Melanotic neuroectodermal tumour of infancy with unexpected peri-operative complications

Abstract

Melanotic neuroectodermal tumour of infancy (MNTI) is a rare rapidly growing, expansile, locally aggressive pigmented neoplasm of neural crest origin and usually encountered in children below 1 year of age in the anterior maxillary region. MNTI is documented as benign neoplasm, but occasional tumours exhibit malignant behaviour with lymph nodal metastasis. Although most of these cases are treated successfully, there is a chance of recurrence in tumours without clear margin which varies from 10% to 50%. In isolated cases, death occurs due to advanced stage of the disease with recurrence and malignant transformation. Here, we report the rare case of MNTI of the left maxilla diagnosed in a 5-month-old boy basing on cytological and histopathological features and subsequently confirmed by immunohistochemical findings. The unexpected perioperative complications encountered in this case in the form of difficulty in airway access, post-surgery pulmonary complications, multi- organ dysfunction, sepsis and other related issues are discussed here which have never been documented in any literature.

Keywords

• melanotic neuroectodermal tumour of infancy
• peri-operative complications
• pigmented neoplasm
• progonoma