Mandibular melanotic neuroectodermal tumor of infancy: a role for neoadjuvant chemotherapy.
Melanotic Neuroectodermal Tumor of Infancy (MNTI) is a rare, locally aggressive neoplasm with a predilection for the head and neck area, most commonly occurring in the maxilla. The vast majority of treatment modalities for all cases of MNTI to date have involved surgical intervention only, with just 9.6 % involving some sort of chemotherapy, radiotherapy, or a combination of the prior mentioned modalities. There is very limited information available regarding the use of neoadjuvant chemotherapy, due to its rare nature. In this report, a 4 month old girl presented to our clinic with a chief complaint of a large oral mass of about 2.5 months in duration. Intraoral examination showed an oral mass arising from the lingual aspect of inferior alveolar ridge with extensive mandibular invasion. The patient received three cycles of vincristine, Adriamycin, and cyclophosphamide as neodajuvant therapy. Upon completion, the tumor had decreased significantly in size. The patient was then scheduled for surgery and underwent surgical resection of the tumor. We were able to obtain adequate shrinkage of the tumor to allow better resectability, easier surgical access and a more minimally invasive approach with no lip split and a smaller neck incision. In conclusion, we have reported an extremely rare case of MNTI of the mandible that was successfully treated with neoadjuvant chemotherapy and surgical resection. This approach was advantageous to minimize the chance of recurrence and improve resectability in particularly large tumors, while maximizing functional outcomes and minimizing deformity.
Faculty of Medicine, American University of Beirut Medical Center, Beirut, Lebanon.
Christopher
C
Maroun
Department of Pathology and Laboratory Medicine, American University of Beirut Medical Center, Beirut, Lebanon.
Ibrahim
I
Khalifeh
Department of Otolaryngology Head and Neck Surgery, American University of Beirut Medical Center, 6th Floor, Hamra, Beirut, 1107 2020, Lebanon.
Elie
E
Alam
Department of Otolaryngology Head and Neck Surgery, American University of Beirut Medical Center, 6th Floor, Hamra, Beirut, 1107 2020, Lebanon.
Pierre Abi
PA
Akl
Department of Pediatrics and Adolescent Medicine, American University of Beirut Medical Center, Beirut, Lebanon.
Raya
R
Saab
Department of Otolaryngology Head and Neck Surgery, American University of Beirut Medical Center, 6th Floor, Hamra, Beirut, 1107 2020, Lebanon. rm17@aub.edu.lb.
Roger V
RV
Moukarbel
D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use*; D003520:Cyclophosphamide / Q000008:administration & dosage; D004317:Doxorubicin / Q000008:administration & dosage; D005260:Female; D006801:Humans; D007223:Infant; D008339:Mandibular Neoplasms / Q000473:pathology / Q000628:therapy*; D020360:Neoadjuvant Therapy; D017600:Neuroectodermal Tumor, Melanotic / Q000473:pathology / Q000628:therapy*; D035583:Rare Diseases; D014750:Vincristine / Q000008:administration & dosage
D014750:Vincristine; D004317:Doxorubicin; D003520:Cyclophosphamide
MNTI; Mandible; Neoadjuvant chemotherapy; Neuroectodermal tumor