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The Role of Chemotherapy in Management of Inoperable, Metastatic and/or Recurrent Melanotic Neuroectodermal Tumor of Infancy-Own Experience and Systematic Review
- Source :
- Cancers, Cancers, Vol 13, Iss 3872, p 3872 (2021)
- Publication Year :
- 2021
-
Abstract
- Simple Summary 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. Abstract 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.
- Subjects :
- Cancer Research
medicine.medical_specialty
medicine.medical_treatment
MEDLINE
chemotherapy
03 medical and health sciences
0302 clinical medicine
recurrent
adjuvant
Neuroblastoma
melanotic neuroectodermal tumor of infancy
medicine
Pediatric oncology
Delayed surgery
In patient
RC254-282
Chemotherapy
business.industry
Melanotic neuroectodermal tumor of infancy
neoadjuvant
Neoplasms. Tumors. Oncology. Including cancer and carcinogens
systemic treatment
medicine.disease
metastatic
Oncology
030220 oncology & carcinogenesis
Pediatric Neoplasm
Radiology
Systematic Review
inoperable
business
030217 neurology & neurosurgery
Subjects
Details
- ISSN :
- 20726694
- Volume :
- 13
- Issue :
- 15
- Database :
- OpenAIRE
- Journal :
- Cancers
- Accession number :
- edsair.doi.dedup.....24c435fcf988645fb36ab4e984801db9