Skip to main content

Advertisement

SpringerLink
Log in

Mandibular melanotic neuroectodermal tumor of infancy: a role for neoadjuvant chemotherapy

  • Case Report
  • Published:
European Archives of Oto-Rhino-Laryngology Aims and scope Submit manuscript

    We’re sorry, something doesn't seem to be working properly.

    Please try refreshing the page. If that doesn't work, please contact support so we can address the problem.

Abstract

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.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Fig. 1
Fig. 2
Fig. 3

References

  1. Lurie HI (1961) Congenital melanocarcinoma, melanotic adamantinoma, retinal anlage tumor, progonoma, and pigmented epulis of infancy. Cancer 14(5):1090–1108

    Article  CAS  PubMed  Google Scholar 

  2. Cui Y, Mao Z, Liao C (2014) Melanotic neuroectodermal tumor of infancy: a case report and review of the surgical treatment. Oncol Lett 9:29–34

    PubMed  PubMed Central  Google Scholar 

  3. Rachidi S, Sood AJ, Patel KG, Nguyen SA, Hamilton H, Neville BW, Day TA (2015) Melanotic neuroectodermal tumor of infancy: a systematic review. J Oral Maxil Surg 73(10):1946–1956. doi:10.1016/j.joms.2015.03.061

    Article  Google Scholar 

  4. Rekhi B, Suryavanshi P, Desai S, Gulia A, Desai S, Juvekar SL, Puri A, Jambhekar NA (2011) Melanotic neuroectodermal tumor of infancy in thigh of an infant—a rare case report with diagnostic implications. Skeletal Radiol 40:1079–1084

    Article  PubMed  Google Scholar 

  5. Omodaka S, Saito R, Kumabe T, Kawagishi J, Jokura H, Sonoda Y, Watanabe M, Kayama T, Tominaga T (2010) Melanotic neuroectodermal tumor of the brain recurring 12 years after complete remission: case report. Brain Tumor Pathol 27:51–57

    Article  PubMed  Google Scholar 

  6. Hameed K, Burslem MRG (1970) A melanotic ovarian neoplasm resembling the ‘retinal anlage’ tumor. Cancer 25:564–567

    Article  CAS  PubMed  Google Scholar 

  7. Desai S, Roy S, Borges AM (2005) Recurrent melanotic neuroectodermal tumour of infancy of the epididymis and testis: a case report. Indian J Pathol Micr 48(3):363–364

    Google Scholar 

  8. Hered R, Smithwick W, Sandler E, Goldstein J (2007) Orbital melanotic neuroectodermal tumor of infancy successfully treated with chemotherapy and subtotal excision. J AAPOS 11(5):504–505

    Article  PubMed  Google Scholar 

  9. Choi IC, Kook H, Han DK, Baek HJ, Jung ST, Lee JH, Park JG, Hwang TJ (2007) Melanotic neuroectodermal tumor of infancy in the femur, a case report and review of the literature. J Pediat Hematol Onc 29(12):854–857

    Article  Google Scholar 

  10. Pierre-Kahn A, Cinalli G, Lellouch-Tubiana A, Villarejo FJ, Sainte-Rose C, Pfister A, Couly G (1992) Melanotic neuroectodermal tumor of the skull and meninges in infancy. Pediatr Neurosurg 18:6–15

    Article  CAS  PubMed  Google Scholar 

  11. Kumari TP, Venugopal M, Mathews A, Kusumakumary P (2015) Effectiveness of chemotherapy in melanotic neurectodermal tumor of infancy. Pediat Hematol Onc 22(3):199–206

    Article  Google Scholar 

  12. Mello RJV, Vidal AKL, Fittipaldi HM, Montenegro LT, Calheiros LMC, Rocha GI (2000) Melanotic neuroectodermal tumor of infancy: clinicopathologic study of a case, with emphasis on the chemotherapeutic effects. Int J Surg Pathol 8(3):247–251

    Article  PubMed  Google Scholar 

  13. Davis J, DeBenedictis M, Frank D, Lessin M (2015) Melanotic neuroectodermal tumor of infancy: a wolf in sheep’s clothing. Ann Oto Rhinol Laryn 124(2):97–101

    Article  Google Scholar 

  14. Kruse-Lösler B, Gaertner C, Bürger H, Seper L, Joos U, Kleinheinz J (2006) Melanotic neuroectodermal tumor of infancy: systematic review of the literature and presentation of a case. Oral Surg Oral Med O 102:204–216

    Article  Google Scholar 

  15. Nikai H, Ijuhin N, Yamasaki A, Niitani K, Imai K (1977) Ultrastructural evidence for neural crest origin of the melanotic neuroectodermal tumor of infancy. J Oral Pathol Med 6:221–232

    Article  CAS  Google Scholar 

  16. Hoshino S, Takahashi H, Himura T, Nakazawa S, Naito Z, Asano G (1994) Melanotic neuroectodermal tumor of infancy in the skull associated with high serum levels of catecholamine: case report. J Neurosurg 80(5):919–924

