eMedicine Specialties > Dermatology > Diseases of the Oral Mucosa

Melanotic Neuroectodermal Tumor of Infancy

Author: William M Carpenter, DDS, MS, Professor, Chairman, Department of Pathology and Medicine, University of the Pacific Arthur A Dugoni School of Dentistry
Contributor Information and Disclosures

Updated: Sep 1, 2009

Introduction

Background

Melanotic neuroectodermal tumor of infancy (MNTI) is a relatively uncommon osteolytic-pigmented neoplasm that primarily affects the jaws of newborn infants. The lesion has had an interesting history since its initial description by Krompecher in 1918 as a congenital melanocarcinoma.1

For the next 5 decades, the lesion was reported under a variety of different names as succeeding authors attempted to identify the cell of origin. Some of the terms applied to this lesion included pigmented ameloblastoma, retinal anlage tumor, melanotic progonoma, melanotic epithelial odontoma, pigmented teratoma, atypical melanoblastoma, melanotic adamantinoma, pigmented epulis, retinal choristoma, melanoameloblastoma, and retinoblastic teratoma. These terms reflected theories of suspected origin from the odontogenic apparatus, the pigmented anlage of the retina, or the sensory neuroectodermal tissues.

In 1966, Borello and Gorlin reported a case with high urinary excretion of vanillylmandelic acid (VMA), suggesting a neural crest origin, and they proposed the term melanotic neuroectodermal tumor of infancy.2 Since then, numerous histochemical, immunohistochemical, electron microscopic, and tissue culture studies have supported the neural crest origin and confirmed the preferred term of melanotic neuroectodermal tumor of infancy.

Pathophysiology

Several patients with melanotic neuroectodermal tumor of infancy (MNTI) have demonstrated a high urinary excretion of VMA. This finding adds credence to a neural crest origin because elevated VMA has been reported in neuroblastoma, ganglioneuroblastoma, pheochromocytoma, and other neural crest tumors. However, the presence of urinary VMA is not diagnostic for MNTI.

Frequency

United States

Approximately 200 cases of melanotic neuroectodermal tumor of infancy (MNTI) have been reported in the literature. An exact number is difficult to discern because of the variety of terms that have been applied to the lesion in the past.3

Sex

The sexual predilection for melanotic neuroectodermal tumor of infancy (MNTI) is nearly equal, with a male-to-female ratio of 6:7.

Age

Most patients, by some estimates more than 90%, present with the tumor in the first year of life, usually from age 1-6 months. The mean age of patients with melanotic neuroectodermal tumor of infancy (MNTI) is 4.3 months. Although extremely rare, a few cases of MNTI have been reported in adults, notably, a 23-year-old man, a 24-year-old woman, and a 67-year-old woman.

Clinical

History

  • Although melanotic neuroectodermal tumor of infancy (MNTI) is classified as a benign lesion, it is often clinically worrisome because of its rapid onset and alarming local growth rate.
  • Often, sucking and feeding are impaired secondary to the swelling.
  • The patient is usually asymptomatic.

Physical

  • The typical melanotic neuroectodermal tumor of infancy (MNTI) begins as a nonulcerated, lightly pigmented, blue or black lesion on the anterior aspect of the maxilla and rapidly expands to form a swelling or a tumescence that is cosmetically obvious to the parents of the infant.


Melanotic neuroectodermal tumor of infancy presen...

Melanotic neuroectodermal tumor of infancy presents as a rapidly growing bluish mass on the anterior aspect of the maxilla.

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Melanotic neuroectodermal tumor of infancy presen...

Melanotic neuroectodermal tumor of infancy presents as a rapidly growing bluish mass on the anterior aspect of the maxilla.

  • The intraoral lesion appears as a sessile, lobulated mass, often reaching 2-4 cm in diameter by the time of diagnosis.
  • Bone destruction and displacement of teeth often occur because of the intraosseous location in the maxilla.
  • No thrill or pulse can be elicited from the MNTI. Although the lesion expands rapidly, the overlying mucosa remains intact.
  • More than 90% of MNTI occur in the head and neck region, with most on the anterior part of the maxillary ridge. Other common sites include the skull, the mandible, the epididymis, and the brain.4 Rare lesions have been reported in the shoulder, the skin, the femur, the mediastinum, and the uterus.
  • All but 2 of the reported cases have been solitary lesions.

Causes

See Pathophysiology.

