Adrenocortical Carcinoma - A 25 Years Tertiary Centre Experience and Short Review of the Literature

Nunes, Joana

doi:10.15406/emij.2018.06.00148

Cited by 3 publications

(3 citation statements)

References 32 publications

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“…Moreover, tumor volume > 500 cm 3 was related with shorter survival rate in the multivariable analysis. In line with previous reports [27,28], we found that most of the ACC cases were women at forthfifth decade of life, with stage II (ENSAT criteria) and located on the left, albeit not all studies agree with the left sided preponderance [29]. We also observed a trend for positive correlation between tumor size (volume and diameter) and left localization.…”

Section: Discussionsupporting

confidence: 91%

Adrenocortical Cancer: A 20-Year Experience of a Single Referral Center in Prognosis and Outcomes

et al. 2021

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Adrenocortical carcinoma (ACC) is a rare but very aggressive endocrine malignancy with poor survival. Histopathology is important for diagnosis, while in some cases immunohistochemical markers and gene profiling of the resected tumor may be superior to current staging systems to determine prognosis. We aimed to present the 20-year experience at a tertiary hospital in patients with ACCs and correlate the immunohistochemical characteristics of ACCs with the clinical and morphological characteristics of the tumors and the survival of the patients. Forty-five patients with ACC were included in the study. All the resections were R0. The tumor size and weight, the disease stage (ENSAT classification), Weiss score and Helsinki score were examined along with immunohistochemical expression of inhibin-A, melan A, calretinin, Ki67, synaptophysin, p53, vimentin, CKAE1/AE3. The male to female ratio was 1:1.37. The median age at diagnosis was 55.5 years (IQR 19–77). The median size of ACCs was 9 cm (IQR 3.5–22 cm) and the median weight 127 g (IQR 18–1400 g). The median follow up period was 18 months (IQR 1–96). Ki67 varied from<1% to 75% (median: 16.4%). The expression of melan-A and lower expression of Ki-67 (≤4) were independently associated with longer OS time (p=0.01 and p=0.04, respectively). In multivariable analysis, tumor volume>400 cm3 (p=0.046), Weiss score>5 (p=0.007) and overexpression of p53 (p=0.036) were independent risk factors for shorter survival. Adrenocortical carcinoma is a rare and very aggressive endocrine malignancy. The most important factors that determine long-term prognosis of ACC are the disease stage at diagnosis, the Weiss score, and the Ki67 index. Immunohistochemical markers such as melan A could also serve as prognostic factors.

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Section: Discussionsupporting

confidence: 91%

Adrenocortical Cancer: A 20-Year Experience of a Single Referral Center in Prognosis and Outcomes

et al. 2021

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“…The tumor stage is also associated with OS, with worse outcome in higher stages. 40 In the patients at stage II—the majority of our cohort, tumor size equal or superior to 10 cm, showed an effect on OS in multivariate analysis, with a high hazard ratio. This finding supports that simply diagnosing ACC is not enough for proper patient management and that more data should be integrated.…”

Section: Discussionmentioning

confidence: 73%

Histological scores and tumor size on stage II in adrenocortical carcinomas

et al. 2021

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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.

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“…2,3 The clinical presentation can be varied and related to either hormone excess (cushiongoid features, hirsutism, virilization) or non specific symptomatology related to tumour growth (pain, abdominal fullness, general malaise). 4 Most adrenal cortical carcinomas occur sporadically but rarely its association with hereditary syndromes like Li Fraumeni syndrome, Beckwith-Weidmann syndrome is reported in pediatric population, while in adults higher prevalence in association with Lynch syndrome, Multiple endocrine neoplasia type 1 (MEN 1) and Type 1 neurofibromatosis is well documented. 5 Although radiological and histopathological characterization of adrenal cortical neoplasm as adenoma or carcinoma is quite straightforward, sometimes a benign tumour showing haemorrhage and revascularization on imaging and intermediate histology can pose a challenge.…”

Section: Introductionmentioning

confidence: 99%

Myxoid variant of adrenocortical carcinoma in a middle aged female - A case report

Hasan¹,

Meraj²,

Rahman³

et al. 2021

JDPO

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Adrenocortical neoplasms are rare tumours that can be diagnostically challenging in some cases. Numerous diagnostic algorithms and scoring systems are in practice to differentiate adenoma from carcinoma. Besides, the different histological variants of adrenocortical carcinoma need to be identified. We present a case of 44 year old female with vaginal bleeding with a large, heterogenous suprarenal mass on imaging. Provisional clinical diagnosis of pheochromocytoma was made and patient underwent adrenalectomy. The histological examination of tumour sections showed sheets and trabeculae of moderately pleomorphic cells with extracellular mucin deposition. Immunohistochemical evaluation showed tumour cells to be negative for chromogranin with focal pan cytokeratin positivity. Finally a diagnosis of adrenal cortical carcinoma-myxoid variant was made. Post operative period was uneventful and following en bloc resection of the tumour patient did not receive any adjuvant adrenolytic treatment. The patient is doing well at 6 months of follow up period, without any evidence of recurrence.

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Adrenocortical Carcinoma - A 25 Years Tertiary Centre Experience and Short Review of the Literature

Cited by 3 publications

References 32 publications

Adrenocortical Cancer: A 20-Year Experience of a Single Referral Center in Prognosis and Outcomes

Adrenocortical Cancer: A 20-Year Experience of a Single Referral Center in Prognosis and Outcomes

Histological scores and tumor size on stage II in adrenocortical carcinomas

Myxoid variant of adrenocortical carcinoma in a middle aged female - A case report

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