scholarly journals Clinicopathological Features of Pediatric Functional Adrenocortical Carcinoma Diagnosed by Weiss Criteria; An Analysis of Four Cases

2001 ◽  
Vol 10 (2) ◽  
pp. 141-146 ◽  
Author(s):  
Yoko Misu ◽  
Shi-Xu Jiang ◽  
Yukifumi Yokota ◽  
Masahiko Shibata ◽  
Osamu Shinohara ◽  
...  
Keyword(s):  
Adrenocortical Carcinoma ◽  
Clinicopathological Features ◽  
Weiss Criteria

scholarly journals Nonfunctioning adrenocortical carcinoma: a study of 12 cases

1970 ◽  
Vol 2 (3) ◽  
pp. 193-197
Author(s):  
S Ghanghoria ◽  
P Raje ◽  
R Jain ◽  
A Ghanghoria
Keyword(s):  
Adrenocortical Carcinoma ◽  
Epithelial Membrane Antigen ◽  
Clinicopathological Features ◽  
Adrenal Cortical Carcinoma ◽  
High Grade ◽  
Histological Features ◽  
Histological Criteria ◽  
Adrenal Cortical Tumor ◽  
High Grade Carcinoma ◽  
Weiss Criteria

Background: Adrenal cortical carcinoma is a rare malignant tumor. Several systems were proposed for diagnosis and evaluation of adrenocortical tumors.Weiss system is most commonly used method for assessment of malignancy, but only few studies has applied this criteria, for assesement of prognosis. The aim of this study was to analyzethe histological features & immunostaining status of adrenocortical carcinoma diagnosed according to Weiss criteria and correlate the data with patient clinical finding and outcomes. Materials and Methods: In this study clinicopathological features of 12 cases were reviewed and diagnosed as adrenocortical carcinoma on the basis of Weiss criteria and immunostaining. Grading was done according to the nine histopathological criteria of Weiss and is applied for assessment of prognosis. Each tumor was labeled malignant, when it scored ≥4 point of this histological criteria, tumors with ≤3 points were considered as adenoma and not included in the series. Immunostains applied were, Vimentin, Cytokeratin, synaptophysin, chromogranin, Vinyl mandilic acid, epithelial membrane antigen and Melan A. Results: All cases studied, presented at least four of the Weiss criteria and thus diagnosed as adrenocortical carcinoma. Patients with 7 and more score of Weiss criteria is considered as high grade carcinoma. Immunostains, have role in the diagnosis of adrenal cortical tumor, but does not have any significant clinical outcome.Conclusion: Weiss criteria was found to be a good prognostic factor in adrenal cortical carcinoma. DOI: http://dx.doi.org/10.3126/jpn.v2i3.6020 JPN 2012; 2(3): 193-197

scholarly journals SUN-180 A Case of Deoxycorticosterone-Producing Malignant Adrenocortical Tumor

2020 ◽  
Vol 4 (Supplement_1) ◽  
Author(s):  
Natasha Scaranello Cartolano ◽  
Vânia Balderrama Brondani ◽  
Amanda Meneses Ferreira Lacombe ◽  
Helaine Charchar ◽  
Bruna Pessoa ◽  
...  
Keyword(s):  
Weight Loss ◽  
Arterial Hypertension ◽  
Ct Scan ◽  
Adrenocortical Carcinoma ◽  
Adrenal Mass ◽  
Laboratory Data ◽  
Pulmonary Nodules ◽  
Adrenocortical Tumor ◽  
Abdominal Ct ◽  
Weiss Criteria

