scholarly journals Histological scores and tumor size on stage II in adrenocortical carcinomas

Rare Tumors ◽  
2021 ◽  
Vol 13 ◽  
pp. 203636132110264
Author(s):  
Rui Caetano Oliveira ◽  
Maria João Martins ◽  
Carolina Moreno ◽  
Rui Almeida ◽  
João Carvalho ◽  
...  
Keyword(s):  
Tumor Size ◽  
Correct Diagnosis ◽  
Stage Ii ◽  
Adrenal Tumors ◽  
Median Size ◽  
Adrenocortical Adenomas ◽  
History Of ◽  
Aggressive Tumors ◽  
Adrenocortical Carcinomas ◽  
Worse Prognosis

Adrenocortical carcinomas (ACC) are aggressive tumors with a poor prognosis. Histological scores are advised for the diagnosis, however, there are borderline cases that may be misjudged as adrenocortical adenomas (ACA). The three main scores used are: Weiss Modified System (WMS), Reticulin Algorithm (RA), and Helsinki Score (HS). We intend to compare the accuracy of the three scores in ACC diagnosis and to identify predictive factors of overall survival (OS). Retrospective study (2004–2016) at Centro Hospitalar e Universitário de Coimbra of the adrenal tumors, classified as ACC or ACA, with a history of posterior tumor relapse/metastases, without lesions in the contralateral adrenal gland: 13F and 6M, with a median age of 51 ± 12.41 years. Nodules’ median size was 9.20 ± 6.2 cm. Patients had a median OS of 52 ± 18.6 months, with 57.9% and 46.3%, at 3 and 5 years. Seven patients had local recurrence and nine had metastases. Thirteen cases were in stage II. The WMS and the HS allowed a diagnosis of ACC in 15 cases and the RA defined ACC in 17 cases. All cases had, at least, focal disruption of the reticulin framework. More than 5 mitosis/50 HPF was associated with worse OS: 49.67 ± 21.43 versus 108.86 ± 14.02 months ( p = 0.026). In patients with stage II, tumor size ⩾10 cm was associated with worse OS: 19.25 ± 7.15 versus 96.11 ± 16.7 months ( p = 0.007), confirmed by multivariate analysis ( p = 0.031). The correct diagnosis of ACC is a pathologist responsibility. The RA seems the most accurate. Any loss of the reticulin framework should raise awareness for malignancy. In patients on stage II, a size ⩾10 cm is a predictor of worse prognosis.

scholarly journals The activating TERT promoter mutation C228T is recurrent in subsets of adrenal tumors

2014 ◽  
Vol 21 (3) ◽  
pp. 427-434 ◽  
Author(s):  
Tiantian Liu ◽  
Taylor C Brown ◽  
C Christofer Juhlin ◽  
Adam Andreasson ◽  
Na Wang ◽  
...  
Keyword(s):  
Reverse Transcriptase ◽  
Transcriptional Activation ◽  
Recurrent Event ◽  
Adrenal Tumors ◽  
Promoter Mutation ◽  
Adrenocortical Adenomas ◽  
Cell Immortalization ◽  
Underlying Mechanisms ◽  
Adrenocortical Carcinomas ◽  
Promoter Mutations

The telomerase reverse transcriptase gene (TERT) encodes the reverse transcriptase component of the telomerase complex, which is essential for telomere stabilization and cell immortalization. Recent studies have demonstrated a transcriptional activation role for theTERTpromoter mutations C228T and C250T in many human cancers, as well as a role in aggressive disease with potential clinical applications. Although telomerase activation is known in adrenal tumors, the underlying mechanisms are not established. We assessed C228T and C250TTERTmutations by direct Sanger sequencing in tumors of the adrenal gland, and further evaluated potential associations with clinical parameters and telomerase activation. A total of 199 tumors were evaluated, including 34 adrenocortical carcinomas (ACC), 47 adrenocortical adenomas (ACA), 105 pheochromocytomas (PCC; ten malignant and 95 benign), and 13 abdominal paragangliomas (PGL; nine malignant and four benign).TERTexpression levels were determined by quantitative RT-PCR. The C228T mutation was detected in 4/34 ACCs (12%), but not in any ACA (P=0.028). C228T was also observed in one benign PCC and in one metastatic PGL. The C250T mutation was not observed in any case. In the ACC and PGL groups,TERTmutation-positive cases exhibitedTERTexpression, indicating telomerase activation; however, since expression was also revealed inTERTWT cases, this could denote additional mechanisms ofTERTactivation. To conclude, theTERTpromoter mutation C228T is a recurrent event associated withTERTexpression in ACCs, but rarely occurs in PGL and PCC. The involvement of theTERTgene in ACC represents a novel mutated gene in this entity.

