Prevalence and natural history of adrenal incidentalomas

Clinically silent adrenal masses discovered by imaging studies performed for unrelated reasons, i.e. adrenal incidentalomas, have become a rather common finding in clinical practice. However, only limited studies of incidence, prevalence, and natural history of adrenal incidentalomas are available. A comprehensive review of the literature shows the prevalence of adrenal incidentalomas to be 2.3% at autopsy and 0.5-2% at abdominal computed tomography scan. Most lesions are adrenocortical adenomas at histology, whereas the prevalence of adrenocortical carcinomas is relatively low. The risk of malignancy over time for masses defined as benign at diagnosis is estimated at about 1/1000, even though 5-25% of masses increase in size during follow-up. Hyperfunction develops in about 1.7% of cases and the risk is higher in patients with lesions larger than 3 cm. Cortisol hypersecretion is the most likely disorder that may ensue, and it remains subclinical in about two-thirds of cases. The lack of controlled studies precludes making specific management recommendations. Large perspective controlled studies to define the epidemiology, natural history, and possible associated morbidity of adrenal incidentalomas and their impact on the quality of life of patients are needed.

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Malignancy Risk and Hormonal Activity of Adrenal Incidentalomas in a Large Cohort of Patients from a Single Tertiary Reference Center

International Journal of Environmental Research and Public Health ◽

10.3390/ijerph16101872 ◽

2019 ◽

Vol 16 (10) ◽

pp. 1872 ◽

Cited By ~ 3

Author(s):

Ewa Cyranska-Chyrek ◽

Ewelina Szczepanek-Parulska ◽

Michal Olejarz ◽

Marek Ruchala

Keyword(s):

Ct Scan ◽

Glucose Metabolism Disorders ◽

Hormonal Activity ◽

Adrenal Incidentalomas ◽

Adrenocortical Adenomas ◽

Asymptomatic Patients ◽

Risk Of Malignancy ◽

Autonomous Cortisol Secretion ◽

Adrenocortical Carcinomas ◽

Adrenal Ct

Background: A rise in adrenal incidentalomas (AIs) detection has been observed recently. Even though AIs are detected in asymptomatic patients, thorough assessment may reveal hormonal and metabolic abnormalities or malignant character. Methods: Medical records of 2005 patients (1301 women, 704 men) with 2498 tumors aged 61 ± 11.3 (18–93) years, who had been hospitalized due to AI diagnosis, were reviewed. Patients underwent clinical examination, adrenal CT and hormonal assessment. In patients subjected to adrenalectomy, histopathological character of AI was confirmed. Results: AIs most frequently occurred in patients in their 7th decade of life. Hypertension was present in 76.6%, glucose metabolism disorders in 41.3%, and hypercholesterolemia in 60.1% of patients. Lipid-rich adenomas (83.2%) and hormonally inactive tumors (83.1%) predominated. Autonomous cortisol secretion was present or suspected in 6.6%, pheochromocytoma in 4.7%, hyperandrogenism in 3.1%, and primary hyperaldosteronism in 2.4% of patients. The risk of malignancy increased in patients with tumors >6 cm was 37.7%. The logistic regression analysis revealed that the strongest predictor of hormonal activity of AIs was lipid-poor picture on CT scan (OR 7.072; CI 5.118–9.771), while the most important factor increasing the risk of malignancy was lipid-poor adenoma or non-adenoma on CT scan (OR 4.843; CI 1.697–13.819). Final histopathology was available for 214 tumors; 106 adrenocortical adenomas, 46 pheochromocytomas, and 18 adrenocortical carcinomas were diagnosed. Conclusion: Most AIs are hormonally inactive adenomas. The most frequent hormonal manifestation of AI is subclinical hypercortisolemia. Presence of AI is often accompanied by features of metabolic syndrome. The tumor density on CT scan picture may be predictive of both hormonal activity and the risk of malignancy. Tumors of all sizes may exhibit hormonal activity, while the risk of malignancy significantly increases with the size above 6 cm.

