Adrenal cortical tumours: 25 years’ experience at the Royal Children's Hospital, Melbourne

McDonnell, Ciara M.; Zacharin, Margaret

doi:10.1046/j.1440-1754.2003.00268.x

Cited by 17 publications

(23 citation statements)

References 22 publications

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“…Although not the topic of this guideline, all children with a diagnosis of ACC should undergo a systematic search of germline TP53 pathogenic variants, because 50-90% of ACC in children are related to germline pathogenic TP53 variants (293,294,295).…”

Section: Reasoningmentioning

confidence: 99%

European Society of Endocrinology Clinical Practice Guidelines on the management of adrenocortical carcinoma in adults, in collaboration with the European Network for the Study of Adrenal Tumors

Fassnacht

Dekkers

Else

et al. 2018

European Journal of Endocrinology

670

867

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Adrenocortical carcinoma (ACC) is a rare and in most cases steroid hormone-producing tumor with variable prognosis. The purpose of these guidelines is to provide clinicians with best possible evidence-based recommendations for clinical management of patients with ACC based on the GRADE (Grading of Recommendations Assessment, Development and Evaluation) system. We predefined four main clinical questions, which we judged as particularly important for the management of ACC patients and performed systematic literature searches: (A) What is needed to diagnose an ACC by histopathology? (B) Which are the best prognostic markers in ACC? (C) Is adjuvant therapy able to prevent recurrent disease or reduce mortality after radical resection? (D) What is the best treatment option for macroscopically incompletely resected, recurrent or metastatic disease? Other relevant questions were discussed within the group. Selected Recommendations: (i) We recommend that all patients with suspected and proven ACC are discussed in a multidisciplinary expert team meeting. (ii) We recommend that every patient with (suspected) ACC should undergo careful clinical assessment, detailed endocrine work-up to identify autonomous hormone excess and adrenal-focused imaging. (iii) We recommend that adrenal surgery for (suspected) ACC should be performed only by surgeons experienced in adrenal and oncological surgery aiming at a complete en bloc resection (including resection of oligo-metastatic disease). (iv) We suggest that all suspected ACC should be reviewed by an expert adrenal pathologist using the Weiss score and providing Ki67 index. (v) We suggest adjuvant mitotane treatment in patients after radical surgery that have a perceived high risk of recurrence (ENSAT stage III, or R1 resection, or Ki67 >10%). (vi) For advanced ACC not amenable to complete surgical resection, local therapeutic measures (e.g. radiation therapy, radiofrequency ablation, chemoembolization) are of particular value. However, we suggest against the routine use of adrenal surgery in case of widespread metastatic disease. In these patients, we recommend either mitotane monotherapy or mitotane, etoposide, doxorubicin and cisplatin depending on prognostic parameters. In selected patients with a good response, surgery may be subsequently considered. (vii) In patients with recurrent disease and a disease-free interval of at least 12 months, in whom a complete resection/ablation seems feasible, we recommend surgery or alternatively other local therapies. Furthermore, we offer detailed recommendations about the management of mitotane treatment and other supportive therapies. Finally, we suggest directions for future research.

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Section: Reasoningmentioning

confidence: 99%

European Society of Endocrinology Clinical Practice Guidelines on the management of adrenocortical carcinoma in adults, in collaboration with the European Network for the Study of Adrenal Tumors

Fassnacht

Dekkers

Else

et al. 2018

European Journal of Endocrinology

670

867

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“…In contrast, Michalkiewicz et al (10) reviewed the cases of 20 patients with small adrenocortical tumors recorded in the International Registry of Childhood Adrenocortical Tumors between May 1988 and December 1994; all of these patients had clinical signs of virilization, 7 had signs or symptoms of Cushing syndrome, and no feminizing features were observed. In another study conducted at the Royal Children's Hospital in Melbourne, there were 12 children with adrenocortical tumors over a 25-year period, none of which had symptoms of feminization (11).…”

Section: Discussionmentioning

confidence: 99%

Estrogen-Only–Producing Adrenal Mass As An Overlooked Etiology Of Isosexual Precocious Puberty In Girls: A Case Report And Literature Review