scholarly journals Laparoscopic Adrenalectomy for Potentially Malignant Adrenal Tumors Greater than 5 Centimeters

2006 ◽  
Vol 91 (8) ◽  
pp. 3080-3083 ◽  
Author(s):  
Chun-Hou Liao ◽  
Shih-Chieh Chueh ◽  
Ming-Kuen Lai ◽  
Po-Jen Hsiao ◽  
Jun Chen
Keyword(s):  
Tumor Size ◽  
Laparoscopic Adrenalectomy ◽  
Malignant Tumors ◽  
Adrenal Tumors ◽  
Benign Tumors ◽  
Conversion To Open Surgery ◽  
Open Conversion ◽  
Potentially Malignant ◽  
Adrenocortical Carcinomas

Abstract Purpose: Laparoscopic adrenalectomy (LA) is controversial for large, potentially malignant tumors. We report a series of LA or hand-assisted LA for large (>5 cm) adrenal tumors. Patients and Methods: Among 210 LAs performed in 6 yr, 39 patients had potentially malignant tumors greater than 5 cm in diameter. Their perioperative and follow-up data were retrospectively analyzed. Results: All 39 patients had successful LAs without perioperative mortality, conversion to open surgery, or capsular disruption during dissection. The mean tumor size was 6.2 cm (range, 5–12 cm), operative time 207 min (115–315 min), and blood loss 75 ml (minimal–1400 ml). Complications included one intraoperative diaphragmatic perforation, three mild wound infections, and one pneumonia. Preoperatively there were 27 nonfunctioning tumors, seven pheochromocytomas, three cortisol-secreting tumors, and two virilizing tumors. Final pathology revealed eight malignant (four adrenocortical carcinomas and four metastatic carcinomas) and 31 benign tumors (14 cortical adenomas, eight pheochromocytomas, six myelolipomas, and three ganglioneuromas). Median follow-up was 39 months. Four patients (two adrenocortical carcinomas, one metastatic hepatoma, and one lymphoma) died 24, 10, 9, and 3 months after surgery, respectively. A hand-assisted device was used in 10 patients. Only the tumor size was larger and length of postoperative hospital stay longer for those in the hand-assisted group. Conclusions: LA is a reasonable option for selected large adrenal tumors when complete resection is technically feasible and there is no evidence of local invasion. Hand-assisted LA is a good alternative to open conversion if a difficult dissection is encountered intraoperatively.

Plasma dehydroepiandrosterone sulfate levels in patients with hyperfunctioning and non-hyperfunctioning adrenal tumors before and after adrenal surgery

1997 ◽  
Vol 136 (3) ◽  
pp. 290-295 ◽  
Author(s):  
Miklós Tóth ◽  
Károly Rácz ◽  
Ibolya Varga ◽  
Vilmos Adleff ◽  
Csilla Jakab ◽  
...  
Keyword(s):  
Normal Subjects ◽  
Dehydroepiandrosterone Sulfate ◽  
High Plasma ◽  
Adrenal Tumors ◽  
Adrenal Surgery ◽  
Adrenocortical Tumors ◽  
Adrenocortical Adenomas ◽  
Before And After ◽  
Adrenocortical Carcinomas ◽  
Glucocorticoid Production

Abstract To investigate the clinical significance of plasma dehydroepiandrosterone sulfate (DHEAS) measurements, 175 patients with histologically confirmed adrenal tumors, 10 cortisol-producing adenomas, 59 aldosterone-producing adenomas, 56 non-hyperfunctioning adenomas, 13 adrenocortical carcinomas, 13 adrenal cysts, and 24 adrenomedullary tumors were studied. Plasma DHEAS levels were expressed as percentage of the mean of sex- and age-matched groups of healthy, normal subjects (DHEAS %). We found that before adrenal surgery, DHEAS % values were significantly reduced in patients with cortisol-producing (mean, 15·2% of control; 95% confidence interval (CI), 9·4–24·7%), non-hyperfunctioning (28·4%; 22·4–36·0% as well as aldosterone-producing adrenocortical adenomas (55·4%; 47·1–65·1%) compared with controls, while values were normal in patients with adrenal cysts and in those with adrenomedullary tumors. Plasma DHEAS % values exhibited a great variability in adrenocortical carcinomas (mean, 84·0%; 95% CI, 33·2–212·5%). Death from adrenocortical carcinoma was more frequent in patients with high plasma DHEAS % values compared with those with low DHEAS %. During long-term postoperative monitoring, we found that plasma DHEAS levels of patients with aldosterone-producing and non-hyperfunctioning adenomas returned to normal in the second and fourth postoperative year respectively. In patients with cortisol-producing adenomas, plasma DHEAS remained suppressed for as long as 8 years after the operation. These findings show that except in adrenocortical carcinomas and cysts, plasma DHEAS levels are significantly decreased in all groups of adrenocortical tumors, including non-hyperfunctioning and aldosteroneproducing tumors. The extent of this decrease and the postoperative persistence of suppressed plasma DHEAS levels may be related to the glucocorticoid production of adrenocortical tumors. European Journal of Endocrinology 136 290–295

