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Melanotic Neuroectodermal Tumor of Infancy Differential Diagnoses

  • Author: Leticia Ferreira, DDS, MS; Chief Editor: William D James, MD  more...
 
Updated: Jan 26, 2015
 
 

Diagnostic Considerations

Consider clinical, radiographic, laboratory, and histologic findings when establishing a proper differential diagnosis for melanotic neuroectodermal tumor of infancy (MNTI). The MNTI often presents as a fast-growing lesion, suggesting a clinical impression of infection or malignant neoplasm. The location in the anterior aspect of the maxilla is consistent with a number of odontogenic cysts and tumors; however, odontogenic cysts (eg, periapical cyst, dentigerous cyst, odontogenic keratocyst, calcifying odontogenic cyst) occur in an older age group, teenaged through middle-aged adults. The same age differential is noted with respect to the more common odontogenic tumors (eg, ameloblastoma, odontoma, adenomatoid odontogenic tumor, calcifying epithelial odontogenic tumor, ameloblastic fibroma, odontogenic myxoma, odontogenic fibroma).

In addition to a diagnosis of MNTI, the young age of the patient and the maxillary alveolar ridge location are also compatible with a clinical diagnosis of congenital epulis of the newborn. However, this entity can be differentiated from the MNTI by its strong female predilection and involvement exclusively of the gingival soft tissues without destruction of the underlying maxillary bone.[1] Many nonodontogenic entities are possible in the jaws, including a central giant cell granuloma, ossifying fibroma, fibromatosis, fibrous dysplasia, hemangioma, arteriovenous malformation, craniopharyngioma, Langerhans cell histiocytosis, rhabdomyosarcoma, Ewing sarcoma, and lymphoma. However, only fibromatosis, Langerhans cell histiocytosis, rhabdomyosarcoma, Ewing sarcoma, and lymphoma are common in young children.

The radiographic appearance of a maxillary alveolar low-density radiolucency is consistent with any of the odontogenic cysts or tumors. Additionally, many of the aforementioned nonodontogenic lesions may also present with a radiographic appearance similar to that of MNTI.

Once a differential diagnosis is established from the clinical and radiographic findings, histologic evaluation is necessary to determine the final diagnosis. The histologic appearance of MNTI is usually that of a small, dark, cell neoplasm suggestive of neuroblastoma, rhabdomyosarcoma, Ewing sarcoma, lymphoma, desmoplastic small round cell tumor, and peripheral primitive neuroectodermal tumor. However, MNTIs can be differentiated from these more ominous entities by its biphasic population of cells, in which not only the small, dark cells are present, but also a second cell population, consisting of larger, polygonal, cells usually is identified. These larger cells exhibit vesicular nuclei and granules of dark-brown melanin. The identification of this second cell population helps differentiate MNTI from other small, round, blue cell tumors. Although the histologic appearance is characteristic, special immunohistochemical stains may be used to make a definitive diagnosis.

 
 
Contributor Information and Disclosures
Author

Leticia Ferreira, DDS, MS Assistant Professor of Pathology and Medicine, Department of Dental Practice, University of the Pacific, Arthur A Dugoni School of Dentistry

Leticia Ferreira, DDS, MS is a member of the following medical societies: American Academy of Oral and Maxillofacial Pathology, American Association of Women Dentists, American Dental Association, American Dental Education Association, California Dental Association, San Francisco Dental Society

Disclosure: Nothing to disclose.

Specialty Editor Board

Richard P Vinson, MD Assistant Clinical Professor, Department of Dermatology, Texas Tech University Health Sciences Center, Paul L Foster 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, Texas Medical Association, Association of Military Dermatologists, Texas Dermatological Society

Disclosure: Nothing to disclose.

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, American Dental Association

Disclosure: Nothing to disclose.

Chief Editor

William D James, MD Paul R Gross Professor of Dermatology, Vice-Chairman, Residency Program Director, Department of Dermatology, University of Pennsylvania School of Medicine

William D James, MD is a member of the following medical societies: American Academy of Dermatology, Society for Investigative Dermatology

Disclosure: Nothing to disclose.

Additional Contributors

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: Received none from Amgen for consultant & investigator; Received none from Novartis for consultant & investigator; Received none from Pfizer for consultant & investigator; Received none from Celgene Corporation for consultant & investigator; Received none from Clinuvel for consultant & investigator; Received none from Eli Lilly & Co. for consultant & investigator; Received none from Janssen Ortho Biotech for consultant & investigator; Received none from LEO Pharmaceuticals for consultant & inves.

Acknowledgements

James Burns, DDS, PhD, MEd Chairman, Professor, Department of Oral and Maxillofacial Pathology, Assistant Dean, Clinical Education, Virginia Commonwealth University School of Dentistry

James Burns 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.

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.

Robert Strauss, DDS Associate Professor, Department of Oral and Maxillofacial Surgery, Virginia Commonwealth University School of Dentistry

Robert Strauss is a member of the following medical societies: American Association of Oral and Maxillofacial Surgeons and American Dental Association

Disclosure: Nothing to disclose.

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Melanotic neuroectodermal tumor of infancy presents as a rapidly growing bluish mass on the anterior aspect of the maxilla.
Axial CT, bone window, noncontrasted scan demonstrates expansile lytic lesion of the left maxilla producing displacement of dental follicles.
The biphasic population of cells demonstrates alveolar structures lined by cuboidal epithelioid cells demonstrating granules of dark-brown melanin pigment. The second cell type is neuroblastic in appearance and consists of small, round, hyperchromatic cells.
 
 
 
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