Clinical and histopathological features of adrenocortical neoplasms in children: Retrospective review from a single specialist center

Sakoda, Akiko; Mushtaq, Imran; Levitt, Gill; Sebire, Neil J.

doi:10.1016/j.jpedsurg.2013.09.008

Cited by 26 publications

(26 citation statements)

References 20 publications

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“…In an analysis of 29 children treated at Great Ormond Street Hospital, London, between 1987 and 2011 there was a strong association between high Weiss score and large tumour size and adverse outcome, suggesting that the same histological criteria can be used as in adult ACC. In very young children aged less than 3 years, however, a discrepancy between adverse pathological features and a positive clinical outcome was observed, suggesting that adrenocortical tumours in this age group should be classified as neoplasms of unknown malignant potential.…”

Section: Adrenocortical Carcinoma In Children – a Different Disease?mentioning

confidence: 99%

Diagnosis, treatment and outcome of adrenocortical cancer

Mihai

2015

British Journal of Surgery

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Background: Adrenocortical cancer (ACC) is a rare disease with a dismal prognosis. The majority of patients are diagnosed with advanced disease and raise difficult management challenges.Methods: All references identified in PubMed, published between 2004 and 2014, using the keywords 'adrenocortical cancer' or 'adrenal surgery' or both, were uploaded into a database. The database was interrogated using keywords specific for each field studied.Results: In all, 2049 publications were identified. There is ongoing debate about the feasibility and oncological outcomes of laparoscopic adrenalectomy for small ACCs, and data derived from institutional case series have failed to provide an evidence level above expert opinion. The use of mitotane (1-(2-chlorophenyl)-1-(4-chlorophenyl)-2,2-dichloroethane) in combination with chemotherapy in the treatment of metastatic disease has been assessed in an international randomized trial (FIRM-ACT trial) involving patients with ACC. Based on this trial, mitotane plus etoposide, doxorubicin and cisplatin is now the established first-line cytotoxic therapy owing to a higher response rate and longer median progression-free survival than achieved with streptozocin-mitotane. For patients with tumours smaller than 5 cm and with no signs of lymph node or distant metastases, survival is favourable with a median exceeding 10 years. However, the overall 5-year survival rate for all patients with ACC is only 30 per cent. Conclusion:Open and potentially laparoscopic adrenalectomy for selected patients is the main treatment for non-metastatic ACC, but the overall 5-year survival rate remains low.Cutting edge articles are invited by the BJS Editorial Team, and focus on how current research and innovation will affect future clinical practice.

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Section: Adrenocortical Carcinoma In Children – a Different Disease?mentioning

confidence: 99%

Diagnosis, treatment and outcome of adrenocortical cancer

Mihai

2015

British Journal of Surgery

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“…Distinguishing an adrenocortical adenoma from adrenocortical carcinoma on the basis of histologic findings is often difficult, especially in children. The Weiss scoring system might be of some value in predicting malignant behavior [8,9]. The prognosis of adrenal adenoma is excellent, in contrast to adrenocortical carcinoma [9].…”

Section: Discussionmentioning

confidence: 99%

Congenital Adrenocortical Tumor in an Asymptomatic Neonate with Positive Newborn Screen for Congenital Adrenal Hyperplasia: A Case Report

Hoang¹,

Eric²,

Mace³

et al. 2017

IJIPEM

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Adrenocortical tumors (ACTs) are rare in childhood, particularly in the neonatal period. ACTs in children usually secrete hormones that can cause virilization, precocious puberty, or Cushing syndrome. However, in many cases the diagnosis is delayed because of the generally healthy appearance of the child and the lack of a palpable abdominal mass. ACTs can also be misdiagnosed as congenital adrenal hyperplasia (CAH) especially if 17-hydroxyprogesterone (17-OHP) is elevated. We describe a case of neonatal ACT diagnosed in an asymptomatic newborn with positive/elevated 17-OHP on newborn screening. On examination there were no signs of virilization, Cushing syndrome, hemihypertrophy, or hypertension, and there was no palpable abdominal mass. While awaiting confirmatory results of an elevated 17-OHP level, an abnormally low adrenocorticotropic hormone level was noted. Abdominal ultrasound, obtained to evaluate the adrenal glands, showed a right adrenal mass with peripheral calcification. MRI revealed a 3.6 x 3.5 x 3.4 cm heterogeneously enhancing ovoid mass in the right adrenal gland. Diagnosis of an ACT was also confirmed by laboratory data, and histopathology of the tumor. Surgical resection was safely performed with perioperative steroid replacement. Genetic analysis of CYP21A2 and CYP11B1 genes was negative. A germ-line point mutation in the TP53 region was discovered, suggesting Li-Fraumeni syndrome. ACTs should be considered in the differential diagnosis of neonates with elevated 17-OHP detected by newborn screening for CAH. Adrenal ultrasound is recommended in cases of significantly abnormal CAH screen results to rule out or treat adrenal neoplasms.

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“…The histological features of ACTs often do not allow unequivocal classification as adenomas or carcinomas, and such cases are currently classified as tumors of indeterminate histology, undetermined malignant potential, or simply “adrenocortical neoplasms” (13,17–18). In our IPACTR series, seven of 52 tumors (13%) had undetermined histological findings.…”

Section: Discussionmentioning

confidence: 99%

Prognostic Significance of Major Histocompatibility Complex Class II Expression in Pediatric Adrenocortical Tumors: A St. Jude and Children's Oncology Group Study

Pinto

Rodríguez‐Galindo

Choi

et al. 2016

Clinical Cancer Research

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Purpose Histological markers that differentiate benign and malignant pediatric adrenocortical tumors (ACTs) are lacking. Previous studies have implicated an association of major histocompatibility complex (MHC) class II expression with ACT prognosis. Here we determined the expression of MHC class II as well as the cell of origin of these immunological markers in pediatric ACT. The impact of MHC class II gene expression on outcome was determined in a cohort of uniformly-treated children with adrenocortical carcinomas. Experimental Design We analyzed the expression of MHC class II and a selected cluster of differentiation genes in 63 pediatric ACTs by Affymetrix Human U133 Plus 2.0 or HT HG-U133+PM gene chip analyses. Cells expressing MHC class II were identified by morphologic and immunohistochemical assays. Results MHC class II expression was significantly greater in adrenocortical adenomas than in carcinomas (P = 4.8 × 10−6) and was associated with a higher progression-free survival (PFS) estimate (P = 0.003). Specifically, HLA-DPA1 expression was most significantly associated with PFS after adjustment for tumor weight and stage. HLA-DPA1 was predominantly expressed by hematopoietic infiltrating cells and undetectable in tumor cells in 23 of 26 cases (88%). Conclusion MHC class II expression, which is produced by tumor- infiltrating immune cells, is an indicator of disease aggressiveness in pediatric ACT. Our results suggest that immune responses modulate adrenocortical tumorigenesis, and may allow the refinement of risk stratification and treatment for this disease.

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Clinical and histopathological features of adrenocortical neoplasms in children: Retrospective review from a single specialist center

Cited by 26 publications

References 20 publications

Diagnosis, treatment and outcome of adrenocortical cancer

Diagnosis, treatment and outcome of adrenocortical cancer

Congenital Adrenocortical Tumor in an Asymptomatic Neonate with Positive Newborn Screen for Congenital Adrenal Hyperplasia: A Case Report

Prognostic Significance of Major Histocompatibility Complex Class II Expression in Pediatric Adrenocortical Tumors: A St. Jude and Children's Oncology Group Study

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