Nevogenesis by Ashfaq A. Marghoob

Author:Ashfaq A. Marghoob
Language: eng
Format: epub
Publisher: Springer Berlin Heidelberg, Berlin, Heidelberg

Neurocutaneous Melanocytosis

Neurocutaneous melanocytosis, cited in earlier literature as neurocutaneous melanosis (NCM), is a neurological and cutaneous disorder characterized by abnormal aggregations of nevomelanocytes within the central nervous system (Fig. 7.3) and the skin. NCM is a complication of large/giant CMN, or multiple smaller CMN, in a fraction of patients. Recent studies using patient registries with many hundreds of registrants find the incidence of NCM to range between 5 and 15 % of all persons with large and giant CMN [2, 18, 74].

Fig. 7.3(a) Macroscopic appearance of a brain from a patient with neurocutaneous melanocytosis (NCM; anterior to right). Dark lesions correspond to foci of parenchymal invasion of frontal and temporal lobes by NCM cells, some of which are indicated by arrowheads. (b) NCM at surface leptomeninges (top) and along Virchow-Robin spaces. H&E  ×  20. (c, d) Brain parenchyma from a previously unreported case of NCM, demonstrating ectopic nevomelanocytes and apparent uptake of melanin granules by adjacent neurons. H&E  ×  10 and ×40, respectively. This patient is alive at the time of writing. (e) Cerebrospinal fluid specimen from a patient with lethal NCM. Atypical nevomelanocytes with short dendrites recapitulate the morphology of melanocytes. (f) “Metastasis” of NCM from same patient to nodules in the peritoneal cavity after ventriculoperitoneal shunting [27]

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