Updating Wieneke (AFIP) scoring systems in pediatric ACC
Andrey Garcia Estevanato, MD, Heitor Ramos Ruellas, MD, Livia Lara Mermejo, MD PhD, Fernando Chahud, MD PhD, Sonir Roberto Antonini, MD PhD, Valdair Francisco Muglia, MD PhD, Silvio Tucci, Jr., MD PhD, Jose De Bessa Junior, MD PhD, Carlos Augusto Fernandes Molina, MD PhD.
Faculdade de Medicina de Ribeirão Preto Universidade de São Paulo, Ribeirão Preto / São Paulo, Brazil.
BACKGROUND: Pediatric adrenal cortical carcinoma (ACCp) is a malignant and extremely rare tumor, but with a higher incidence in Brazil than in other countries(1). The histological diagnosis of adrenal cortical tumors (ACT) still finds it difficult to define the malignant/benign clinical behavior of these tumors(2). The Wieneke criteria, also called Armed Forces Institute of Pathology (AFIP®), is the best one for the pediatric population comparing to Weiss scoring system used for adults(3). However AFIP did not reach 100% sensitivity for defining malignant clinical behavior in one of the largest patients series as recently published(4). The weight (> 400g) and size (>10.5cm) criteria used by AFIP to define the malignant/benign clinical behavior of ACT may be good for kids/adolescents, but may also be huge for toddlers, specially, infants. The aim is to adjust the weight and size threshold of the Wieneke criteria (AFIP) to improve its sensitivity.
Methods: Retrieve medical records of 55 pediatric population treated at the Hospital das Clinicas of Ribeirao Preto Medical School, University of São Paulo, over 35 years (1975-2010), with a minimum postoperative follow-up of 12 months. To determine the malignant/benign clinical behavior of the TACs, the samples was processed and reviewed by two experienced pathologists considering the Wieneke criteria (AFIP) for pediatric ACT. After that, weight (> 400g) and size (> 10.5cm) criteria were adjusted to ≥ 250g and ≥ 7cm and the sensitivity of the method was checked again.
Results: Most patients were female, 38 (69.1%) and infants/toddlers/kids, 50 (90.9%), with a median age of the sample of 3 [1-5] years. Regarding the 55 patients, 16 (29.1%) presented malignant clinical behavior, and in 15 (93.8%) Wieneke criteria identified it. The remaining 39 patients with benign clinical behavior, Wieneke identified it in 28 (71.8%). In adjusting the weight to ≥ 250 g and to ≥ 7 cm, and considering the score of ≥ 4 as proposed by Wieneke scoring for malignancy, all 16 patients who presented malignant clinical behavior were identified by it, improving the sensitivity of the method to 100% in the present sample.
Conclusions: Adjusting weight and size criteria improved the sensitivity of Wieneke’s histopathological criteria (AFIP) from 93.8% to 100% in identifying pediatric ACT with malignant clinical behavior (ACCp).
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