    Article  CAS  PubMed  Google Scholar 

  17. Borello ED, Gorlin RJ (1966) Melanotic neuroectodermal tumor of infancy—a neoplasm of neural crese origin: report of a case associated with high urinary excretion of vanilmandelic acid. Cancer 19(2):196–206

    Article  CAS  PubMed  Google Scholar 

  18. Wass SH (1948) Melanotic adamantinoma of the mandible in a child aged 5 months. P Roy Soc Med 41(5):281–283

    CAS  Google Scholar 

  19. Battle RJV, Hovell JH, Spencer H (1952) Pigmented adamantinomata. Brit J Surg 39(156):368–370

    Article  CAS  PubMed  Google Scholar 

  20. Tiecke RW, Bernier JL (1956) Melanotic ameloblastoma. Oral Surg Oral Med O 9(11):1197–1209

    Article  Google Scholar 

  21. Kerr DA, Weiss AW (1963) Pigmented ameloblastoma of the mandible: report of a case. Oral Surg Oral Med O 16(11):1339–1343

    Article  CAS  Google Scholar 

  22. Panabokke RG, Eaton HL (1964) Congenital pigmented epulis of infancy (melanotic adamantinoma). Ceylon Med J 9:63–70

    CAS  PubMed  Google Scholar 

  23. Allen MS, Harrison W, Jahrsdoerfer RA (1969) Retinal anlage tumors: melanotic progonoma, melanotic adamantinoma, pigmented epulis, melanotic neuroectodermal tumor of infancy, benign melanotic tumor of infancy. Am J Clin Pathol 51(3):309–313

    Article  PubMed  Google Scholar 

  24. Baughman RA, Laskin JL, Lebowitz MS (1978) Mandibular tumorous condition in a 9-week-old child. J Oral Pathol Med 7:209–213

    Article  CAS  Google Scholar 

  25. Navas Palacios J (1980) Malignant melanotic neuroectodermal tumor: light and electron microscopic study. Cancer 46:529–536

    Article  CAS  PubMed  Google Scholar 

  26. Hupp JR, Topazian RG, Krutchkoff DJ (1981) The melanotic neuroectodermal tumor of infancy: report of two cases and review of the literature. Int J Oral Max Surg 10:432–446

    Article  CAS  Google Scholar 

  27. Jerrell RG, Hill CJ (1982) Melanotic neuroectodermal tumor of infancy and postsurgical dental complications: report of a case. J Dent Child 49(5):371–373

    CAS  Google Scholar 

  28. Abdin HA, Prabhu SR, Jose L (1982) Melanotic neuroectodermal tumor of the mandible in a Sudanese infant. Int J Oral Max Surg 11:383–387

    Article  CAS  Google Scholar 

  29. Melissari M, Tragni G, Gaetti L, Gabrielli M, Bozzetti A, Raffaini M (1988) Melanotic neuroectodermal tumour of infancy (MNTI): immunohistochemical and ultrastructural study of a case. J Cranio Maxill Surg 16:330–336

    Article  CAS  Google Scholar 

  30. Pettinato G, Manivel JC, d’Amore ESG, Jaszcz W, Gorlin RJ (1991) Melanotic neuroectodermal tumor of infancy: a reexamination of a histogenetic problem based on immunohistochemical, flow cytometric and ultrastructural study of 10 cases. Am J Surg Pathol 15(3):233–245

    Article  CAS  PubMed  Google Scholar 

  31. Kapadia SA, Frisman DM, Hitchcock CL, Ellis GL, Popek EJ (1993) Melanotic neuroectodermal tumor of infancy: clinicopathological, immunohistochemical and flow cytometric study. Am J Surg Pathol 17(6):566–573

    Article  CAS  PubMed  Google Scholar 

  32. Nelson ZL, Newman L, Loukota RA, Williams DM (1995) Melanotic neuroectodermal tumour of infancy: an immunohistochemical and ultrastructural study. Brit J Oral Max Surg 33:375–380

    Article  CAS  Google Scholar 

  33. Howell RE, Cohen MM (1996) Pathological case of the month. Arch Pediat Adol Med 150:1103–1104

    Article  CAS  Google Scholar 

  34. Hoshina Y, Hamamoto Y, Suzuki I, Nakajima T, Ida-Yonemochi H, Saku T (2000) Melanotic neuroectodermal tumor of infancy in the mandible: report of a case. Oral Surg Oral Med O 89(5):594–599

    Article  CAS  Google Scholar 

  35. Eckardt A, Swennen G, Teltzrow T (2001) Melanotic neuroectodermal tumor of infancy involving the mandible: 7-year follow-up after hemimandibulectomy and costochondral graft reconstruction. J Craniofac Surg 12(4):349–354

    Article  CAS  PubMed  Google Scholar 

  36. Barrett AW, Morgan M, Ramsay AD, Farthing PM, Newman L, Speight PM (2002) A clinicopathologic and immunohistochemical analysis of melanotic neuroectodermal tumor of infancy. Oral Surg Oral Med O 93(6):688–698

