Objective: To present a case of melanotic neuroectodermal tumor of infancy (MNTI), its clinical course and treatment.
Case : A 4-month-old female, Filipino, was referred due to an expansive growth of the maxilla. Born term to a 39 year old G7P7 (7007) via normal spontaneous delivery. Swelling was first noticed at age 1 ½ month and was diagnosed as an oral abscess at their local health center. At 2 months of age, increase of the mass was noted prompting consult to a tertiary hospital. Water’s view test was done revealing a soft tumor swelling in the right mallar area. Ct scan result showed a heterogeneously enhancing soft tissue mass in the alveolar process of the right maxillary bone with destruction of the adjacent maxillary bone. Incision biopsy was performed. Gross specimen comprised of a firm, tan-brown mass measuring 2.5 x1.7 x 0.2 cm. Diagnosis at this point was mucoepidermoid carcinoma. Patient was then transferred in this institution. Clinical examination revealed a firm, non-ulcerated tumor, about 9x9 cm on the anterior region of the maxilla protruding through the lips, covered by an intact mucosa. Microscopically, the tumor composed of nests of neoplastic cells, arranged in an alveolar pattern separated by fibrovascular stroma. The tumor cell population is biphasic, showing consistent features of MNTI. Urine VMA was elevated and patient was positive for hmb-45, desmin and CD-99. Hence, a final diagnosis of MNTI was given. Patient was referred to oncology department and was subsequently discharged after chemotherapy.
Conclusion: it is noteworthy that swellings in the maxillary area, which could resemble an abscess, can cause unnecessary treatments and delay in diagnosing MNTI. Its rarity and unique features make diagnosis and management challenging. Hence, patients must be considered cautiously, multidisciplinary approach must be used, leading to successful diagnosis and treatment.