The who, when, and why of primary adrenal malignancies: Insights into the epidemiology of a rare clinical entity

Chandrasekar, Thenappan; Goldberg, Hanan; Klaassen, Zachary; Wallis, Christopher J.D.; Woon, Dixon T.S.; Herrera-Cáceres, Jaime O.; Kulkarni, Girish S.; Fleshner, Neil

doi:10.1002/cncr.31916

Cited by 37 publications

(46 citation statements)

References 33 publications

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“…122 patients (80.3%) of white race accounted for the majority, and more patients were married than unmarried (53.3% vs 44.1%). Regarding the literality, tumors occurred more common on the left (55.9%) than on the right (40.1%), which was contrary to the previous report that there were slightly more on the right than on the left [3]. In terms of tumor histology, the number of patients identi ed as grade I + II, grade III and grade IV were 6 (3.9%), 15(9.9%) and 4 (2.6%), respectively.…”

Section: Patient Characteristicscontrasting

confidence: 95%

“…The occurrence rate, although still low, has increased steadily, owing to the improvement of functional and radiographic examination. However, the survival rates of these rare cancers have not improved signi cantly which, to a great extent, attributes to the poor survival outcomes in advanced disease, the 5-year survival rate less than 20% in most series [3,4]. To date, the majority of previous studies have discussed prognosis for localized or the whole adrenocortical carcinoma patients [5][6][7][8][9].…”

Section: Introductionmentioning

confidence: 99%

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Survival Predictors of Metastatic Adrenocortical Carcinoma: a SEER-based Retrospective Study

Yang

Wang

Chen

et al. 2021

Preprint

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Background: Metastatic adrenocortical carcinomas (MAC) have a poor prognosis. Advanced adrenocortical carcinoma often metastasizes to lung and liver. Prognostic factors of MAC have been rarely reported. This study aims to identify the association between specific metastasis and overall survival (OS) in MAC and determine the survival predictors for MAC patients.Methods: MAC patients' data was obtained from Surveillance, Epidemiology, and End Results (SEER) database between 2010 and 2016. Survival differences were analyzed by Kaplan–Meier analysis and log-rank tests. Cox proportional hazard model was used to identify the prognostic factors associated with overall survival.Results: A total of 152 MAC patients were selected, among whom 77 patients (50.7%) were diagnosed with one metastatic site, 75 (49.3%) diagnosed with more than one distant metastasis. For the whole MAC cohort, multivariable analysis showed that year of diagnosis between 2013 and 2016, without liver metastasis, surgery and chemotherapy were significantly favorable predictors of OS. For patients with one metastatic site, lung metastases had a better survival outcome than liver metastases(p=0.037). Besides, compared with patients who didn’t received surgery, patients underwent surgery were correlated with longer OS (p=0.004). For patients with more than one site of distant metastases, married status, surgery, and chemotherapy predicted a better OS. Radiotherapy did not improve overall survival outcomes in the three cohort.Conclusion: Liver metastasis has a poor prognosis. Year of diagnosis, metastatic sites, surgery and chemotherapy were significant prognostic factors for OS in MAC patients. For patients with single metastasis, surgery was a favorable prognostic factor, while married patients, surgery and chemotherapy predicted a better survival outcome in patients with more than one metastasis. Based on the collective findings, surgery can be regarded as the preferred treatment option for all MAC patients. Besides, chemotherapy is also a good choice for patients with multiple metastases.

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Section: Patient Characteristicscontrasting

confidence: 95%

Section: Introductionmentioning

confidence: 99%

Survival Predictors of Metastatic Adrenocortical Carcinoma: a SEER-based Retrospective Study

Yang

Wang

Chen

et al. 2021

Preprint

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“…Patients with PAL might die before pathological confirmation (13,35). In addition, recent studies suggested that unlike other adrenal malignancies, adrenalectomy preceding surgery provided no benefit to PAL patients (4,5,9). In contrast, surgery may expose PAL patients to the risk of delay in starting chemotherapy (36).…”

Section: Discussionmentioning

confidence: 99%

“…Though only 5-10% of them are malignant, the prognoses of these adrenal malignancies are dismal ( 8 ). Furthermore, among these adrenal malignancies, the therapeutic regimen for PAL is different ( 4 , 5 , 9 ). Therefore, early diagnosis of PAL is essential.…”

Section: Introductionmentioning

confidence: 99%

A Novel Diagnostic Model for Primary Adrenal Lymphoma

Xue

Zhou

et al. 2021

Front. Endocrinol.