    Article  CAS  Google Scholar 

  37. Ahmed J, Chuckwulobelu R, Sebire NJ, Hartley BEJ, Dunaway DJ (2007) Hemimandibulectomy and autologous costochondral rib graft reconstruction for a case of melanotic neuroectodermal tumour of infancy arising within the mandible. Int J Pediatr Otorhi 2:189–193

    Google Scholar 

  38. Chen Y, Li T, Yu G, Shi Z (2008) Diagnosis and management of oral melanotic nenroectodermal tumor of infancy. Journal of Peking University 40(1):19–23

    CAS  Google Scholar 

  39. Neven J, Hulsbergen-van der Kaa C, Groot-Loonen J, de Wilde PCM, Merkx MA (2008) Recurrent melanotic neuroectodermal tumor of infancy: a proposal for treatment protocol with surgery and adjuvant chemotherapy. Oral Surg Oral Med O 106:493–496

    Article  Google Scholar 

  40. Eckhardt A, Barth E, Berten J, Gellrich N (2010) Pediatric mandibular resection and reconstruction: long-term results with autogenous rib grafts. Craniomaxillofacial Trauma and Reconstruction 3(1):25–32

    Article  Google Scholar 

  41. Piperi EP, Rake SA, Tosios KI, Vasilopoulou EE, Rake AP, Sandler NA, Issacson T, Sklavounou A, Koutlas IG (2010) Mandibular melanotic neuroectodermal tumor of infancy treated conservatively with enucleation. J Craniofac Surg 21(3):685–688

    Article  PubMed  Google Scholar 

  42. Krishnamurthy A, Vaidhyanathan A, Majhi U (2011) Malignant melanotic neuroectodermal tumor of infancy arising in the mandible. J Canc Res Ther 7(3):368–372

    Article  Google Scholar 

  43. Faria JCM, Batista BN, Sennes LU, Longo MVL, Danila AH, Ferreira MC (2014) Mandibular reconstruction with a fibular osteocutaneous free flap in an 8-month-old girl with a 12 year follow-up. Microsurgery 34:51–53

    Article  PubMed  Google Scholar 

  44. Chaudhary A, Wakhlu A, Mittal N, Misra S, Mehrotra D, Wakhlu AK (2009) Melanotic neuroectodermal tumor of infancy: 2 decades of clinical experience with 18 patients. J Oral Maxil Surg 67:47–51

    Article  Google Scholar 

  45. Nagase M, Ueda K, Fukushima M, Nakajima T (1983) Recurrent Melanotic neuroectodermal tumour of infancy: case report and survey of 16 cases. J Maxillofac Surg 11:131–136

    Article  CAS  PubMed  Google Scholar 

  46. Franchi G, Sleilati F, Soupre V, Boudjemaa S, Josset P, Diner PA, Vazquez MP (2002) Melanotic neuroectodermal tumour of infancy involving the orbit and maxilla: surgical management and follow-up strategy. Brit J Plast Surg 55(6):526–529

    Article  CAS  PubMed  Google Scholar 

  47. Woessmann W, Neugebauer M, Gossen R, Blutters-Sawatzki R, Reiter A (2003) Successful chemotherapy for melanotic neuroectodermal tumor of infancy in a baby. Med Pediatr Oncol 40:198–204

    Article  PubMed  Google Scholar 

  48. Garden A (2014) The never-ending story: finding a role for neoadjuvant chemotherapy in the management of head and neck cancer. J Clin Oncol 32(25):2685–2686

    Article  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Faculty of Medicine, American University of Beirut Medical Center, Beirut, Lebanon

    Christopher Maroun

  2. Department of Pathology and Laboratory Medicine, American University of Beirut Medical Center, Beirut, Lebanon

    Ibrahim Khalifeh

  3. Department of Otolaryngology Head and Neck Surgery, American University of Beirut Medical Center, 6th Floor, Hamra, Beirut, 1107 2020, Lebanon

    Elie Alam, Pierre Abi Akl & Roger V. Moukarbel

  4. Department of Pediatrics and Adolescent Medicine, American University of Beirut Medical Center, Beirut, Lebanon

    Raya Saab

Authors
  1. Christopher Maroun
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Ibrahim Khalifeh
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Elie Alam
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Pierre Abi Akl
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. Raya Saab
    View author publications

    You can also search for this author in PubMed Google Scholar

  6. Roger V. Moukarbel
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Roger V. Moukarbel.

Ethics declarations

Funding

None received.

Conflict of interest

All authors declare that they have no conflict of interest.

Ethical approval

This article does not contain any studies with human participants or animals performed by any of the authors.

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Maroun, C., Khalifeh, I., Alam, E. et al. Mandibular melanotic neuroectodermal tumor of infancy: a role for neoadjuvant chemotherapy. Eur Arch Otorhinolaryngol 273, 4629–4635 (2016). https://doi.org/10.1007/s00405-016-4066-6

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00405-016-4066-6

Keywords

Search

Navigation