Surgical Pathology Criteria–

Melanotic Neuroectodermal Tumor of Infancy

Differential Diagnosis

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

	Nblast	ALL	Ewing Sarc (PNET)	Rhabdo	MNTI	DSCT	Wilms Tumor
Synapto	pos	neg	neg	neg	pos	neg	neg
NB84	pos	neg	neg	neg	neg	neg	neg
CD45RB (LCA)	neg	pos	neg	neg	neg	neg	neg
CD99	neg	pos	pos	neg	rare	neg	neg
Myogenin	neg	neg	neg	pos	rare	neg	neg
MyoD1	neg	neg	neg	pos	neg	neg	neg
Keratin	neg	neg	rare	rare	pos	pos	neg
Desmin	neg	neg	neg	pos	rare	pos	neg
WT1(C)	neg	neg	neg	neg	neg	pos	pos
S100	neg	neg	rare	neg	rare	neg	neg
HMB45	neg	neg	neg	neg	pos	neg	neg

Nblast = Neuroblastoma; ALL = Precursor T Lymphoblastic Leukemia; Rhabdo = Rhabdomyosarcoma; MNTI = Melanotic Neuroectodermal Tumor of Infancy; DSCT = Desmoplastic Small (Round) Cell Tumor; Synapto = Synaptophysin

Identification of melanin positive cells may raise the differential of melanoma or of teratoma with retina differentiation
- Melanoma is rare in infants, lacks the bipasic pattern containing neuroblastic cells and usually arises in the sinonasal mucosal lining, not the bones
- Teratoma contains other differentiated elements and occurs intracranially rather than in the bones or in the testis rather than the epididymis

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