Adrenal Cortical Carcinoma in Infancy

Longui, Carlos Alberto

doi:10.1590/1984-0462/;2019;37;1;00021

Cited by 4 publications

(6 citation statements)

References 4 publications

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“…Adrenocortical tumors are more likely to form in children under the age of 5 years if they are virilized 19 , exhibit symptoms of hormonal hypersecretion, Cushing syndrome, hypertension, weight gain, or voice changes 14 , enlarged genitalia, hirsutism 7 , increased height, moon face, osteoporosis 20 , or muscle hypertrophy 14 . Clinical symptoms for females younger than 8 years old and males younger than 10 years old include growth spurts, bone age advancement, axillary sweating, odor, an increase in skin oiliness, the appearance of acne, and the beginning of secondary sexual characteristics like pubic hair, darkening of genital skin, and growth in the penile or clitoral corpus cavernous 21 . For those under the age of 14, the main clinical symptoms were fever, pain, and abdominal distention.…”

Section: Discussionmentioning

confidence: 99%

“…Also, Tp53 mutation analysis makes accurate diagnosis possible 29 . Regarding radiographs, CT, which is the most helpful, is used to make the diagnosis in the first place 21 . CT scan helps determine the stage and location of the tumor and the treatment plan 27 .…”

Section: Discussionmentioning

confidence: 99%

“…CT scan helps determine the stage and location of the tumor and the treatment plan 27 . However, echocardiography of the adrenals is a possibility and requires a high level of experience and expertise 21 . Bone scintigraphy to look for metastases in the bones 8 .…”

Section: Discussionmentioning

confidence: 99%

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Adrenocortical carcinoma in a 10-month-old infant: A literature review and a rare case report

Al-Ghotani

Alabdallah

Shaaban

et al. 2023

Annals of Medicine &Amp; Surgery

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Introduction and importance: Adrenocortical carcinoma (ACC) in children is a rare condition. The annual incidence of ACC is extremely low, with only 0.2–0.3 cases per million children. The clinical presentations of ACC are numerous, such as terminal hair appearance, pubertal progress, hypercortisolism, enlarged clitoris, acne, systemic arterial hypertension, weight gain, and voice change. Case presentation: A 10-month-old female infant presented by her parents to the Department of Endocrinology with a mass on the right adrenal gland and Cushing’s syndrome symptoms. Surgery was performed. The death occurred after two times resuscitation due to sudden cardiac arrest. Clinical discussion: The adrenal gland consists of two distinct parts. Different types of tumors arise from each part of the adrenal gland. The most common tumor in adrenomedullary tumors was neuroblastoma which accounted for 60.4% of adrenal tumors. ACC in children is a rare condition. The etiology of ACTs is unclear. Conclusion: This case emphasizes that early diagnosis has a considerable role in preventing major complications. Also to advise considering ACC as a differential diagnosis when similar symptoms are found in an infant.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

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Adrenocortical carcinoma in a 10-month-old infant: A literature review and a rare case report

Al-Ghotani

Alabdallah

Shaaban

et al. 2023

Annals of Medicine &Amp; Surgery

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“…In the past 20 years, molecular biology studies on ACC have made significant progress [ 46 , 47 ], but this cancer's primary pathogenesis is still unclear. Moreover, recent epidemiological studies have shown that the incidence of ACC has increased yearly in the past 40 years, but the survival rate of patients has not improved [ 3 ].…”

Section: Discussionmentioning

confidence: 99%

Comprehensive Multiomics Analysis Reveals Potential Diagnostic and Prognostic Biomarkers in Adrenal Cortical Carcinoma