scholarly journals Prevalence and natural history of adrenal incidentalomas

2003 ◽  
pp. 273-285 ◽  
Author(s):  
L Barzon ◽  
N Sonino ◽  
F Fallo ◽  
G Palu ◽  
M Boscaro
Keyword(s):  
Natural History ◽  
Common Finding ◽  
Adrenal Incidentalomas ◽  
Adrenocortical Adenomas ◽  
Risk Of Malignancy ◽  
History Of ◽  
Adrenal Masses ◽  
Adrenocortical Carcinomas ◽  
Management Recommendations

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.

Cardiac transthyretin wild-type amyloidosis (ATTRwt): a prospective study of 400 patients followed at the Italian referral center

2020 ◽  
Vol 41 (Supplement_2) ◽  
Author(s):  
P Milani ◽  
L Obici ◽  
R Mussinelli ◽  
M Basset ◽  
G Manfrinato ◽  
...  
Keyword(s):  
Natural History ◽  
Median Survival ◽  
Troponin I ◽  
Stage I ◽  
Stage Ii ◽  
Stage Iii ◽  
Wild Type ◽  
Staging Systems ◽  
Significant Difference ◽  
History Of

Abstract Background Cardiac wild type transthyretin (ATTRwt) amyloidosis, formerly known as senile systemic amyloidosis, is an increasingly recognized, progressive, and fatal cardiomyopathy. Two biomarkers staging systems were proposed based on NT-proBNP (in both cases) and troponin or estimated glomerular filtration rate, that are able to predict survival in this population. The availability of novel effective treatments requires large studies to describe the natural history of the disease in different populations. Objective To describe the natural history of the disease in a large, prospective, national series. Methods Starting in 2007, we protocolized data collection in all the patients diagnosed at our center (n=400 up to 7/2019). Results The referrals to our center increased over time: 5 cases (1%) between 2007–2009, 33 (9%) in 2010–2012, 90 (22%) in 2013–2015 and 272 (68%) in 2016–2019. Median age was 76 years [interquartile range (IQR): 71–80 years] and 372 patients (93%) were males. One hundred and seventy-three (43%) had atrial fibrillation, 63 (15%) had a history of ischemic cardiomyopathy and 64 (15%) underwent pacemaker or ICD implantation. NYHA class was I in 58 subjects (16%), II in 225 (63%) and III in 74 (21%). Median NT-proBNP was 3064 ng/L (IQR: 1817–5579 ng/L), troponin I 0.096 ng/mL (IQR: 0.063–0.158 ng/mL), eGFR 62 mL/min (IQR: 50–78 mL/min). Median IVS was 17 mm (IQR: 15–19 mm), PW 16 mm (IQR: 14–18 mm) and EF 53% (IQR: 45–57%). One-hundred and forty-eight subjects (37%) had a concomitant monoclonal component in serum and/or urine and/or an abnormal free light chain ratio. In these patients, the diagnosis was confirmed by immunoelectron microscopy or mass spectrometry. In 252 (63%) the diagnosis was based on bone scintigraphy. DNA analysis for amyloidogenic mutations in transthyretin and apolipoprotein A-I genes was negative in all subjects. The median survival of the whole cohort was 59 months. The Mayo Clinic staging based on NT-proBNP (cutoff: 3000 ng/L) and troponin I (cutoff: 0.1 ng/mL) discriminated 3 different groups [stage I: 131 (35%), stage II: 123 (32%) and stage III: 127 (33%)] with different survival between stage I and II (median 86 vs. 81 months, P=0.04) and between stage II and III (median 81 vs. 62 months, P<0.001). The UK staging system (NT-proBNP 3000 ng/L and eGFR 45 mL/min), discriminated three groups [stage I: 170 (45%), stage II: 165 (43%) and stage III: 45 (12%)] with a significant difference in survival: between stage I and stage II (86 vs. 52 months, P<0.001) and between stage II and stage III (median survival 52 vs. 33 months, P=0.045). Conclusions This is one of the largest series of patients with cardiac ATTRwt reported so far. Referrals and diagnoses increased exponentially in recent years, One-third of patients has a concomitant monoclonal gammopathy and needed tissue typing. Both the current staging systems offered good discrimination of staging and were validated in our independent cohort. Funding Acknowledgement Type of funding source: None

scholarly journals Factors Associated with Clinician Adherence to USPSTF Diabetes Screening Recommendations

