Melanotic neuroectodermal tumour of infancy (progonoma) treated by radical maxillary surgery

Melanotic neuroectodermal tumor of infancy: a rare presentation of an extremely rare neoplasm and diagnostic implications in Gombe, Nigeria

“…The trend is also baffling because the child was birthed in a hospital, albeit a village cottage one. One would expect that with onset at birth, and with the prominent site of occurrence-the anterior maxilla, similar to most literature that report that 70% of cases occur here [ 1 , 14 ], the wait would be shorter. Like most reported cases of MNTI, our patient presented with a painless swelling.…”

“…Ultimately, the bluish-blackish granules revealed by the labial incision in the excisional biopsy confirmed MNTI as the working diagnosis. Prescribed treatment includes radical surgery [ 3 , 6 , 14 ] and wide surgical excision with ostectomy [ 1 ] for new lesions. Radical surgery with adjuvant treatment is suggested for recurrent lesions [ 3 , 17 ].…”

“…Carnevale et al [ 18 ] even suggested the use of operating microscopes during surgical excision to remove unseen remnants of the pigmented lesion to prevent its recurrence. Recurrence is also reported to be commoner amongst younger patients [ 14 , 17 ] although this may be because it is easier to be more radical when there is more tissue to manipulate, as would naturally occur in older patients. We decided on less radical surgery in this case and performed a peripheral ostectomy of 5mm with the surgical excision.…”

Melanotic neuroectodermal tumor of infancy: A rare case report with differential diagnosis and review of the literature

“…Oral and maxillofacial tumors rarely occur in the pediatric population compared with the adult population. [ 11 ] Most jaw swellings that occur in the infants are usually benign odontogenic cysts or tumors. [ 11 ] However, some lesions although benign tend to have an alarming growth rate.…”

“…[ 11 ] Most jaw swellings that occur in the infants are usually benign odontogenic cysts or tumors. [ 11 ] However, some lesions although benign tend to have an alarming growth rate. Therefore, there is a possibility of misdiagnosing them clinically as malignant lesions.…”