Info

Fig. 4.38. Melanotic neuroectodermal tumour of infancy consists of small dark cells and larger cells with vesicular nuclei. Melanin is usually associated with the latter cell population

py consists of debulking and irradiation. Five-year survival rate is approximately 50%. Histologic features related with prognosis have not been identified [29]. Metastatic disease is unusual [25].

4.7.3 Melanotic Neuroectodermal Tumour of Infancy

Quite often, immature odontogenic tissues form part of the material excised or biopsied, due to the early age of occurrence and the close association of the tumour with tooth germs. This should not be mistaken as evidence of an odontogenic tumour. The highly characteristic histological pattern leaves no room for other differential diagnostic considerations.

Clinically, melanotic neuroectodermal tumour of infancy manifests as a rapidly growing blue tissue mass, usually at the anterior alveolar maxillary ridge. Radio-logically, bone resorption may be seen, although this is difficult to evaluate in the delicate bony structures of the infantile maxilla. Tooth germs are displaced and may lie within the tumour mass. Conservative excision usually constitutes adequate treatment. Recurrences have been described, but metastases are exceptionally rare [113]. There are no histological features predicting more aggressive behaviour [9].

References

ICD-O:9363/0

Cells derived from the neural crest play a major role in the formation of the jaws and teeth. These cells are also thought to be the source from which the melanotic neu-roectodermal tumour of infancy develops [106]. Most of the lesions occur before the age of 1 year. The majority of them occur in the anterior maxilla [68].

The tumour shows dense fibrous stroma with nests composed of two different cell types: centrally placed small dark cells without any discernable cytoplasm and peripherally located larger cells with vesicular nuclei and ample cytoplasm with melanin pigment (Fig. 4.38) [9, 68, 113]. Maturation of the small cells to ganglion cells has been reported [144]. Although the cells may be atypical, mitotic figures are rare [9]. Sometimes, a transition of the large cells to osteoblasts forming tiny bony trabeculae can be observed [157]. The lesion is not encapsulated.

Immunohistochemically, the large cells are positive for a wide variety of cytokeratins, neuron-specific eno-lase, S-100, HMB45 and chromogranin. The small cells show positivity for CD56, neuron-specific enolase, syn-aptophysin and chromogranin [9]. This pattern can be summarised as evidence for neural, melanocytic and epithelial differentiation. In addition, the large cells have been shown to be positive for vimentin [157]. Ultra-structurally, the small cells show neurosecretory granules and the large cells show melanosomes at different stages of development [113].

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10 Ways To Fight Off Cancer

10 Ways To Fight Off Cancer

Learning About 10 Ways Fight Off Cancer Can Have Amazing Benefits For Your Life The Best Tips On How To Keep This Killer At Bay Discovering that you or a loved one has cancer can be utterly terrifying. All the same, once you comprehend the causes of cancer and learn how to reverse those causes, you or your loved one may have more than a fighting chance of beating out cancer.

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