Small neuroblastic cells resemble those of neuroblastoma
Small dense nuclei with scant cytoplasm
Large melanin pigmented epithelioid cells
Large vesicular nuclei with prominent nucleoli
Cells are set in a dense collagenous stroma
Present as cords, poorly formed tubules, and clusters of cells
Clusters are frequently discohesive with an alveolar pattern
Large and small cells may be in adjacent discrete nodules or mixed
Large cells frequently form an outer layer surrounding small cells
Tumor may be grossly circumscribed but is usually invasive
Over 90% occur during the first year of life
May be present at birth
Over 90% occur in bones of the head and neck (see Clinical at left)
Maxilla is most common site, followed by mandible and skull
Most common peripheral site is epididymis
Over 90% are benign if entirely resected (see Clinical at left)
Florette K. Gray Hazard MD
Department of Pathology
Stanford University School of Medicine
Stanford CA 94305-5342
Original posting/updates: 2/5/11
Pigmented Epithelioid Cells
Neuron Specific Enolase
Predominant reactivity from literature given above
Even for these markers there is considerable variability in reports
Infrequent to rare positive cases are reported for the following:
S100, CD99, Desmin, Actin, Myogenin, Chromogranin, GFAP, EMA, Neurofilaments
NB84 negative - both populations
There is no specific cytogenetic translocation
A wide range of chromosomal abnormalities may be identified
While the morphology suggests many “small” round blue cell tumors, the clinical context of a maxillary tumor in a newborn or infant narrows the differential diagnosis to include rhabdomyosarcoma, neuroblastoma and lymphoma
In such cases, immunohistochemistry may be required
Barrett AW, Morgan M, Ramsay AD, et al:: A Clinicopathologic and Immunohistochemical Analysis of Melanotic Neuroectodermal Tumor of Infancy. Oral Surgery, Oral Medicine, Oral Pathology, Oral Radiology & Endodontics 2002;93(6):688-698.
Chaudhary A, Wakhlu A, Mittal N, et al:: Melanotic Neuroectodermal Tumor of Infancy: 2 Decades of Clinical Experience with 18 Patients. Journal of Oral and Maxillofacial Surgery 2009;67(1):47-51.
Dehner LP. Skeletal System. IN: Dehner LP, Husain AN, Stocker JT, (eds) Pediatric Pathology, Third edition, Lippincott Williams & Wilkins, Philadelphia, 2011, 1190-1270.
Ellison DA, Adada B, Qualman SJ, et al:: Melanotic Neuroectodermal Tumor of Infancy: Report of a Case with Myogenic Differentiation. Pediatric and Developmental Pathology 2007;10(2):157-160.
Fowler DJ, Chisholm J, Roebuck D, et al:: Melanotic Neuroectodermal Tumor of Infancy: Clinical, Radiological, and Pathological Features. Fetal & Pediatric Pathology 2006;25(2):59-72.
Gonçalves CF, Costa NdL, Oliveira-Neto HH, et al:: Melanotic Neuroectodermal Tumor of Infancy: Report of 2 Cases. Journal of Oral and Maxillofacial Surgery 2010;68(9):2341-2346.
Kruse-Lösler B, Gaertner C, Bürger H, et al:: Melanotic Neuroectodermal Tumor of Infancy: Systematic Review of the Literature and Presentation of a Case. Oral Surgery, Oral Medicine, Oral Pathology, Oral Radiology, and Endodontology 2006;102(2):204-216.
Metwaly H, Cheng J, Maruyama S, et al:: Establishment and Characterization of New Cell Lines Derived from Melanotic Neuroectodermal Tumor of Infancy Arising in the Mandible. Pathology International 2005;55(6):331-342.
Pettinato G, Manivel JC, d'Amore ESG, et al:: Melanotic Neuroectodermal Tumor of Infancy: A Reexamination of a Histogenetic Problem Based on Immunohistochemical, Flow Cytometric, and Ultrastructural Study of 10 Cases. The American Journal of Surgical Pathology 1991;15(3):233-245.
Selim H, Shaheen S, Barakat K, et al:: Melanotic Neuroectodermal Tumor of Infancy: Review of Literature and Case Report. Journal of Pediatric Surgery 2008;43E25-E29.