Uncommon Adrenal Tumors in Children and Adolescents

Ribeiro, Raul C.; Rodríguez‐Galindo, Carlos; Pinto, Emília M.; Zambetti, Gerard P.; Figueiredo, Bonald C.

doi:10.1002/9781119196235.ch77

Cited by 3 publications

(15 citation statements)

References 121 publications

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“…Most patients with Li-Fraumeni syndrome (about 80%), a rare autosomal dominantly inherited cancer predisposition syndrome, have it. This muta-tion is found in 50-60% of young children with ACT [3]. The result of the aforementioned test in our patient was negative.…”

Section: Pediatr Endocrinol Diabetes Metab 2023contrasting

confidence: 40%

“…The incidence of ACT is higher among girls than boys, with the overall female/male ratio being 1.6 : 1. However, the ratio ranged from 1.7 : 1 in the 0-3 years group to 6.2 : 1 in adolescents (≥ 13 years) [3].…”

Section: Discussionmentioning

confidence: 91%

“…Primary hyperaldosteronism (Conn syndrome) and pure feminization occur very rarely, i.e. in less than 1% of patients [3]. The diagnosis of ACT is based on laboratory tests, which include serum levels of cortisol daily (i.e.…”

Section: Discussionmentioning

confidence: 99%

“…In children, it accounts for 0.6% of tumours and 0.2% of malignancies. It accounts for 5% of all adrenocortical tumours [1][2][3]. The purpose of this article is to present the case of a 10-year-old female patient with symptoms of hypercortisolaemia and androgenization, who was diagnosed with ACC.…”

Section: Introductionmentioning

confidence: 99%

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Adrenocortical carcinoma – 12-year observation period in a single centre. Case report with literature review

Dasiewicz,

Moszczyńska,

Perek

et al. 2023

pedm

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Background: Adrenocortical carcinoma (ACC) accounts for 0.2% of childhood malignancies. The most common symptom in children is rapidly progressive androgenization. Herein, we report a case of a patient with symptoms of hypercortisolaemia and androgenization, who was diagnosed with ACC. Case presentation: In a 10-year-old patient with ACC the course of the disease was complicated by 3 recurrences. She was treated with surgery, chemo-, and radiotherapy. Currently, 8 years after the end of treatment, there have been no signs of recurrence. Conclusions: A patient after ACC treatment requires regular check-ups and long-term observation. Constant supervision enables early diagnosis of disease recurrence, and the use of treatment improves the prognosis.

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Section: Pediatr Endocrinol Diabetes Metab 2023contrasting

confidence: 40%

Section: Discussionmentioning

confidence: 91%

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

See 2 more Smart Citations

Adrenocortical carcinoma – 12-year observation period in a single centre. Case report with literature review

Dasiewicz,

Moszczyńska,

Perek

et al. 2023

pedm

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“…It accounts for 5% of all adrenocortical tumours (ACTs). There are 2 peaks of incidence: in the first decade of life and between 40 and 50 years of age [1][2][3]. Based on an analysis of the literature on adrenocortical carcinoma, this article presents the diagnosis, treatment modalities, genetic background, and prognostic factors of ACC in children.…”

Section: Introductionmentioning

confidence: 99%

Adrenal cortical carcinoma: Paediatric aspects – literature review

Dasiewicz,

Moszczyńska,

Grajkowska

2024

pedm

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Introduction: Adrenocortical carcinoma (ACC) is a rare malignancy in children. Because of this, each patient with suspected ACC requires individualised management, which should be determined at a meeting of a team of multidisciplinary experts in the field. Aim of the study: To summarise data on symptoms, genetic predisposition, and diagnostic procedures for ACC in children. Material and methods: Papers were searched in the PubMed database to identify published randomised clinical trials, reviews, systematic reviews, meta-analyses, and case reports. Results: Most cases of ACC in children occur under the age of 5 years. The most common presenting symptom in 60-80% of paediatric patients is rapidly progressive virilisation. Diagnostics are based on laboratory and imaging evaluation. The mainstay of treatment is surgery, with laparotomy being the preferred method of surgery. Diagnosis is based on histological examination of surgically removed tissue. The Wieneke index is most commonly used in paediatric practice. However, some cases are still classified as "indeterminate histology". Predisposing genetic factors are found in most children with ACC, most commonly a mutation of the TP53 gene. Conclusions: Patients should be diagnosed in large clinical centres with experience in this field. The treatment strategy should be individualised. Genetic testing for TP53 gene mutations is indicated in patients with ACC.

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Biomolecular Engineering of Antidehydroepiandrosterone Antibodies: A New Perspective in Cancer Diagnosis and Treatment Using Single-Chain Antibody Variable Fragment

Fogaça

Alvarenga

Woiski³

et al. 2019

Nanomedicine (Lond.)

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Aim: To develop a monoclonal antibody against dehydroepiandrosterone (DHEA) and miniaturize it, generating a single-chain antibody variable fragment (scFv) against DHEA as an adrenocortical carcinoma (ACC) marker. Material & methods: DHEA conjugated to keyhole limpet hemocyanin was used as an immunogen to obtain anti-DHEA hybridomas. Variable fragments were cloned from hybridoma 5B7 total RNA, and used to detect DHEA in normal adrenal tissue and ACC cells. Results: IgM monoclonal antibody was highly specific, and the recombinant scFv preserved parental antibody characteristics, allowing tissue localization of DHEA. Conclusion: Undefined small lesions are challenges for clinicians and impact clinical adrenocortical tumor management. Generating an anti-DHEA scFv facilitates development of imaging tests for early diagnosis of pediatric ACC.

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Uncommon Adrenal Tumors in Children and Adolescents

Cited by 3 publications

References 121 publications

Adrenocortical carcinoma – 12-year observation period in a single centre. Case report with literature review

Adrenocortical carcinoma – 12-year observation period in a single centre. Case report with literature review

Adrenal cortical carcinoma: Paediatric aspects – literature review

Biomolecular Engineering of Antidehydroepiandrosterone Antibodies: A New Perspective in Cancer Diagnosis and Treatment Using Single-Chain Antibody Variable Fragment

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