eMedicine Specialties > Dermatology > Diseases of the Oral Mucosa

Melanotic Neuroectodermal Tumor of Infancy

Author: James Burns, DDS, MSEd, PhD, Chairman, Professor, Department of Oral and Maxillofacial Pathology, Medical College of Virginia School of Dentistry
Coauthor(s): Robert Strauss, DDS, Associate Professor, Department of Oral and Maxillofacial Surgery, Medical College of Virginia-Virginia Commonwealth University
Contributor Information and Disclosures

Updated: Feb 7, 2007

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 Krompecker in 1918 as a congenital melanocarcinoma.

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. 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 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 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.

Sex

The sexual predilection 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 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 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 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.
  • 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. 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. 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].

  2. 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].

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

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

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

  6. 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].

  7. Hoffman S, Jacoway J, Krolls S. Melanotic neuroectodermal tumor of infancy. In: AFIP fascicle 24: Intraosseous and Parosteal Tumors of the Jaws. 1987:165-169.

  8. 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].

  9. 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].

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

  11. 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].

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

  13. 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].

  14. 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].

  15. 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].

  16. 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].

  17. Neville B, Damm D, Allen C. Melanotic Neuroectodermal Tumor of Infancy. 1995:385-386.

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

  19. 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].

  20. 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].

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Further Reading

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Keywords

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

James Burns, DDS, MSEd, PhD, Chairman, Professor, Department of Oral and Maxillofacial Pathology, Medical College of Virginia School of Dentistry
James Burns, DDS, MSEd, PhD is a member of the following medical societies: American Academy of Oral and Maxillofacial Pathology, American Association for Cancer Education, American Dental Association, and International Association of Oral Pathologists
Disclosure: Nothing to disclose.

Coauthor(s)

Robert Strauss, DDS, Associate Professor, Department of Oral and Maxillofacial Surgery, Medical College of Virginia-Virginia Commonwealth University
Robert Strauss, DDS is a member of the following medical societies: American Association of Oral and Maxillofacial Surgeons and American Dental Association
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; Basilea Honoraria Consulting; Briston-Myers Squibb Honoraria Consulting; Celtic Pharma  Other; Centocor Honoraria Consulting; Chattem Honoraria Other; DermiPsor Honoraria Consulting

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

William D James, MD, Paul R Gross Professor of Dermatology, University of Pennsylvania School of Medicine; Vice-Chair, Program Director, Department of Dermatology, University of Pennsylvania Health System
William D James, MD is a member of the following medical societies: American Academy of Dermatology and Society for Investigative Dermatology
Disclosure: elsevier Royalty Other; american college of physicians Honoraria Other

 
 
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