eMedicine Specialties > Dermatology > Diseases of the Oral Mucosa

Melanotic Neuroectodermal Tumor of Infancy: Treatment & Medication

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

Treatment

Surgical Care

The treatment of choice for melanotic neuroectodermal tumor of infancy (MNTI) is surgical excision, and it is usually curative. This treatment can usually be accomplished with a partial maxillectomy by using a Weber-Fergusson incision and a facial degloving approach. Teeth, developing teeth, and the adjacent bone must be sacrificed when they lie near the borders of MNTI, since many clinicians advocate that a 5-mm margin of healthy tissue be included with the surgical specimen.6

The possibility of local recurrence is a problem that has been documented to range from 10-60% of patients depending on the study quoted. Overall, the average recurrence rate is 15-20%. The recurrent lesions, possibly secondary to inadequate excision or multicentricity, usually become apparent within the first year after surgery. In instances of inoperable recurrence or where clear margins are impossible to obtain, radiation therapy and/or chemotherapy have been used, but too few examples exist for preferences to be established.7

Although MNTI is an aggressive benign tumor, malignant variants have been reported, ranging from 1.5% of total MNTI to 2.1% of the maxillary subset.8 Metastatic spread of MNTI occurs infrequently, in less than 5% of malignant cases. Management of these rare cases is different. Few, if any, parameters exist, either clinically or histologically, to predict the development of metastatic lesions. In a few cases reported as malignant, the histologic features have taken on a neuroblastomalike appearance.

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

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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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