Haghshenas

Mollaian

Alizadeh

et al. 2017

AACE Clinical Case Reports

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Objective: An estrogen-only-producing adrenal tumor is a rare etiology of isosexual precocious puberty (PP) in girls. Methods: We describe a 2.5-year-old girl who presented with signs and symptoms of isosexual PP. In primary laboratory and imaging investigations, serum estradiol level was found to be increased, while follicle-stimulating hormone, luteinizing hormone, adrenocorticotropic hormone, cortisol, testosterone, and 17-hydroxyprogesterone levels were shown to be within normal ranges. Abdominopelvic ultrasound and abdominal computed tomography revealed a right-sided adrenal mass, which was initially assumed to be an incidentaloma. Diagnosis of an adrenal cortical tumor was confirmed by tumor resection. Histologic examination revealed the tumor to be a benign adenoma with scattered areas of necrosis. Results: Tumor resection resulted in normalized serum estradiol and diminished clinical signs after 3 weeks. The child received no additional treatment and remains symptom free after 30 months of close observation. Conclusion: With the intent to spread the awareness of adrenocortical tumors as a potentially malignant cause of PP in children, and due to the rarity of an estrogen-producing adrenal mass, we discuss the clinical and biochemical features of our patient and a brief review of the literature.

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“…Data regarding ACT during childhood is scarce; however, it was stated that in childhood, the most prevalent form is virilizing ACT (10,11,12,13,14,15,16). Michalkiewickz et al (13) investigated the data of 254 children with ACT from an international registry and suggested that 55.1% of children with ACT had a tumor leading to isolated virilization and 29.2% had a tumor leading to combined virilization and Cushing’s syndrome.…”

Section: Discussionmentioning

confidence: 99%

Need for Comprehensive Hormonal Workup in the Management of Adrenocortical Tumors in Children

Gönç

Özön²,

Çakır³

et al. 2014

Jcrpe

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Objective: Clinical findings do not reflect the excess hormonal status in adrenocortical tumors (ACTs) in children. Identification of abnormal hormone secretion may help provide the tumor marker and delineate those patients with a risk of adrenal suppression following tumor removal. To analyze the impact of complete hormonal assessment regardless of the clinical presentation in hormone-secreting ACTs in childhood. Methods: Association of hormonal workup at diagnosis with the clinical findings and frequency of adrenal suppression postoperatively were analyzed in 18 children with ACT.Results: Seventeen of the 18 patients had functional ACT. Clinical findings suggested isolated virilization and isolated Cushing’s syndrome in 38.8% and 17.6% of patients, respectively. Hormonal workup revealed a frequency of 83.3% for hyperandrogenism. The majority of the tumors (50%) had mixed type hormonal secretion. Hypercortisolism existed in 28.5% of children with isolated virilization and hyperandrogenism was found in 2/3 of children with isolated Cushing’s syndrome. Various androgens other than dehydroepiandrosterone sulfate were also determined to be high in hyperandrogenism. Increased testosterone was a highly prevalent tumor marker. Nine patients (3 with no signs of hypercortisolism) had adrenal suppression following tumor removal which lasted 1-24 months.Conclusion: Complete hormonal workup showed the predominance of mixed hormone-secreting type of tumor in the patients who lacked the appropriate clinical findings and also showed that patients lacking signs of Cushing’s syndrome could have postoperative adrenal suppression. Clinical findings may not reflect the abnormal hormone secretion in all cases and tumor markers as well as risk of postoperative adrenal suppression can best be determined by complete hormonal evaluation at the time of diagnosis.

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Adrenal cortical tumours: 25 years’ experience at the Royal Children's Hospital, Melbourne

Abstract: Adrenal cortical tumours should always be considered in the differential for adrenal hormone excess. New information provided by mutational analysis may predict ongoing risks. Lifelong regular follow up is required.

Cited by 17 publications

References 22 publications

European Society of Endocrinology Clinical Practice Guidelines on the management of adrenocortical carcinoma in adults, in collaboration with the European Network for the Study of Adrenal Tumors

European Society of Endocrinology Clinical Practice Guidelines on the management of adrenocortical carcinoma in adults, in collaboration with the European Network for the Study of Adrenal Tumors

Estrogen-Only–Producing Adrenal Mass As An Overlooked Etiology Of Isosexual Precocious Puberty In Girls: A Case Report And Literature Review

Need for Comprehensive Hormonal Workup in the Management of Adrenocortical Tumors in Children

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