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Adrenal incidentaloma: evaluation and management

Journal of Clinical Pathology ◽

10.1136/jcp.2006.044313 ◽

2008 ◽

Vol 61 (11) ◽

pp. 1168-1173 ◽

Cited By ~ 39

Author(s):

P K Singh ◽

H N Buch

Keyword(s):

Surgical Intervention ◽

Adrenal Incidentaloma ◽

Adrenal Incidentalomas ◽

Imaging Studies ◽

Imaging Characteristics ◽

Risk Of Malignancy ◽

Life Threatening ◽

History Of ◽

Adrenal Masses

Adrenal incidentalomas are adrenal masses discovered incidental to imaging studies performed for reasons unrelated to adrenal pathology. Although most adrenal incidentalomas are non-functioning benign adenomas, their increasing prevalence presents diagnostic and therapeutic challenges. The assessment of adrenal incidentalomas is aimed at deciding whether or not the tumour should be surgically removed. Adrenalectomy is indicated for phaeochromocytoma, other symptomatic hormone-secreting tumours and those with a high risk of malignancy. Biochemical screening for tumour hypersecretion is mandatory in all adrenal incidentalomas, since hormone secreting tumours may be clinically silent. The diagnosis of phaeochromocytoma is of paramount importance because of its life-threatening complications. Non-functioning adrenal incidentalomas need assessment for risk of malignancy, and this is based on the size of the tumour and its imaging characteristics. An observational policy with periodic radiological and biochemical reassessment is pursued in patients with non-functioning incidentalomas with low malignancy risk. The duration and frequency of reassessment remains unclear, as the natural history of adrenal incidentalomas has yet to be clearly defined, and there is a lack of controlled studies comparing surgical intervention with observation. However, the possibility of acquiring autonomous hypersecretion or conversion to malignancy in an incidentaloma diagnosed to be a benign non-functioning lesion is very low, and most patients may be safely discharged after an initial follow-up period of 2 years.

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Natural history of adrenal incidentalomas with and without mild autonomous cortisol excess; a systematic review and meta-analysis

Endocrine Abstracts ◽

10.1530/endoabs.59.p015 ◽

2018 ◽

Author(s):

Yasir Elhassan ◽

Fares Alahdab ◽

Alessandro Prete ◽

Danae Delivanis ◽

Aakanksha Khanna ◽

...

Keyword(s):

Systematic Review ◽

Natural History ◽

Meta Analysis ◽

Adrenal Incidentalomas ◽

History Of

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Improving the understanding of how patients with non-dystrophic myotonia are selected for myotonia treatment with mexiletine (NaMuscla): outcomes of treatment impact using a European Delphi panel

BMC Neurology ◽

10.1186/s12883-021-02491-3 ◽

2021 ◽

Vol 21 (1) ◽

Author(s):

Ann-Marie Chapman ◽

Marieke Schurer ◽

Laure Weijers ◽

Amer Omar ◽

Hiba Lee ◽

...

Keyword(s):

