Piperi Evangelia P, Rake Scott A, Tosios Konstantinos I, Vasilopoulou Evdokia E, Rake Angela P, Sandler Noah A, Issacson Tim, Sklavounou Alexandra, Koutlas Ioannis G
Department of Oral Medicine and Pathology, Dental School, National and Kapodistrian University of Athens, Athens, Greece.
J Craniofac Surg. 2010 May;21(3):685-8. doi: 10.1097/SCS.0b013e3181d7f0c5.
Melanotic neuroectodermal tumor of infancy (MNTI) is an uncommon, rapidly growing neoplasm of neural crest origin that primarily develops in the maxilla of infants during their first year of life. Mandibular lesions are rare and account for about 6% of all cases. Radical surgical excision is usually curative, but patients should be followed up closely because recurrence may occur in approximately 10% to 20% of cases. In this study, we report a case of mandibular MNTI in a 4-month-old male patient that was conservatively treated with enucleation and curettage and has shown no recurrence 11 years after surgery. Review of the English-language literature revealed that of more than 350 cases of MNTI reported so far, only 23, including the present one, have been encountered in the mandible. Most patients (91.3%) were younger than 1 year, whereas the male-to-female ratio was 1.3:1. Most lesions were treated with wide surgical excision, with only 2 cases being conservatively treated with enucleation. Recurrence was noted in 36.8% of cases at less than 3 months postoperatively. In conclusion, MNTI lesions in the mandible, albeit rare, show high recurrence rate. However, small-size mandibular MNTI lesions may be successfully treated with conservative enucleation. Close follow-up is highly recommended, in particular during the first 6 postoperative months.
婴儿黑色素性神经外胚层肿瘤（MNTI）是一种罕见的、生长迅速的神经嵴源性肿瘤，主要发生于1岁以内婴儿的上颌骨。下颌骨病变罕见，约占所有病例的6%。根治性手术切除通常可治愈，但患者应密切随访，因为约10%至20%的病例可能复发。在本研究中，我们报告了1例4个月大男性下颌骨MNTI病例，该病例采用摘除术和刮除术进行保守治疗，术后11年未复发。回顾英文文献发现，在迄今报道的350多例MNTI病例中，只有23例（包括本病例）发生于下颌骨。大多数患者（91.3%）年龄小于1岁，男女比例为1.3:1。大多数病变采用广泛手术切除治疗，只有2例采用摘除术进行保守治疗。36.8%的病例在术后不到3个月出现复发。总之，下颌骨MNTI病变虽罕见，但复发率高。然而，小型下颌骨MNTI病变可通过保守摘除术成功治疗。强烈建议密切随访，尤其是术后前6个月。
