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Melanotic neuroectodermal tumor of infancy
Xiao Wu1, Samantha Shankar1, William R Munday2
1Department of Diagnostic Radiology, Yale School of Medicine, BOX 208042, Tompkins East 2, 333 Cedar Street, New Haven, CT 06520-8042, USA.
Abstract:
Melanotic neuroectodermal tumor of infancy (MNTI) is a rare pigmented craniofacial tumor of newborns and infants. We report the imaging findings of a 3-month old male patient with a maxillary MNTI. Detailed discussion on imaging features on various magnetic resonance sequences and CT scan are included. Characteristic radiographic appearance is also described. MNTI, of neural crest origin, display a biphasic population of melanin containing cells and neuroblastic cells, within a moderately vascularized fibrous stroma. The child underwent complete surgical excision with no evidence of recurrence at one year follow up. MNTI is an unusual tumor occurring in early childhood with a predilection for the maxilla. Clinical findings, CT scan and MRI may allow a preoperative diagnosis.
Insights
Melanotic neuroectodermal tumor of infancy (MNTI) is a rare craniofacial tumor in infants. Imaging findings from CT and MRI can aid in preoperative diagnosis of this neural crest tumor.
Area of Science:
- Pediatric Oncology
- Radiology
- Pathology
Background:
- Melanotic neuroectodermal tumor of infancy (MNTI) is a rare, pigmented craniofacial neoplasm.
- This tumor originates from neural crest cells and typically affects newborns and infants.
Observation:
- A case report details the imaging findings of a 3-month-old male infant with a maxillary MNTI.
- The report includes detailed discussions of MNTI's appearance on various MRI sequences and CT scans.
Findings:
- MNTI exhibits a characteristic radiographic appearance.
- Histologically, it shows a biphasic cell population comprising melanin-containing cells and neuroblastic cells within a vascularized stroma.
Implications:
- Accurate preoperative diagnosis of MNTI is possible using clinical findings, CT, and MRI.
- Complete surgical excision is the primary treatment, with no recurrence noted at one-year follow-up in this case.
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