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Melanotic neuroectodermal tumor of infancy: a wolf in sheep's clothing
Joel M Davis1, Marianna DeBenedictis2, Douglas K Frank3
1Division of Oral and Maxillofacial Surgery, Hofstra North-Shore Long Island Jewish Health System, New Hyde Park, New York, USA jdavis2009@dents.uwo.ca.
Introduction:
The melanotic neuroectodermal tumor of infancy (MNTI) is an exceptionally rare neoplasm. Despite their potential for local invasion, MNTI are considered benign neoplasms. Sporadic cases have been reported of MNTI acting in a malignant fashion; however, the majority of these tumors were of extragnathic origin.
Methods:
A 2-month-old male presented with a rapidly expanding maxillary mass. The patient underwent excisional biopsy of the mass and a diagnosis of MNTI was returned. Tumor recurrence was appreciated postoperatively. Neoadjuvant chemotherapy was initiated to render it more amenable to surgical resection. Chemotherapy was suspended prematurely and the patient returned to the operating room for hemimaxillectomy. The postchemotherapy surgical pathology report demonstrated a unique mixed chemotherapy response.
Discussion:
This report highlights the aggressive nature of gnathic MNTI, the importance of early diagnosis and definitive intervention, and the mixed clinical and histologic response of the lesion to neoadjuvant chemotherapy.
Insights
Melanotic neuroectodermal tumor of infancy (MNTI) can exhibit aggressive behavior in the jaw, even when initially considered benign. Early diagnosis and intervention are crucial for effective management of this rare condition.
Area of Science:
- Pediatric oncology
- Oral pathology
- Neoplasia research
Background:
- Melanotic neuroectodermal tumor of infancy (MNTI) is a rare neoplasm, typically benign but with potential for local invasion.
- Extragnathic MNTI have rarely shown malignant behavior.
- Gnathic MNTI, originating in the jaw, present unique clinical challenges.
Observation:
- A 2-month-old male presented with a rapidly growing maxillary mass.
- Initial diagnosis was MNTI, with postoperative recurrence noted.
- Neoadjuvant chemotherapy was administered, followed by hemimaxillectomy due to premature suspension of treatment.
Findings:
- The gnathic MNTI demonstrated an aggressive clinical course.
- A unique mixed response to neoadjuvant chemotherapy was observed histologically.
- Complete surgical resection via hemimaxillectomy was performed.
Implications:
- This case underscores the aggressive potential of gnathic MNTI.
- Highlights the critical need for prompt diagnosis and definitive treatment.
- Suggests a complex and variable response of MNTI to neoadjuvant chemotherapy.
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