{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,8]],"date-time":"2026-07-08T05:59:12Z","timestamp":1783490352562,"version":"3.55.0"},"reference-count":33,"publisher":"Walter de Gruyter GmbH","issue":"1","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2022,1,27]]},"abstract":"<jats:title>Abstract<\/jats:title>\n               <jats:sec id=\"j_jpem-2021-0229_abs_001\">\n                  <jats:title\/>\n                  <jats:p>Pediatric adrenocortical tumors are rare and heterogeneous endocrine malignancies. <\/jats:p>\n               <\/jats:sec>\n               <jats:sec id=\"j_jpem-2021-0392_abs_002\">\n                  <jats:title>Objectives<\/jats:title>\n                  <jats:p>To report clinical, biochemical, and histological features, staging, and therapeutic interventions in a cohort of 28 patients treated at a single tertiary center.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec id=\"j_jpem-2021-0392_abs_003\">\n                  <jats:title>Methods<\/jats:title>\n                  <jats:p>A retrospective review of medical records of children with PACT (diagnosed before &lt;18 years of age) followed between 1987\u20132018 at Hospital de Pediatr\u00eda Garrahan, Buenos Aires, Argentina.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec id=\"j_jpem-2021-0392_abs_004\">\n                  <jats:title>Results<\/jats:title>\n                  <jats:p>Mean age at diagnosis was 4.6 years (range, 0.3\u201317.3 years) and median follow-up was 4.17 years (range, 0\u201312 years). Female to male ratio was 2.5:1. Signs and symptoms that prompted medical intervention were hormonal overproduction (57%), abdominal complaints (36%), and hypertensive encephalopathy (7%). In patients with clinically virilizing tumors (n=16) mean height standard deviation score (SDS) and bone age advance were significantly higher while body mass index (BMI) SDS was significantly lower than in those with clinical Cushing\u2019s (n=10) (p&lt;0.05). Serum dehydroepiandrosterone sulfate (DHEAS) levels were significantly higher in stage IV than in stage I (p=0.03). Total adrenalectomy was performed in 26 patients. Eight patients (stage III-IV) received adjuvant chemotherapy. Five-year overall and disease-free survival were 100% for ST I-II, and 51% (95%\u00a0CI 21\u201382) and 33% (95% CI 1.2\u201365) for ST III-IV, respectively (p=0.002). No statistical difference was found when comparing 2-year parameters with and without adjuvant chemotherapy.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec id=\"j_jpem-2021-0392_abs_005\">\n                  <jats:title>Conclusions<\/jats:title>\n                  <jats:p>Height SDS and BMI SDS seem to mirror hormonal secretion in pediatric adrenocortical tumors. Higher DHEAS levels were found in patients with more advanced disease. Further large-scale studies are needed to validate a possible role for DHEAS as a biochemical marker of tumor stage and to draw robust conclusions on the use of adjuvant chemotherapy.<\/jats:p>\n               <\/jats:sec>","DOI":"10.1515\/jpem-2021-0392","type":"journal-article","created":{"date-parts":[[2021,10,22]],"date-time":"2021-10-22T03:29:47Z","timestamp":1634873387000},"page":"19-27","source":"Crossref","is-referenced-by-count":5,"title":["Pediatric adrenocortical tumors cohort characteristics and long-term follow-up at a single Argentinian tertiary center"],"prefix":"10.1515","volume":"35","author":[{"given":"Mar\u00eda Celeste","family":"Mattone","sequence":"first","affiliation":[{"name":"Hospital de Pediatr\u00eda Garrahan , Buenos Aires , Argentina"},{"name":"Consejo Nacional de Investigaciones Cient\u00edficas y T\u00e9cnicas (CONICET) , Buenos Aires , Argentina"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Silvia","family":"Gil","sequence":"additional","affiliation":[{"name":"Hospital de Pediatr\u00eda Garrahan , Buenos Aires , Argentina"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mariana","family":"Costanzo","sequence":"additional","affiliation":[{"name":"Hospital de Pediatr\u00eda Garrahan , Buenos Aires , Argentina"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8609-6856","authenticated-orcid":false,"given":"Mar\u00eda Laura","family":"Galluzzo Mutti","sequence":"additional","affiliation":[{"name":"Hospital de Pediatr\u00eda Garrahan , Buenos Aires , Argentina"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alejandra","family":"Casanovas","sequence":"additional","affiliation":[{"name":"Hospital de Pediatr\u00eda Garrahan , Buenos Aires , Argentina"},{"name":"Instituto Nacional del C\u00e1ncer , Buenos Aires , Argentina"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ver\u00f3nica","family":"Zaidman","sequence":"additional","affiliation":[{"name":"Hospital de Pediatr\u00eda Garrahan , Buenos Aires , Argentina"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Juan Manuel","family":"Lazzati","sequence":"additional","affiliation":[{"name":"Hospital de Pediatr\u00eda Garrahan , Buenos Aires , Argentina"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marta","family":"Ciaccio","sequence":"additional","affiliation":[{"name":"Hospital de Pediatr\u00eda Garrahan , Buenos Aires , Argentina"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alicia","family":"Belgorosky","sequence":"additional","affiliation":[{"name":"Hospital de Pediatr\u00eda Garrahan , Buenos Aires , Argentina"},{"name":"Consejo Nacional de Investigaciones Cient\u00edficas y T\u00e9cnicas (CONICET) , Buenos Aires , Argentina"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3122-6734","authenticated-orcid":false,"given":"Gabriela","family":"Guercio","sequence":"additional","affiliation":[{"name":"Hospital de Pediatr\u00eda Garrahan , Buenos Aires , Argentina"},{"name":"Consejo Nacional de Investigaciones Cient\u00edficas y T\u00e9cnicas (CONICET) , Buenos Aires , Argentina"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"374","published-online":{"date-parts":[[2021,10,22]]},"reference":[{"key":"2023040100325814649_j_jpem-2021-0392_ref_001","doi-asserted-by":"crossref","unstructured":"McAteer, JP, Huaco, JA, Gow, KW. 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