Melanotic neuroectodermal tumor of infancy: clinical, radiological, and pathological features |
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| Authors: | Fowler D J Chisholm J Roebuck D Newman L Malone M Sebire N J |
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| Affiliation: | a Department of Pediatric Histopathology, Great Ormond Street Hospital, London, United Kingdomb Department of Oncology, Great Ormond Street Hospital, London, United Kingdomc Department of Radiology, Great Ormond Street Hospital, London, United Kingdomd Department of Head and Neck Surgery, Great Ormond Street Hospital, London, United Kingdom |
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| Abstract: | We present a case of a 4-month-old female infant with a maxillary melanotic neuroectodermal tumor of infancy (MNTI) and review the pooled data from previous publications on this entity. The literature to date comprises 378 reported cases from 1918 to the present, from which data on the presence or absence of metastatic disease was available in 311, and on the presence or absence of local recurrence in 165. These pooled data suggest a local recurrence rate of 36% with metastasis occurring in 7% of cases. At present, the optimal management includes complete surgical excision with clear margins, but there are no reliable histopathological or molecular features to predict the biological behavior in individual cases. |
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| Keywords: | maxilla melanotic neuroectodermal tumor pigmented |
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