Abstract Background: Hypermineralocorticism (hypertension, hypokalemia, and low plasma renin activity) due to deoxycorticosterone (DOC) excess associated with adrenocortical carcinoma is extremely rare. DOC-producing tumors cause primary aldosteronism-like symptoms presenting low plasma aldosterone with very high DOC levels, and due to weak hormonal DOC activity, its diagnostic is done lately. Generally, malignant cases are progressive with a dismal prognosis. Clinical case: A 61-year-old woman was admitted to our hospital presenting lumbar pain and weight loss of 8 kg, in 2018. Previously, arterial hypertension was diagnosed in 2015, showing a satisfactory control with two classes of antihypertensive drugs. Physical exam: The patient presented no features of Cushing syndrome, but a palpable abdominal mass was noticed in the right flank. Blood pressure was 160x100 mmHg, with sustained high levels, despite regular treatment. Laboratory data: a hypokalemia (K 2.4 mEq/L, nr 3.5 -5.0 mEq/L) and hypernatremia (Na 146 mEq/L, nr 135 to 145 mEq/L), with metabolic alkalosis (venous pH 7.46 and serum bicarbonate 32 mmol/L, nr 23-27 mmol/L) was confirmed. Hormonal tests excluded hypercortisolism and pheocromocytoma. Serum aldosterone and renin were suppressed. Mineralocorticoid precursors dosage was extremely high, DOC (654 ng/dL, nr < 25 ng/dL) and progesterone (5.0 ng/mL, nr <0.89 ng/mL), as well 11-deoxycortisol (7.2 ng/mL, nr <0.5 ng/mL). Radiological imaging: abdominal CT showed a heterogeneous hypervascular adrenal mass (13.0x13.0x21.0 cm) exhibiting central necrosis, suggesting malignancy. FDG-PET/CT scan showed a hypermetabolic adrenal mass (SUVmax=13.8). Also, two metabolically active pulmonary nodules (SUVmax=3.7) measuring 0.7 and 0.4 cm were detected. The patient underwent right adrenalectomy, and the tumor was removed (24x13x13 cm). According to Weiss criteria (8/9) and modified Weiss criteria (5/7), the tumor was considered an adrenocortical carcinoma. Immunohistochemistry revealed a low Ki-67 index (10%). After the surgical procedure, all adrenal steroid levels normalized, and mitotane was prescribed as adjuvant therapy. Although the pulmonary nodules were stable at the four-month follow-up, the abdominal CT-scan revealed a heterogeneous nodule (3.7cm) in the left adrenal gland, which was suspicious of metastasis. Conclusion: DOC-producing adrenocortical tumors are heterogeneous regarding tumor size, clinical behavior, hormonal and metabolites secretion, and disease-free and overall survival; however, it is common hypokalemia, hypertension, and other symptoms as abdominal pain, due to tumor growth, and weight loss. The association of arterial hypertension with hypokalemia and elevated 11-deoxycortisol, with normal aldosterone and renin, lead to the need for mineralocorticoid precursors evaluation in patients with adrenocortical tumor.

scholarly journals Clinicopathological features, biochemical and molecular markers in 5 patients with adrenocortical carcinoma

2011 ◽  
Vol 58 (7) ◽  
pp. 527-534 ◽  
Author(s):  
Ryuji Kouyama ◽  
Kiichiro Hiraishi ◽  
Toru Sugiyama ◽  
Hajime Izumiyama ◽  
Takanobu Yoshimoto ◽  
...  
Keyword(s):  
Molecular Markers ◽  
Adrenocortical Carcinoma ◽  
Clinicopathological Features

scholarly journals Clinicopathological Features, Treatment Outcomes, and Prognostic Factors in Adrenocortical Carcinoma: A Single-Center Experience

2021 ◽  
Vol 7 (3) ◽  
pp. 133-138
Author(s):  
Nazım Can DEMİRCAN ◽  
Tuğba AKIN TELLİ ◽  
Tuğba BAŞOĞLU ◽  
Rukiye ARIKAN ◽  
Alper YAŞAR ◽  
...  
Keyword(s):  
Prognostic Factors ◽  
Treatment Outcomes ◽  
Adrenocortical Carcinoma ◽  
Clinicopathological Features ◽  
Single Center ◽  
Single Center Experience

scholarly journals Clinicopathological features and outcomes of adrenocortical carcinoma: A single institution experience

2019 ◽  
Vol 35 (3) ◽  
pp. 213
Author(s):  
FrancisVadakkumparambil James ◽  
LekhaMadhavan Nair ◽  
KM Jagathnath Krishna ◽  
Aswin Kumar ◽  
Susan Mathews ◽  
...  
Keyword(s):  
Adrenocortical Carcinoma ◽  
Clinicopathological Features ◽  
Single Institution

scholarly journals Adrenocortical Carcinoma in Children: A Clinicopathological Analysis of 41 Patients at the Mayo Clinic from 1950 to 2017

