Ferrito Lucia, Cobellis Giovanni, Giobbi Diana, Pannunzi Cecilia Proietti, Iannilli Antonio, Cherubini Valentino
Azienda Ospedaliero Universitaria Ospedali Riuniti Ancona, Ancona, Italy.
Pediatric Surgery, Azienda Ospedaliero Universitaria Ospedali Riuniti Ancona, Ancona, Italy.
J Pediatr Adolesc Gynecol. 2017 Feb;30(1):e1-e4. doi: 10.1016/j.jpag.2016.09.006. Epub 2016 Sep 22.
Adrenocortical tumors (ACTs) represent less than 0.2% of all childhood neoplasms. Frequent clinical manifestations are virilization, hypercortisolism, and peripheral precocious puberty (PPP).
We describe two cases in which ACTs were responsible for virilization (case 1) and PPP (case 2) in prepubertal girls. In both cases an ACT diagnosis was made after 5-6 months from the first appearance of clinical signs. Surgery was performed within 1 month of diagnosis, and the benign nature of tumors was histologically confirmed. Despite complete tumor resection, virilizing features persisted.
Adrenocortical tumors should be considered early in the assessment of PPP. There is often a significant delay between the onset of symptoms and accurate diagnosis but early treatment is essential to limit the clinical manifestations of androgen overproduction.
肾上腺皮质肿瘤（ACTs）占所有儿童肿瘤的比例不到0.2%。常见的临床表现为男性化、皮质醇增多症和外周性性早熟（PPP）。
我们描述了两例肾上腺皮质肿瘤导致青春期前女孩男性化（病例1）和外周性性早熟（病例2）的病例。两例中，从临床症状首次出现到确诊肾上腺皮质肿瘤均经过了5 - 6个月。确诊后1个月内进行了手术，肿瘤的良性性质经组织学证实。尽管肿瘤已完全切除，但男性化特征仍持续存在。
在评估外周性性早熟时应尽早考虑肾上腺皮质肿瘤。症状出现与准确诊断之间往往有显著延迟，但早期治疗对于限制雄激素过度产生的临床表现至关重要。
