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Melanotic neuroectodermal tumor of infancy to the skull: case-based review

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Abstract

Background

Melanotic neuroectodermal tumor of infancy (MNTI) is a rare tumor, which usually occurs in infants under the age of one. Early diagnosis and radical surgery seem to be critical for long-term cure.

Case presentation

We describe a case of a 4-month-old boy with a MNTI to the skull. The mass was first noticed at 4 month of age and grew very rapidly over a time of 2 weeks. Initially, a fine needle biopsy ruled out a sarcoma and led to the diagnosis. The tumor originated from the sphenoid wing and infiltrated the frontotemporal bone, the lateral wall of the right orbit, and the underlying dura mater. A total excision of the tumor, including the adjacent bone and dura, was achieved. Reconstruction of the bone was performed using absorbable plates and Tutobone. Histology confirmed the initial diagnosis, while molecular diagnosis showed high conformity of the MNTI with medulloblastoma group 3. The patient recovered well, while the reconstruction led to a good cosmetic result. A local recurrence occurred leading to a single-dose chemotherapy with Vincristine and a second surgery after 15 weeks. Thereafter, the patient developed recurrent large pseudomeningocele, which was treated by multiple shunt procedures and finally reconstruction of the bone using Palacos. Radiological follow-up 3 months after the second resection showed no tumor recurrence.

Conclusion

Radical surgery for MNTI is to date the gold standard since it seems to minimize recurrence rates. Because of the rapid and destructive growth within the bone, reconstruction is necessary, which can be very challenging in infants.

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Authors and Affiliations

  1. Department of Neurosurgery, University Hospital of Basel, Basel, Switzerland

    Florian Ebel, Raphael Guzman & Jehuda Soleman

  2. Department of Oral & Cranio-Maxillo-Facial Surgery, University Hospital of Basel, Basel, Switzerland

    Florian M. Thieringer & Christoph Kunz

  3. Medical Additive Manufacturing Research Group, Department of Biomedical Engineering, University of Basel, Allschwil, Switzerland

    Florian M. Thieringer

  4. Department of Pediatrics, McMaster Children’s Hospital and McMaster University, Hamilton, Canada

    Katrin Scheinemann

  5. Division of Pediatric Oncology, Department of Pediatrics, Kantonsspital Aarau, Aarau, Switzerland

    Andreas Klein-Franke & Katrin Scheinemann

  6. Division of Pediatric Oncology, University Children’s Hospital of Basel and University of Basel, Basel, Switzerland

    Katrin Scheinemann

  7. Division of Pediatric Neurosurgery, University Children’s Hospital of Basel, Basel, Switzerland

    Raphael Guzman & Jehuda Soleman

  8. Faculty of Medicine, University of Basel, Basel, Switzerland

    Florian M. Thieringer, Christoph Kunz, Raphael Guzman & Jehuda Soleman

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  1. Florian Ebel
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Ebel, F., Thieringer, F.M., Kunz, C. et al. Melanotic neuroectodermal tumor of infancy to the skull: case-based review. Childs Nerv Syst 36, 679–688 (2020). https://doi.org/10.1007/s00381-020-04509-6

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