More on Melanotic Neuroectodermal Tumor of Infancy

Overview: Melanotic Neuroectodermal Tumor of Infancy
Differential Diagnoses & Workup: Melanotic Neuroectodermal Tumor of Infancy
Treatment & Medication: Melanotic Neuroectodermal Tumor of Infancy
Follow-up: Melanotic Neuroectodermal Tumor of Infancy
Multimedia: Melanotic Neuroectodermal Tumor of Infancy
References
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References

  1. Krompecher Z. Zur histogenese and morphologic den adamantinome und sonstiger kiefergeschwulste. Beitr Pathol Anat. 1918;165-97.

  2. Borello ED, Gorlin RJ. Melanotic neuroectodermal tumor of infancy--a neoplasm of neural crese origin. Report of a case associated with high urinary excretion of vanilmandelic acid. Cancer. Feb 1966;19(2):196-206. [Medline].

  3. Neville B, Damm D, Allen C. Melanotic Neuroectodermal Tumor of Infancy. In: Oral and Maxillofacial Pathology. 3rd. St Louis, MO: Saunders/Elsevier; 2009:533-534.

  4. Matsumoto M, Sakuma J, Suzuki K, et al. Melanotic neuroectodermal tumor of infancy in the skull: case report and review of the literature. Surg Neurol. Mar 2005;63(3):275-80. [Medline].

  5. Dammann O, Hagel C, Allers B, et al. Malignant melanotic neuroectodermal tumor of infancy. Childs Nerv Syst. Mar 1995;11(3):186-8. [Medline].

  6. Dashti SR, Cohen ML, Cohen AR. Role of radical surgery for intracranial melanotic neuroectodermal tumor of infancy: case report. Neurosurgery. Jul 1999;45(1):175-8. [Medline].

  7. Woessmann W, Neugebauer M, Gossen R, et al. Successful chemotherapy for melanotic neuroectodermal tumor of infancy in a baby. Med Pediatr Oncol. Mar 2003;40(3):198-9. [Medline].

  8. Cutler LS, Chaudhry AP, Topazian R. Melanotic neuroectodermal tumor of infancy: an ultrastructural study, literature review, and reevaluation. Cancer. Jul 15 1981;48(2):257-70. [Medline].

  9. Barrett AW, Morgan M, Ramsay AD, Farthing PM, Newman L, Speight PM. A clinicopathologic and immunohistochemical analysis of melanotic neuroectodermal tumor of infancy. Oral Surg Oral Med Oral Pathol Oral Radiol Endod. Jun 2002;93(6):688-98. [Medline].

  10. Bouckaert MM, Raubenheimer EJ. Gigantiform melanotic neuroectodermal tumor of infancy. Oral Surg Oral Med Oral Pathol Oral Radiol Endod. Nov 1998;86(5):569-72. [Medline].

  11. Dehner LP, Sibley RK, Sauk JJ Jr. Malignant melanotic neuroectodermal tumor of infancy: a clinical, pathologic, ultrastructural and tissue culture study. Cancer. Apr 1979;43(4):1389-410. [Medline].

  12. Fletcher C. Melanotic neuroectodermal tumor of infancy. Clinicopathological, immunohistochemical and flow cytometry study. Am J Surg Pathol. 1995;17:566-73.

  13. Gaiger de Oliveira M, Thompson LD, Chaves AC, et al. Management of melanotic neuroectodermal tumor of infancy. Ann Diagn Pathol. Aug 2004;8(4):207-12. [Medline].

  14. Hoffman S, Jacoway J, Krolls S. Melanotic neuroectodermal tumor of infancy. In: AFIP fascicle 24: Intraosseous and Parosteal Tumors of the Jaws. 1987:165-9.

  15. Hoshina Y, Hamamoto Y, Suzuki I, et al. Melanotic neuroectodermal tumor of infancy in the mandible: report of a case. Oral Surg Oral Med Oral Pathol Oral Radiol Endod. May 2000;89(5):594-9. [Medline].

  16. Johnson RE, Scheithauer BW, Dahlin DC. Melanotic neuroectodermal tumor of infancy. A review of seven cases. Cancer. Aug 15 1983;52(4):661-6. [Medline].

  17. Kapadia SB, Frisman DM, Hitchcock CL, et al. Melanotic neuroectodermal tumor of infancy. Clinicopathological,immunohistochemical, and flow cytometric study. Am J Surg Pathol. Jun 1993;17(6):566-73. [Medline].

  18. Kaya S, Unal OF, Sarac S, Gedikoglu G. Melanotic neuroectodermal tumor of infancy: report of two cases and review of literature. Int J Pediatr Otorhinolaryngol. Apr 15 2000;52(2):169-72. [Medline].

