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Melanotic neuroectodermal tumor of infancy to the skull: case-based review
Florian Ebel1, Florian M Thieringer2,3,4, Christoph Kunz2,4
1Department of Neurosurgery, University Hospital of Basel, Basel, Switzerland. florianebel@hotmail.com.
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.
Insights
Melanotic neuroectodermal tumor of infancy (MNTI) is a rare condition in infants. Radical surgery is crucial for cure, but reconstruction in infants presents challenges.
Area of Science:
- Pediatric Oncology
- Craniofacial Surgery
- Molecular Pathology
Background:
- Melanotic neuroectodermal tumor of infancy (MNTI) is a rare neoplasm typically affecting infants under one year old.
- Early diagnosis and complete surgical resection are vital for achieving a long-term cure.
Observation:
- A case of a 4-month-old boy with a rapidly growing MNTI involving the skull base is presented.
- The tumor infiltrated the sphenoid wing, frontotemporal bone, orbit, and dura mater.
- Initial fine needle biopsy excluded sarcoma, leading to diagnosis.
Findings:
- Complete tumor excision was achieved, followed by cranial reconstruction using absorbable plates and Tutobone.
- Histology confirmed MNTI, with molecular analysis revealing high similarity to medulloblastoma group 3.
- A local recurrence necessitated chemotherapy and a second surgery, followed by management of pseudomeningocele and further reconstruction.
Implications:
- Radical surgery remains the gold standard for MNTI to minimize recurrence.
- Reconstruction following MNTI resection in infants is complex due to the tumor's destructive growth.
- This case highlights the challenges and management strategies for MNTI in infants, including surgical reconstruction and recurrence treatment.