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ObjectivePrimary adrenal lymphoma (PAL) is easily misdiagnosed as other adrenal masses, such as adrenocortical carcinoma and pheochromocytoma, but patients with PAL benefit little from surgery. The diagnostic method for PAL thus far is limited to adrenal biopsy. In our study, we aimed to develop a quick and efficient diagnostic method for PAL.Methods and ResultsAt the same institution, 505 patients (between 2009 and 2019) and 171 patients (between 2019 and 2020) were separately included in the primary and validation studies. Univariate and multivariate analyses were conducted to evaluate clinical manifestations, laboratory findings, and radiological characteristics. Four determinants (age, bilateral masses, high-density lipoprotein cholesterol, and lactate dehydrogenase) were selected and further incorporated into a regression model to screen PAL. Accordingly, the nomogram was developed for clinical practice. In the primary study, the nomogram showed good discrimination, with an area under the receiver operating characteristic (ROC) curve (AUC) of 95.4% (95% CI, 90.6%–100.0%). Further validation study verified the efficacy of the nomogram, with an AUC of 99.0% (95% CI, 96.9%–100.00%) and 100.0% in all patients and patients with bilateral masses, respectively, and a sensitivity/specificity/positive predictive value (PPV)/negative predictive value (NPV) of 66.67%/99.40%/66.67%/99.40%, 66.67%/100%/100%/92.86%, 50%/99.20%/50%/99.20%, and 100%/100%/100%/100%, in all patients, patients with bilateral adrenal masses, patients with nonfunctional adrenal masses, and patients with positive catecholamine results, respectively. The validation study also revealed a diagnostic specificity of 99.35% and 100% for patients with a unilateral adrenal mass and functional PCC, respectively.ConclusionsThe presented nomogram is the first user-friendly diagnostic model for PAL that simplifies the complex diagnostic process into personalized numeric estimates. We deem that patients who score below 50 are less likely to have PAL. We suggest that clinicians should arrange adrenal biopsy and surgery for patients with nonfunctional tumors and overt catecholamine-secreting tumors, respectively, who receive a score of 50 points or higher to confirm the diagnosis as soon as possible.

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“…histologic groups: adrenocortical carcinomas, pheochromocytomas and paragangliomas, neuroblastomas, non-Hodgkin lymphomas, and sarcomas [17] . However, according to clinical experience, the common primary adrenal malignancies in adults mainly included adrenocortical carcinoma (ACC), pheochromocytomas and paraganglioma (PPGL), lymphoma, and sarcoma (SA).…”

Section: Discussionmentioning

confidence: 99%

Summary of clinical characteristics of common primary adrenal malignancies in adults

zhang¹,

wang²,

Li³

et al. 2020

Preprint

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Background To summary the clinical characteristics of common primary adrenal malignancies in adults, the survival time of the patients, and the prognostic factors associated with these rare malignancies. Methods The Surveillance, Epidemiology, and End Results database (1975–2016) was queried for all patients who were diagnosed with primary adrenal malignancies, including adrenocortical carcinoma(ACC), pheochromocytoma and paraganglioma (PPGL), lymphoma, and sarcoma (SA). The clinical characteristics, overall survival (OS) and cancer-specific survival (CSS) were analyzed, in which propensity score methodology, logistic regression modeling and Kaplan-Meier survival curves were used. The Multivariate Cox proportional hazard model was used to identify factors affecting the prognosis of patients with ACC. Results 3204 patients with primary adrenal malignancies were identified, including 2180 with ACC, 593 with PPGL, 307 with lymphoma, and 124 with SA. Generally, PPGL had a better prognosis than others. For ACC and PPGL, the non-metastatic group had better OS and CSS than the metastatic group; for SA, the non-metastatic group had better OS, however, there was no significant difference in CSS between the two groups. Furthermore, For ACC, the single metastasis group had a better OS than the multiple metastasis group. After tumors metastasis, the surgery group, surgery of primary adrenal lesions, had better OS and CSS than the non-surgery group for ACC and PPGL. For lymphoma, the chemotherapy group had better OS and CSS; for ACC and PPGL, the chemotherapy group had better CSS and worse OS; for SA, the survival showed no significant difference between the two groups. Radiotherapy had the same effect on patients' survival as chemotherapy, except for the lymphoma and the ACC. As for lymphoma, the radiotherapy group had better CSS, whereas the survival showed no significant difference in OS between the two groups. For ACC, the non- radiotherapy group had better CSS and OS. More importantly, for lymphoma, under the condition that both cohorts were the same basically, the surgical group had a better prognosis. The prognosis of ACC was related to sex, age, seer historic stage, surgery, chemotherapy. Conclusion Understanding the clinical characteristics of these tumors and factors affecting prognosis can facilitate the selection of more appropriate clinical treatment options.

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The who, when, and why of primary adrenal malignancies: Insights into the epidemiology of a rare clinical entity

Cited by 37 publications

References 33 publications

Survival Predictors of Metastatic Adrenocortical Carcinoma: a SEER-based Retrospective Study

Survival Predictors of Metastatic Adrenocortical Carcinoma: a SEER-based Retrospective Study

A Novel Diagnostic Model for Primary Adrenal Lymphoma

Summary of clinical characteristics of common primary adrenal malignancies in adults

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