  19. Khoddami M, Squire J, Zielenska M, Thorner P. Melanotic neuroectodermal tumor of infancy: a molecular genetic study. Pediatr Dev Pathol. Jul-Aug 1998;1(4):295-9. [Medline].

  20. Liu HH, Chen TW, Chang HS. Melanotic neuroectodermal tumour of infancy in the maxilla: a case report. Int J Paediatr Dent. Sep 2004;14(5):371-5. [Medline].

  21. Massachusetts General Hospital. Case records of the Massachusetts General Hospital. Weekly clinicopathological exercises. Case 7-2001. A male infant with a right maxillary mass. N Engl J Med. Mar 8 2001;344(10):750-7. [Medline].

  22. Nelson ZL, Newman L, Loukota RA, Williams DM. Melanotic neuroectodermal tumour of infancy: an immunohistochemical and ultrastructural study. Br J Oral Maxillofac Surg. Dec 1995;33(6):375-80. [Medline].

  23. Oruckaptan HH, Soylemezoglu F, Kutluk T, Akalan N. Benign melanocytic tumor in infancy: discussion on a rare case and review of the literature. Pediatr Neurosurg. May 2000;32(5):240-7. [Medline].

  24. Neurotodermal tumours. In: Barnes L, Eveson JW, Reichart P, Sidransky D, eds. Pathology & Genetics: Head and Neck Tumors. Lyon, France: World Health Organization Classification of Tumours; 2005:70-72.

  25. Pettinato G, Manivel JC, d'Amore ES, Jaszcz W, Gorlin RJ. 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. Mar 1991;15(3):233-45. [Medline].

  26. Puchalski R, Shah UK, Carpentieri D, et al. Melanotic neuroectodermal tumor of infancy (MNTI) of the hard palate: presentation and management. Int J Pediatr Otorhinolaryngol. Jun 30 2000;53(2):163-8. [Medline].

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Further Reading

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Keywords

melanotic neuroectodermal tumor of infancy, MNTI, pigmented ameloblastoma, melanoameloblastoma, retinal anlage tumor, melanotic progonoma, melanotic epithelial odontoma, pigmented teratoma, atypical melanoblastoma, melanotic adamantinoma, pigmented epulis, retinal choristoma, retinoblastic teratoma, congenital melanocarcinoma

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Contributor Information and Disclosures

Author

William M Carpenter, DDS, MS, Professor, Chairman, Department of Pathology and Medicine, University of the Pacific Arthur A Dugoni School of Dentistry
William M Carpenter, DDS, MS is a member of the following medical societies: American Academy of Oral and Maxillofacial Pathology and American Academy of Oral Medicine
Disclosure: Nothing to disclose.

Medical Editor

Mark G Lebwohl, MD, Chairman, Department of Dermatology, Mount Sinai School of Medicine
Mark G Lebwohl, MD is a member of the following medical societies: American Academy of Dermatology
Disclosure: Abbott Laboratories Honoraria Consulting; Actelion Honoraria Consulting; Amgen Honoraria Consulting; Astellas Honoraria Consulting; Centocor Honoraria Consulting; DermiPsor Honoraria Consulting; Galderma  Consulting; Genentech Honoraria Consulting; Helix BioMedix Honoraria Consulting; Medicis Honoraria Investigator

Pharmacy Editor

Richard P Vinson, MD, Assistant Clinical Professor, Department of Dermatology, Texas Tech University School of Medicine; Consulting Staff, Mountain View Dermatology, PA
Richard P Vinson, MD is a member of the following medical societies: American Academy of Dermatology, Association of Military Dermatologists, Texas Dermatological Society, and Texas Medical Association
Disclosure: Nothing to disclose.

Managing Editor

Drore Eisen, MD, DDS, Consulting Staff, Department of Dermatology, Dermatology Research Associates of Cincinnati
Drore Eisen, MD, DDS is a member of the following medical societies: American Academy of Dermatology, American Academy of Oral Medicine, and American Dental Association
Disclosure: Nothing to disclose.

CME Editor

Glen H Crawford, MD, Assistant Clinical Professor, Department of Dermatology, University of Pennsylvania School of Medicine; Chief, Division of Dermatology, The Pennsylvania Hospital
Glen H Crawford, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, Phi Beta Kappa, and Society of USAF Flight Surgeons
Disclosure: Nothing to disclose.

Chief Editor

Dirk M Elston, MD, Director, Department of Dermatology, Geisinger Medical Center
Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology
Disclosure: Nothing to disclose.

 
 
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