up (P = 0.031). Total lymphocyte TL during active CD and at follow-up did not correlate with markers of hypercortisolemia. There was strong inverse correlation between TL during active disease and at follow-up with triglyceride levels at active disease (adjusted [Adj] R2 = 0.64; P = 0.02 and Adj R2 = 0.5; P = 0.036, respectively), suggesting that the higher the triglycerides, the shorter the TL in patients with CD. The change of TL between active disease and follow-up was positively correlated with systolic blood pressure (Adj R2 = 0.76; P = 0.006). Conclusions In this pilot study, TL is shorter in children with hypercortisolemia, a difference that becomes detectable only after cure of CD. Triglycerides and blood pressure appear to be factors that are associated with TL in these patients. Further studies are required to confirm these results.

scholarly journals Recurrent benign adrenal pheochromocytomas associated with hemihypertrophy

2014 ◽  
Vol 2014 ◽  
Author(s):  
Maria Pikilidou ◽  
Maria Yavropoulou ◽  
Marios Katsounaros
Keyword(s):  
Blood Pressure ◽  
Early Diagnosis ◽  
Young Age ◽  
Diagnosis And Treatment ◽  
List Type ◽  
Genetic Syndrome ◽  
Recurrent Tumors ◽  
High Propensity ◽  
Learning Points

Summary We report a case of a female with hemihypertrophy, who developed five recurrences of pheochromocytomas until the age of 35. Timely follow-up of the patient's blood pressure assisted in early diagnosis and treatment of recurrent tumors. Learning points Recurrent benign pheochromocytomas should raise suspicion of a genetic syndrome. A pheochromocytoma at a young age has a high propensity to recur and strict follow-up is mandatory.

Getting to know a new protocol in hypertension care

2020 ◽  
Vol 16 (1) ◽  
Author(s):  
Mona Lundin
Keyword(s):  
Blood Pressure ◽  
Digital Technology ◽  
Interaction Analysis ◽  
Medical Discourse ◽  
Graphical Form ◽  
Graph Data ◽  
Average Blood Pressure ◽  
Infrastructure Design

This study explores the use of a new protocol in hypertension care, in which continuous patient-generated data reported through digital technology are presented in graphical form and discussed in follow-up consultations with nurses. This protocol is part of an infrastructure design project in which patients and medical professionals are co-designers. The approach used for the study was interaction analysis, which rendered possible detailed in situ examination of local variations in how nurses relate to the protocol. The findings show three distinct engagements: (1) teasing out an average blood pressure, (2) working around the protocol and graph data and (3) delivering an analysis. It was discovered that the graphical representations structured the consultations to a great extent, and that nurses mostly referred to graphs that showed blood pressure values, which is a measurement central to the medical discourse of hypertension. However, it was also found that analysis of the data alone was not sufficient to engage patients: nurses' invisible and inclusion work through eliciting patients' narratives played an important role here. A conclusion of the study is that nurses and patients both need to be more thoroughly introduced to using protocols based on graphs for more productive consultations to be established. 

Scoring System for the Diagnosis of COVID-19

2020 ◽  
Vol 13 (1) ◽  
pp. 413-414 ◽  
Author(s):  
Mohamed Farouk Allam
Keyword(s):  
Scoring System ◽  
Clinical Symptoms ◽  
False Negative ◽  
Nasopharyngeal Swab ◽  
Rt Pcr ◽  
Pcr Assay ◽  
Negative Results ◽  
False Negative Results ◽  
Symptoms And Signs

Due to the international spread of COVID-19, the difficulty of collecting nasopharyngeal swab specimen from all suspected patients, the costs of RT-PCR and CT, and the false negative results of RT-PCR assay in 41% of COVID-19 patients, a scoring system is needed to classify the suspected patients in order to determine the need for follow-up, home isolation, quarantine or the conduction of further investigations. A scoring system is proposed as a diagnostic tool for suspected patients. It includes Epidemiological Evidence of Exposure, Clinical Symptoms and Signs, and Investigations (if available). This scoring system is simple, could be calculated in a few minutes, and incorporates the main possible data/findings of any patient.

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