Quality Of Life ◽

Natural History ◽

Negative Impact ◽

Phase Iii ◽

Future Research ◽

Delphi Panel ◽

Economic Modelling ◽

Patient Access ◽

History Of

Abstract Background Non-dystrophic myotonias (NDMs) comprise muscle chloride and sodium channelopathies due to genetic defects of the CLCN1- and SCN4A-channels. No licensed antimyotonic treatment has been available until approval of mexiletine (NaMuscla®) for adult patients by the EMA in December 2018. This Delphi panel aimed to understand how outcomes of the pivotal phase III Mexiletine study (MYOMEX) translate to real world practice and investigate health resource use, quality of life and the natural history of NDM to support economic modelling and facilitate patient access. Methods Nine clinical experts in treating NDM took part in a two-round Delphi panel. Their knowledge of NDM and previous use of mexiletine as an off-label treatment prior to NaMuscla’s approval ensured they could provide both qualitative context and quantitative estimates to support economic modelling comparing mexiletine (NaMuscla) to best supportive care. Consensus in four key areas was sought: healthcare resource utilization (HRU), treatment with mexiletine (NaMuscla), patient quality of life (QoL), and the natural history of disease. Concept questions were also asked, considering perceptions on the feasibility of mapping the validated Individualized Neuromuscular Quality of Life (INQoL) instrument to the generic EQ-5D™, and the potential impact on caregiver QoL. Results Consensus was achieved for key questions including the average long-term dosage of mexiletine (NaMuscla) in practice, the criteria for eligibility of myotonia treatment, the clinical importance of QoL outcomes in MYOMEX, the higher proportion of patients with increased QoL, and the reduction in the need for mental health resources for patients receiving mexiletine (NaMuscla). While consensus was not achieved for other questions, the results demonstrated that most experts felt mexiletine (NaMuscla) reduced the need for HRU and was expected to improve QoL. The QoL mapping exercise suggested that it is feasible to map domains of INQoL to EQ-5D. Points of interest for future research were identified, including that mexiletine (NaMuscla) may slow the annual decrease in QoL of patients over their lifetime, and a significant negative impact on QoL for some caregivers. Conclusions This project successfully provided data from an informed group of clinical experts, complementing the currently available clinical trial data for mexiletine (NaMuscla) to support patient access decisions.

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Natural history of mixed chimerism after bone marrow transplantation with CD6-depleted allogeneic marrow: a stable equilibrium

Blood ◽

10.1182/blood.v75.1.296.bloodjournal751296 ◽

1990 ◽

Vol 75 (1) ◽

pp. 296-304 ◽

Cited By ~ 1

Author(s):

DC Roy ◽

R Tantravahi ◽

C Murray ◽

K Dear ◽

B Gorgone ◽

...

Keyword(s):

Bone Marrow ◽

Bone Marrow Transplantation ◽

Natural History ◽

Marrow Transplantation ◽

Cytogenetic Analysis ◽

Common Finding ◽

Mixed Chimerism ◽

Allogeneic Bone ◽

Donor Cells ◽

History Of

Mixed hematopoietic chimerism (MC) is a common finding after allogeneic bone marrow transplantation (BMT), but the natural history of this phenomenon remains unclear. To understand the evolution and the implications of this finding, we performed a prospective analysis of the development of mixed chimerism in 43 patients with hematologic malignancies who received bone marrow (BM) from human leukocyte antigen (HLA)-identical sibling donors. T-cell depletion in vitro with anti-T12 (CD6) monoclonal antibody and rabbit complement was used as the only method of graft-versus-host disease (GVHD) prophylaxis. Overall, MC was identified in peripheral blood (PB) and BM in 22 of 43 (51%) patients evaluated. MC was found by restriction fragment length polymorphism (RFLP) analysis in 21 of 40 (53%) patients, by cytogenetic analysis in 6 of 29 (21%) patients, and by red blood cell phenotyping in 4 of 9 (44%) patients. RFLP studies were performed at 0.5, 1, 3, 6, 9, and 12 months post-BMT and then every 6 months, and showed a high probability of developing MC in the first 6 months after BMT followed by stabilization after 12 months. Cytogenetic analysis was less sensitive in detecting MC. Once MC was detected after BMT, the percentage of recipient cells increased very slowly over more than 3 years of follow- up, and no patient reverted to complete donor hematopoiesis (CDH). Thus, recipient and donor cells remained in a relative state of equilibrium for prolonged periods that seemed to favor recipient cells over donor cells. Patient's disease, remission status, or intensity of the transplant preparative regimen did not influence the subsequent development of mixed chimerism. Early immunologic reconstitution was the only factor that correlated with the subsequent chimeric status of the patients. The percentage and absolute number of T3 (CD3) and T4 (CD4) positive cells at day 14 after BMT were significantly higher in the patients who maintained CDH but NK cell reconstitution was similar in both groups, suggesting that early reconstitution with T cells may play a role in preventing recovery of recipient cells after BMT. GVHD was also associated with maintenance of CDH, but the probability of relapse, survival, and disease-free survival was identical in patients with MC and CDH.

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