Styczewska Małgorzata, Krawczyk Małgorzata A, Brecht Ines B, Haug Konrad, Iżycka-Świeszewska Ewa, Godziński Jan, Raciborska Anna, Ussowicz Marek, Kukwa Wojciech, Cwalina Natalia, Lundstrom Emil, Bień Ewa
The English Division Pediatric Oncology Scientific Circle, Medical University of Gdansk, 80-210 Gdansk, Poland.
Department of Pediatrics, Hematology and Oncology, Medical University of Gdansk, 80-210 Gdansk, Poland.
Cancers (Basel). 2021 Jul 31;13(15):3872. doi: 10.3390/cancers13153872.
Melanotic Neuroectodermal Tumor of Infancy (MNTI) is a very rare pediatric neoplasm of neural crest origin. In most cases, it develops in infants as a localized tumor of the maxilla, and surgery is usually curative. In less than 10% of patients with inoperable, metastatic or persistently recurring MNTI, chemotherapy (CHT) may be considered; however, its role is still unclear. The aim of our study was to assess the efficacy of CHT in children with large, inoperable, metastatic and/or recurrent MNTI. Four such infants, treated with CHT in Polish and German centers of pediatric oncology, were presented. Additionally, a systematic literature search of the PubMed/MEDLINE, Scopus and Web of Science databases was performed, yielding 38 similar cases within the last 42 years. Neoadjuvant CHT, based mainly on the protocols for neuroblastoma, was often effective, allowing for complete delayed surgery in most cases. However, the role of adjuvant CHT in preventing recurrences after incomplete resection of MNTI remains unclear. Disseminated inoperable MNTI was almost universally associated with poor response to CHT and unfavorable outcome. Further investigations to elaborate standards of management in patients with inoperable, metastatic or persistently recurring MNTIs are necessary to improve outcomes.
婴儿黑色素性神经外胚层肿瘤（MNTI）是一种非常罕见的起源于神经嵴的儿科肿瘤。在大多数情况下，它在婴儿期表现为上颌骨的局限性肿瘤，手术通常可治愈。在不到10%的无法手术、发生转移或持续复发的MNTI患者中，可考虑化疗（CHT）；然而，其作用仍不明确。我们研究的目的是评估CHT对患有大型、无法手术、转移和/或复发MNTI的儿童的疗效。本文介绍了在波兰和德国儿科肿瘤中心接受CHT治疗的4例此类婴儿。此外，我们对PubMed/MEDLINE、Scopus和Web of Science数据库进行了系统的文献检索，在过去42年中发现了38例类似病例。主要基于神经母细胞瘤方案的新辅助CHT通常有效，在大多数情况下可实现延迟完全手术。然而，辅助CHT在预防MNTI不完全切除后复发中的作用仍不明确。播散性无法手术的MNTI几乎普遍与对CHT反应不佳和不良预后相关。有必要进一步开展研究，以制定针对无法手术、转移或持续复发MNTI患者的管理标准，从而改善治疗结果。
