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Melanotic neuroectodermal tumour of infancy: a rare brain tumour of childhood
Muhammad Babar Khan1, Delvene Soares, Muhammad Zubair Tahir
1The Aga Khan University, Karachi.
Insights
Melanotic neuroectodermal tumour of infancy, a rare neural crest tumor, can occur in the skull. This case highlights successful surgical removal of a rare infant skull tumor with no recurrence.
Area of Science:
- Pediatric Oncology
- Neurosurgery
- Pathology
Background:
- Melanotic neuroectodermal tumour of infancy (NMTI) is a rare neoplasm of neural crest origin.
- While typically affecting the maxilla, cranial and intracranial NMTI are exceptionally uncommon.
Observation:
- An 8-month-old infant presented with a 6-month history of a scalp swelling.
- CT imaging revealed an osteolytic lesion in the anterior parasagittal skull with dural invasion.
Findings:
- The tumor was surgically excised in an en bloc fashion.
- Histopathological analysis confirmed the diagnosis of melanotic neuroectodermal tumour of infancy.
Implications:
- This case demonstrates that NMTI, though rare in the skull, can be successfully treated with complete surgical resection.
- Long-term follow-up is crucial for monitoring potential recurrence in these rare pediatric neoplasms.
Abstract:
Melanotic neuroectodermal tumour of infancy is a rare, mostly benign but locally aggressive tumour of neural crest cell origin occurring in infants. The most commonly affected anatomic site is the maxilla. Such tumours of the brain and skull are very rare. We present the case of an 8 months old baby girl whose presenting complaint was a swelling in the scalp for 6 months. She was otherwise asymptomatic. CT imaging confirmed the presence of an osteolytic tumour in the anterior parasagittal skull with dural involvement. The tumour was surgically excised enbloc. The patient has been well at 2 years follow-up without any evidence of recurrence.
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