2018 ◽  
Vol 90 (1) ◽  
pp. 8-18 ◽  
Author(s):  
Nidhi Gupta ◽  
Michael Rivera ◽  
Paul Novotny ◽  
Vilmarie Rodriguez ◽  
Irina Bancos ◽  
...  
Keyword(s):  
Adrenocortical Carcinoma ◽  
Mayo Clinic ◽  
Age At Onset ◽  
Survival Rates ◽  
Adrenocortical Adenoma ◽  
Limited Evidence ◽  
Clinicopathological Analysis ◽  
Total Adrenalectomy ◽  
Sites Of Metastases ◽  
Weiss Criteria

Background/Aims: Adrenocortical carcinoma (ACC) is an aggressive childhood cancer. Limited evidence exists on a definite histopathological criterion to differentiate ACC from adrenocortical adenoma. The aim of this study was to investigate the clinicopathological data of children with ACC, identify prognostic factors, and validate a histopathological criterion to differentiate ACC from adrenocortical adenoma. Methods: This retrospective cohort included 41 children, followed at the Mayo Clinic from 1950 to 2017 (onset of symptoms ≤21 years). Outcomes of interest were: alive with no evidence of disease, alive with evidence of disease, and dead of disease. Results: Median age at onset of symptoms was 15.7 years (n = 41; range, 0.2–21 years). Female:male ratio was 3.6: 1. Mixed symptomatology (> 1 hormone abnormality) was the most common presentation (54%, n = 22). Sixty-six percent of patients (n = 27 out of 41) underwent total adrenalectomy. Metastatic disease was more common in children aged > 12 years (p = 0.002 compared to < 4 years). The most common sites of metastases were the liver and lungs. Overall 2-year and 5-year survival rates were 61% (95% CI 45–77) and 46% (95% CI 30–62), respectively. Metastasis at the time of diagnosis was independently associated with poor prognosis (risk ratio 13.7%; 95% CI 3.9–87.7). Weiss criteria (29%) and modified Weiss criteria (33%) were less accurate in younger patients (< 12 years), compared to the Wieneke index (100%). Conclusion: The presence of metastases was an independent prognostic factor. The Wieneke index was the most accurate in predicting clinical outcomes in younger children.

scholarly journals Establishment and Validation of a Gene Signature-Based Prognostic Model to Improve Survival Prediction in Adrenocortical Carcinoma Patients

2021 ◽  
Vol 2021 ◽  
pp. 1-12
Author(s):  
Xiaoqin Ge ◽  
Zhenzhen Liu ◽  
Xuehua Jiao ◽  
Xueyan Yin ◽  
Xiujie Wang ◽  
...  
Keyword(s):  
Adjuvant Therapy ◽  
Decision Tree ◽  
Risk Stratification ◽  
Risk Prediction ◽  
Adrenocortical Carcinoma ◽  
Gene Signature ◽  
Clinicopathological Features ◽  
Survival Prediction ◽  
Current Guideline ◽  
Improve Risk Stratification

Background. The current guideline for the management of adrenocortical carcinoma (ACC) is insufficient for accurate risk prediction to guide adjuvant therapy. Given frequent and severe therapeutic side effects, a better estimate of survival is warranted for risk-specific assignment to adjuvant treatment. We attempted to construct an integrated model based on a prognostic gene signature and clinicopathological features to improve risk stratification and survival prediction in ACC. Methods. Using a series of bioinformatic and statistical approaches, a gene-expression signature was established and validated in two independent cohorts. By combining the signature with clinicopathological features, a decision tree was generated to improve risk stratification, and a nomogram was constructed to personalize risk prediction. Time-dependent receiver operating characteristic (tROC) and calibration analysis were performed to evaluate the predictive power and accuracy. Results. A three-gene signature could discriminate high-risk patients well in both training and validation cohorts. Multivariate regression analysis demonstrated the signature to be an independent predictor of overall survival. The decision tree could identify risk subgroups powerfully, and the nomogram showed high accuracy of survival prediction. Particularly, expression of a gene hitherto unknown to be dysregulated in ACC, TIGD1, was shown to be prognostically relevant. Conclusion. We propose a novel gene signature to guide decision-making about adjuvant therapy in ACC. The score shows unprecedented survival prediction and hence constitutes a huge step towards personalized management. As a secondary important finding, we report the discovery and validation of a new oncogene, TIGD1, which was consistently overexpressed in ACC. TIGD1 might shed further light on the biology of ACC and might give rise to targeted therapies that not only apply to ACC but potentially also to other malignancies.

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