PMID- 35317446
OWN - NLM
STAT- PubMed-not-MEDLINE
LR  - 20220324
IS  - 1792-1015 (Electronic)
IS  - 1792-0981 (Print)
IS  - 1792-0981 (Linking)
VI  - 23
IP  - 4
DP  - 2022 Apr
TI  - Adrenocortical carcinoma: Pediatric aspects (Review).
PG  - 287
LID - 10.3892/etm.2022.11216 [doi]
LID - 287
AB  - 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.
CI  - Copyright (c) 2020, Spandidos Publications.
FAU - Sandru, Florica
AU  - Sandru F
AD  - Department of Dermatology, 'Carol Davila University of Medicine and Pharmacy, 
      050474 Bucharest, Romania.
AD  - Department of Dermatology, 'Elias' Emergency Hospital, 011461 Bucharest, Romania.
FAU - Petca, Razvan-Cosmin
AU  - Petca RC
AD  - Department of Urology, 'Carol Davila' University of Medicine and Pharmacy, 050474 
      Bucharest, Romania.
AD  - Department of Urology, 'Prof. Dr. Theodor Burghele' Clinical Hospital, 061344 
      Bucharest, Romania.
FAU - Carsote, Mara
AU  - Carsote M
AD  - Department of Endocrinology, 'Carol Davila' University of Medicine and Pharmacy, 
      050474 Bucharest, Romania.
AD  - Department of Endocrinology, 'C.I. Parhon' National Institute of Endocrinology, 
      011863 Bucharest, Romania.
FAU - Petca, Aida
AU  - Petca A
AD  - Department of Obstetrics and Gynecology, 'Carol Davila' University of Medicine 
      and Pharmacy, 050474 Bucharest, Romania.
AD  - Department of Obstetrics and Gynecology, 'Elias' Emergency Hospital, 022461 
      Bucharest, Romania.
FAU - Dumitrascu, Mihai Cristian
AU  - Dumitrascu MC
AD  - Department of Obstetrics and Gynecology, 'Carol Davila' University of Medicine 
      and Pharmacy, 050474 Bucharest, Romania.
AD  - Department of Obstetrics and Gynecology, University Emergency Hospital, 050098 
      Bucharest, Romania.
FAU - Ghemigian, Adina
AU  - Ghemigian A
AD  - Department of Endocrinology, 'Carol Davila' University of Medicine and Pharmacy, 
      050474 Bucharest, Romania.
AD  - Department of Endocrinology, 'C.I. Parhon' National Institute of Endocrinology, 
      011863 Bucharest, Romania.
LA  - eng
PT  - Journal Article
PT  - Review
DEP - 20220216
PL  - Greece
TA  - Exp Ther Med
JT  - Experimental and therapeutic medicine
JID - 101531947
PMC - PMC8908472
OTO - NOTNLM
OT  - Li-Fraumeni syndrome
OT  - Weiss score
OT  - adrenal cortex
OT  - adrenal tumor
OT  - adrenalectomy
OT  - adrenocortical carcinoma
OT  - cortisol
OT  - mitotane
OT  - pediatric
OT  - virilization
COIS- The authors declare that they have no competing interests.
EDAT- 2022/03/24 06:00
MHDA- 2022/03/24 06:01
PMCR- 2022/02/16
CRDT- 2022/03/23 05:14
PHST- 2021/10/25 00:00 [received]
PHST- 2021/11/24 00:00 [accepted]
PHST- 2022/03/23 05:14 [entrez]
PHST- 2022/03/24 06:00 [pubmed]
PHST- 2022/03/24 06:01 [medline]
PHST- 2022/02/16 00:00 [pmc-release]
AID - ETM-0-0-11216 [pii]
AID - 10.3892/etm.2022.11216 [doi]
PST - ppublish
SO  - Exp Ther Med. 2022 Apr;23(4):287. doi: 10.3892/etm.2022.11216. Epub 2022 Feb 16.