Zhao

et al. 2022

Computational and Mathematical Methods in Medicine

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Adrenal cortical carcinoma (ACC) is a severe malignant tumor with low early diagnosis rates and high mortality. In this study, we used a variety of bioinformatic analyses to find potential prognostic markers and therapeutic targets for ACC. Gene Expression Omnibus (GEO) and The Cancer Genome Atlas (TCGA) data sets were used to perform differential expressed analysis. WebGestalt was used to perform enrichment analysis, while String was used for protein-protein analysis. Our study first detected 28 up-regulation and 462 down-regulation differential expressed genes through the GEO and TCGA databases. Then, GO functional analysis, four pathway analyses (KEGG, REACTOME, PANTHER, and BIOCYC), and protein-protein interaction network were performed to identify these genes by WebGestalt tool and KOBAS website, as well as String database, respectively, and finalize 17 hub genes. After a series of analyses from GEPIA, including gene mutations, differential expression, and prognosis, we excluded one candidate unrelated to the prognosis of ACC and put the remaining genes into pathway analysis again. We screened out CCNB1 and NDC80 genes by three algorithms of Degree, MCC, and MNC. We subsequently performed genomic analysis using the TCGA and cBioPortal databases to better understand these two hub genes. Our data also showed that the CCNB1 and NDC80 genes might become ACC biomarkers for future clinical use.

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“…Adrenalectomy is the first line therapy offering the best prognosis, while the adjuvant therapies are less standardized in children; genetic testing is recommended before surgery and lifelong follow-up is required (6,7). The tumor rapidly spreads at the local level and to distant sites (6,8). In general, the pattern of this condition renders it a rather distinct entity than the same tumor identified in adults, despite the fact than even in pediatric cases the disease is extremely severe (for instance, in children the 5-year survival rate is 46-55%) (9).…”

Section: Introductionmentioning

confidence: 99%

Adrenocortical carcinoma: Pediatric aspects (Review)

et al. 2022

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Adrenocortical carcinoma (adrenal cortex-derived cancer), an orphan malignancy, is a very aggressive disease that affects both adults and children with an annual incidence of 1-2 adult and 0.2-0.38 pediatric cases/million (in the pediatric population it represents 0.2% of all cancers), with a female predominance. A total of 80-90% of cases have hormonal imbalances such as Cushing syndrome, virilization, and puberty anomalies. Precocious puberty (PP) of iso-or hetero-sexual pattern is independent of gonadotropin-releasing hormone (GnRH) (high testosterone/estrogens and low FSH/LH) but post-operative activation of GnRH may be expected (central PP). PP is accompanied by accelerated growth while Cushing syndrome by reduced growth velocity. Pure androgen-secreting tumors have been exceptionally described. A total of 50-80% of children have different genetic/epigenetic anomalies involving tumor protein p53 (most often, almost half of the cases; with a population cluster in Southern Brazilian children), insulin-like growth factor, multiple endocrine neoplasia type 1 (MEN1), PRKAR1A, dysfunctional alternative lengthening of telomeres. Hereditary syndromes associated to adrenocortical carcinoma include Li-Fraumeni, Beckwith-Wiedemann, MEN1, and Lynch. Recently, mutations in epidermal growth factor receptor have been reported in teenagers, suggesting the future use of tyrosine kinase inhibitors. Adrenalectomy is the first line therapy offering the best prognosis if complete tumor removal is achieved; genetic testing is recommended before surgery. Adjuvant therapies are less standardized in children (mitotane is a key adjuvant drug in addition with different regimes of chemotherapy such as etoposide, Adriamycin and cisplatin). A Ki-67 value of at least 15% is a predictor of poor outcome. Weiss score also serves as a prognostic factor, as well as the tumor size at diagnosis. The prognosis of adrenocortical carcinoma is poor with an overall 5-year survival rate of 55%; a Weiss score of at least 6 is associated with a 2-year survival rate of 35%. At present, pediatric adrenocortical carcinoma still represents a severe condition that requires prompt intervention and a multidisciplinary team. Further development of molecular markers is required for an improved understanding of the disease thus improving the protocols of approach and the prognostic.

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Adrenal Cortical Carcinoma in Infancy

Cited by 4 publications

References 4 publications

Adrenocortical carcinoma in a 10-month-old infant: A literature review and a rare case report

Adrenocortical carcinoma in a 10-month-old infant: A literature review and a rare case report

Comprehensive Multiomics Analysis Reveals Potential Diagnostic and Prognostic Biomarkers in Adrenal Cortical Carcinoma

Adrenocortical carcinoma: Pediatric aspects (Review)

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