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Inflammatory myofibroblastic tumor of the lung involving the central nervous system in pediatric patients

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Abstract

Introduction

The inflammatory myofibroblastic tumor (IMT) is a very rare lesion with an incidence of less than 0.1% of total neoplasms and with main affection in the lungs. Involvement in the central nervous system is extremely rare, but with a much more aggressive course than IMT diagnosed in the rest of the body. We report the 2 cases presented in our neurosurgery department to date; both were treated satisfactorily without intercurrences in 10 years of follow-up.

Historical background

The World Health Organization described the IMT as a distinctive lesion composed of myofibroblastic spindle cells accompanied by an inflammatory infiltrate of plasma cells, lymphocytes, and eosinophils.

Clinical presentation

Clinical manifestations of patients with CNS IMT vary and may consist of headache, vomiting, seizures, and blindness. Seizures are the most common symptom in patients with focal lesions.

Diagnosis

The true origin of this entity remains to be elucidated, but to date, etiologies ranging from chromosomal alterations to autoimmune or postinfectious mechanisms have been described. Due to its rarity and non-specificity in imaging, the final diagnosis of IMT in the brain parenchyma relies on pathological examination.

Management

Treatment options are controversial and include total or subtotal removal, high-dose steroids, and radiation therapy. In the last decade, the development of ALK Tyrosine Kinase Inhibitors allows the possibility of chemotherapy in those patients harboring ALK mutations.

Conclusion

IMT is a rare tumor that can exceptionally be found in the CNS. The cause is still unknown although the different studies focus on a neoplastic origin. The diagnosis is based in the use of different modalities of imaging and with histological confirmation. Optimal management is gross total resection whenever possible, is the only established curative treatment. Further research with longer follow-up is needed to clarify the natural history of this rare tumor.

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Data availability

The datasets generated during and/or analyzed during the current study are not publicly available due to the sensitive nature of the information and because the participants are underage but are available from the corresponding author on reasonable request.

Code availability

Not applicable.

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Authors and Affiliations

  1. Division of Pediatric Neurosurgery, Hospital de Pediatría Juan P. Garrahan, Combate de los Pozos 1881, ZC: 1245, Buenos Aires, Argentina

    Javier Gonzalez Ramos, Adalberto Ochoa, Santiago Cicutti & Ramiro del Rio

  2. Division of Pathology, Hospital de Pediatría Juan P. Garrahan, Buenos Aires, Argentina

    Fabiana Lubienieki

Authors
  1. Javier Gonzalez Ramos
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  2. Adalberto Ochoa
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  3. Santiago Cicutti
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  4. Ramiro del Rio
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  5. Fabiana Lubienieki
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Contributions

All authors contributed to the study conception and design. The bibliographic research, summary of the case, and edition of images were carried out by Adalberto Ochoa and Santiago Cicutti. The first draft of the manuscript was made by Javier Gonzalez Ramos and the rest of the authors commented and made contributions to it. All authors read and approved the final manuscript.

Corresponding author

Correspondence to Javier Gonzalez Ramos.

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Ethical approval

This study was performed in line with the principles of the Declaration of Helsinki. Approval was granted by the Ethics Committee of Hospital de Pediatría Juan P. Garrahan.

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Informed consent was obtained from the patient in this study.

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Patient signed informed consent regarding publishing their data and photographs.

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The authors declare no competing interests.

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Ramos, J.G., Ochoa, A., Cicutti, S. et al. Inflammatory myofibroblastic tumor of the lung involving the central nervous system in pediatric patients. Childs Nerv Syst 39, 1137–1145 (2023). https://doi.org/10.1007/s00381-023-05925-0

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