Umano Giuseppina Rosaria, Errico Maria Elena, D'Onofrio Vittoria, Delehaye Giulia, Trotta Letizia, Spinelli Claudio, Strambi Silvia, Franco Renato, D'Abbronzo Giuseppe, Ronchi Andrea, Papparella Alfonso
Department of Woman, Child and General and Specialized Surgery, University of Campania "Luigi Vanvitelli", Naples, Italy.
Department of Pathology, Azienda Ospedaliera di Rilievo Nazionale (AORN) Santobono Pausilipon, Pediatric Hospital, Naples, Italy.
Front Oncol. 2021 Jun 14;11:688410. doi: 10.3389/fonc.2021.688410. eCollection 2021.
Pediatric melanoma is a rare disease especially in children aged younger than 10 years old. Recent estimates report a rise of disease incidence in both adults and children. Diagnostic work-up is challenging in pediatric melanoma, as it displays a wide range of clinical presentations. Immunohistochemical biomarkers have been reported as predictors of malignancy in melanoma, however data specific to pediatric melanoma are poor. Our study aims to contribute to provide evidence of pediatric melanoma clinical features and differential diagnosis in this patient population. We describe our experience with a retrospective case series of pigmented skin lesions including malignant melanoma, atypical spitzoid tumor, and benign nevi in children and adolescents aged less than 16 years. We described the clinical and demographic characteristics of the cohort and evaluated the immunohistochemical expression of the PReferentially expressed Antigen in MElanoma (PRAME) for differential diagnosis of melanoma in children. The series displayed a similar distribution of melanoma between males and females, and the most common site of melanoma onset were the upper and lower limbs. In our cohort, PRAME was negative in most cases. Focal and slight positivity (from 1 to 5% of the neoplastic cells) was observed in four cases (two Spitz nevi and two atypical Spitz tumors). A moderate positivity in 25% of the neoplastic cells was observed in one case of atypical Spitz tumor. Immunohistochemical expression of PRAME might be useful in the differential diagnosis of malignant melanoma.
小儿黑色素瘤是一种罕见疾病，尤其在10岁以下儿童中更为少见。最近的估计报告显示，成人和儿童的疾病发病率均有所上升。小儿黑色素瘤的诊断检查具有挑战性，因为其临床表现多种多样。免疫组织化学生物标志物已被报道为黑色素瘤恶性程度的预测指标，然而，小儿黑色素瘤的相关数据却很匮乏。我们的研究旨在为该患者群体提供小儿黑色素瘤临床特征及鉴别诊断的证据。我们描述了对16岁以下儿童和青少年色素沉着性皮肤病变（包括恶性黑色素瘤、非典型斯皮茨样肿瘤和良性痣）的回顾性病例系列研究经验。我们描述了该队列的临床和人口统计学特征，并评估了黑色素瘤优先表达抗原（PRAME）的免疫组织化学表达，以用于儿童黑色素瘤的鉴别诊断。该系列研究显示，黑色素瘤在男性和女性中的分布相似，黑色素瘤最常见的发病部位是上肢和下肢。在我们的队列中，大多数病例的PRAME呈阴性。在4例病例（2例斯皮茨痣和2例非典型斯皮茨肿瘤）中观察到局灶性和轻度阳性（肿瘤细胞的1%至5%）。在1例非典型斯皮茨肿瘤中，观察到25%的肿瘤细胞呈中度阳性。PRAME的免疫组织化学表达可能有助于恶性黑色素瘤的鉴别诊断。
