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Systematic Review

The Role of Chemotherapy in Management of Inoperable, Metastatic and/or Recurrent Melanotic Neuroectodermal Tumor of Infancy—Own Experience and Systematic Review

1
The English Division Pediatric Oncology Scientific Circle, Medical University of Gdansk, 80-210 Gdansk, Poland
2
Department of Pediatrics, Hematology and Oncology, Medical University of Gdansk, 80-210 Gdansk, Poland
3
Department of Pediatric Hematology and Oncology, University of Tübingen, 72076 Tübingen, Germany
4
Department of Pathology and Neuropathology, Medical University of Gdansk, 80-210 Gdansk, Poland
5
Department of Pediatric Surgery, Marciniak Hospital, 54-049 Wroclaw, Poland
6
Department of Pediatric Traumatology and Emergency Medicine, Wroclaw Medical University, 50-345 Wroclaw, Poland
7
Department of Oncology and Surgical Oncology for Children and Youth, Institute of Mother and Child, 01-211 Warsaw, Poland
8
Department of Pediatric Hematology, Oncology and Bone Marrow Transplantation, Wroclaw Medical University, 50-556 Wroclaw, Poland
9
Department of Otorhinolaryngology, Faculty of Medicine and Dentistry, Medical University of Warsaw, 00-739 Warsaw, Poland
*
Authors to whom correspondence should be addressed.
The authors M.S. and M.A.K. contributed equally to the study.
Academic Editors: Saurabh Agarwal and Jianhua Yang
Cancers 2021, 13(15), 3872; https://doi.org/10.3390/cancers13153872
Received: 31 May 2021 / Revised: 19 July 2021 / Accepted: 28 July 2021 / Published: 31 July 2021
(This article belongs to the Special Issue Pediatric Cancers)
Melanotic Neuroectodermal Tumor of Infancy (MNTI) is a very rare neoplasm that most commonly develops within maxilla in infants. It usually has a benign clinical course and is treated with only surgery. However, patients with large, inoperable, metastatic or multiply recurring MNTI may require systemic treatment. The role of pre- and post-surgery chemotherapy (CHT) in the management of MNTI is unclear. Here, we have presented the disease courses and outcomes of four infants treated with multidrug CHT due to inoperable/recurrent MNTI. Additionally, a systematic literature review was performed which revealed 38 similar cases in the last 42 years. Most children with primarily inoperable MNTI responded to CHT, which allowed physicians to perform complete, non-mutilating delayed surgery. However, it is still uncertain whether CHT administered after incomplete resection of MNTI prevents recurrence. This study aimed to contribute to the establishment of standards of management in patients with inoperable, metastatic or persistently recurring MNTIs, which are currently lacking.
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. View Full-Text
Keywords: melanotic neuroectodermal tumor of infancy; inoperable; metastatic; recurrent; chemotherapy; adjuvant; neoadjuvant; systemic treatment melanotic neuroectodermal tumor of infancy; inoperable; metastatic; recurrent; chemotherapy; adjuvant; neoadjuvant; systemic treatment
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MDPI and ACS Style

Styczewska, M.; Krawczyk, M.A.; Brecht, I.B.; Haug, K.; Iżycka-Świeszewska, E.; Godziński, J.; Raciborska, A.; Ussowicz, M.; Kukwa, W.; Cwalina, N.; Lundstrom, E.; Bień, E. The Role of Chemotherapy in Management of Inoperable, Metastatic and/or Recurrent Melanotic Neuroectodermal Tumor of Infancy—Own Experience and Systematic Review. Cancers 2021, 13, 3872. https://doi.org/10.3390/cancers13153872

AMA Style

Styczewska M, Krawczyk MA, Brecht IB, Haug K, Iżycka-Świeszewska E, Godziński J, Raciborska A, Ussowicz M, Kukwa W, Cwalina N, Lundstrom E, Bień E. The Role of Chemotherapy in Management of Inoperable, Metastatic and/or Recurrent Melanotic Neuroectodermal Tumor of Infancy—Own Experience and Systematic Review. Cancers. 2021; 13(15):3872. https://doi.org/10.3390/cancers13153872

Chicago/Turabian Style

Styczewska, Małgorzata, Małgorzata A. Krawczyk, Ines B. Brecht, Konrad Haug, Ewa Iżycka-Świeszewska, Jan Godziński, Anna Raciborska, Marek Ussowicz, Wojciech Kukwa, Natalia Cwalina, Emil Lundstrom, and Ewa Bień. 2021. "The Role of Chemotherapy in Management of Inoperable, Metastatic and/or Recurrent Melanotic Neuroectodermal Tumor of Infancy—Own Experience and Systematic Review" Cancers 13, no. 15: 3872. https://doi.org/10.3390/cancers13153872

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