Melanotic neuroectodermal tumor of infancy. Clinicopathological, immunohistochemical, and flow cytometric study.

Department of Otolaryngic Pathology, Armed Forces Institute of Pathology, Washington, D.C.
American Journal of Surgical Pathology (Impact Factor: 4.59). 07/1993; 17(6):566-73.
Source: PubMed

ABSTRACT Twenty cases of melanotic neuroectodermal tumor of infancy (MNTI) are reported. The patients (13 females, seven males), whose ages ranged from 1 to 9 months (mean, 5 months), typically presented with a rapidly growing mass. Tumor sites included the maxilla (13 cases), mandible (three cases), dura (two cases), brain (one case), and skull/orbit (one case). The mean tumor size was 3.5 cm (range, 1.0-10.0 cm). Follow-up was obtained on 12 cases. Five tumors (45%) recurred within 4 months of diagnosis, but none metastasized. One surgical death occurred. Histologic appearance was distinctive, with tubular or alveolar formations of large melanin-containing cells around nests of smaller neuroblastic cells possessing scant or fibrillar cytoplasm. Twelve tumors were studied immunohistochemically; tumor was positive for cytokeratin in 12 of 12, for HMB 45 in 12 of 12, for vimentin in seven of eight, and for epithelial membrane antigen (EMA) in four of nine tumors, mainly in the large cells. Neuron-specific enolase (NSE) (seven of 12) and Leu 7 (nine of 12) were positive in small and large cells; some tumors also expressed synaptophysin (four of 12), glial fibrillary acidic protein (GFAP, three of 12 tumors), or S-100 protein (two of 12 tumors). No staining was found for chromogranin, desmin, or carcinoembryonic antigen (CEA). Eight of 10 tumors studied had interpretable results on flow cytometry (FCM) (four DNA diploid, three DNA aneuploid, and one DNA diploid with a prominent shoulder). Tumor recurred locally in two of five cases with follow-up, and we were unable to demonstrate the usefulness of FCM in predicting recurrences. Further studies are necessary to define better the potential usefulness of FCM in predicting aggressive behavior. Distinctive morphology and multiphenotypic (epithelial, neural, melanocytic) expression distinguish MNTI from melanoma and metastatic neuroblastoma.

  • Source
    Modern Pathology 03/2002; 15(3):278-278. DOI:10.1038/modpathol.3880523 · 6.36 Impact Factor
  • [Show abstract] [Hide abstract]
    ABSTRACT: Melanotic neuroectodermal tumor of infancy (MNTI) is a relatively uncommon osteolytic-pigmented neoplasm that primarily affects the jaws of infants. The early onset and its rapid disfiguring spread necessitate early diagnosis. A 4-month-old male child reported with the complaint of swelling in the right back tooth region of the upper jaw, which rapidly increased in size causing disfigurement of the face. Radiographic examination showed a diffuse osteolytic radiolucent lesion in the right maxilla and displacement and dysmorphic changes in the developing primary tooth buds. Wide surgical excision was performed under general anesthesia. Histopathological report revealed characteristic large pigmented epitheloid cells (melanocyte like cells). The biphasic tumor cell population arranged in a background of fibrous connective tissue stroma is suggestive of MNTI involving the cancellous bone. Early diagnosis and management of such aggressive tumors precludes significant morbidity of the patient.
    04/2013; 4(4):559-562. DOI:10.4103/0976-237X.123091
  • Source
    [Show abstract] [Hide abstract]
    ABSTRACT: A biphasic tumour is a truly histological term that refers to neoplastic tissue which is characterized by two different cellular elements. Several histogenetic theories have been proposed for the aetiogenesis of the biphasic tumours. Literatures have been published on the individual lesions, which have described their biphasic nature but, biphasic tumours have not been categorized singly . Categorizing biphasic tumours is not likely to highlight diagnostic standards, but it may sensitize the therapeutic planning and post operative monitoring. This review article focuses on the histogenetic concepts of biphasic tumours, and the histopathological description of the lesions that are suggested to be biphasic tumours.
    02/2014; 8(2):266-270. DOI:10.7860/JCDR/2014/7506.4078