2021 ◽  
Vol 12 ◽  
pp. 215013272110165
Author(s):  
Elaine Seaton Banerjee ◽  
Kyle Shaak ◽  
Nicole Burgess ◽  
Melanie Johnson ◽  
Beth Careyva
Keyword(s):  
Gestational Diabetes ◽  
Adult Population ◽  
Correct Diagnosis ◽  
Retrospective Chart Review ◽  
Diabetes Screening ◽  
Factors Associated ◽  
The Us ◽  
History Of ◽  
Missed Diagnosis ◽  
Significant Health

Introduction/Objectives: Diabetes and prediabetes impact nearly half of the US adult population and are associated with significant health risks but may be underdiagnosed. Effective screening may improve diagnosis and give patients opportunity to manage their disease. The purpose of this study was to determine screening rates, identify characteristics predictive of screening, and evaluate correct diagnosis of diabetes and prediabetes. Methods: Retrospective chart review of 71 433 patients eligible for diabetes screening, defined by completing A1c test within the 3-year study period. Results: A total of 31.3% of eligible patients received diabetes screening. Factors associated with screening include older age, female sex, non-white race, Hispanic ethnicity, Medicare or Medicaid insurance, higher BMI, and having a medical comorbidity. History of prediabetes or gestational diabetes were the strongest predictors for diabetes screening, but history of gestational diabetes was under-documented. Of those screened, 10.4% had a result consistent with diabetes and 51.8% had a result consistent with prediabetes. However, 52.9% of these patients had a missed diagnosis. Conclusions: Findings of this study indicate the need for uniform coverage for diabetes screening for all insurances, increased documentation of gestational diabetes to improve screening for patients with this history, and improving accurate diagnosis after screening is completed.

scholarly journals Tuberculosis presenting as dacryoadenitis in the USA

2019 ◽  
Vol 12 (11) ◽  
pp. e231694 ◽  
Author(s):  
Harry Ross Powers ◽  
Mark Anthony Diaz ◽  
Julio C Mendez
Keyword(s):  
Correct Diagnosis ◽  
Developed Countries ◽  
Histopathological Analysis ◽  
Orbital Mass ◽  
Antituberculosis Therapy ◽  
The Usa ◽  
History Of ◽  
Patient’S History ◽  
High Degree ◽  
Filipino Woman

A 25-year-old Filipino woman living in the USA was evaluated for a 5-month history of left eye pain and a subsequent orbital mass. Histopathological analysis of the lacrimal mass showed a mixed inflammatory process with necrotising granulomas and positive cultures for Mycobacterium tuberculosis. She was treated with antituberculosis therapy, with resolution of symptoms. Tuberculosis dacryoadenitis is extremely rare in the USA and other developed countries. It requires a high degree of clinical suspicion with special attention to the patient’s history to make the correct diagnosis. It can be treated successfully with antituberculosis therapy.

scholarly journals Clinicopathological characteristics and prognostic factors of cervical adenocarcinoma

2021 ◽  
Vol 11 (1) ◽  
Author(s):  
Min Wang ◽  
Bo Yuan ◽  
Zhen-huan Zhou ◽  
Wei-wei Han
Keyword(s):  
Prognostic Factors ◽  
Tumor Size ◽  
Survival Rates ◽  
Tumor Grade ◽  
Figo Stage ◽  
Cervical Adenocarcinoma ◽  
Stage Ii ◽  
Prognostic Indicators ◽  
Rank Test ◽  
Cancer Specific Survival

AbstractWe aimed to assess the clinicopathological features and to determine the prognostic factors of cervical adenocarcinoma (AC). Relevant data were extracted from surveillance, epidemiology and end results database from 2004 to 2015. The log-rank test and Cox proportional hazard analysis were subsequently utilized to identify independent prognostic factors. A total of 3102 patients were identified. The enrolled patients were characterized by higher proportion of early FIGO stage (stage I: 65.9%; stage II: 14.1%), low pathological grade (grade I/II: 49.1%) and tumor size ≤ 4 cm (46.8%). The 5- and 10-year cancer-specific survival rates of these patients were 74.47% and 70.00%, respectively. Meanwhile, the 5- and 10-year overall survival (OS) rates were 71.52% and 65.17%, respectively. Multivariate analysis revealed that married status, surgery as well as chemotherapy were independent favorable prognostic indicators. Additionally, aged > 45, tumor grade III/IV, tumor size > 4 cm, advanced FIGO stage and pelvic lymph node metastasis (LNM) were unfavorable prognostic factors (all P < 0.01). Stratified analysis found that patients without surgery could significantly benefit from chemotherapy and radiotherapy. In addition, chemotherapy could significantly improve the survival in stage II–IV patients and radiotherapy could only improve the survival in stage III patients (all P < 0.01). Marital status, age, grade, tumor size, FIGO stage, surgery, pelvic LNM and chemotherapy were significantly associated with the prognosis of cervical AC.

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