Metastatic peritoneal neurocutaneous melanocytosis

Mariana M. Cajaiba, Denis Benjamin, Ruth Halaban, Miguel Reyes-Múgica*

*Corresponding author for this work

Research output: Contribution to journalArticle

14 Scopus citations

Abstract

Neurocutaneous melanosis, better referred to as neurocutaneous melanocytosis (NCM), is a rare congenital disorder occurring in childhood characterized by proliferation of melanocytes in the central nervous system (CNS), associated with large congenital melanocytic nevi. The phenotype of the CNS lesions varies, ranging from that of a benign, nevuslike lesion, to one of an aggressive-looking, atypical cell proliferation; however, specific diagnostic criteria allow the distinction from CNS metastasis of a primary skin melanoma. NCM can present with severe neurologic manifestations, and usually has a relentless clinical progression whence neurologic symptoms appear. Dissemination to the peritoneal surface by ventriculo-peritoneal shunting has been exceptionally observed, and we describe 2 cases of such occurrence, one of which was associated with a "bulky perineal nevocytoma" with complex cytogenetic rearrangements. This "metastatic" spreading supports an aggressive phenotype, able to seed and establish new colonies, although only after facilitated translocation of the proliferating cells through the shunt conduit; the aggressiveness of these lesions in our cases is further supported by the histopathologic features and clinical course. The biologic features of NCM cells merit further exploration, as they may shed light on a much more frequent neoplastic neurocristopathy, namely, malignant melanoma.

Original languageEnglish (US)
Pages (from-to)156-161
Number of pages6
JournalAmerican Journal of Surgical Pathology
Volume32
Issue number1
DOIs
StatePublished - Jan 1 2008

Keywords

  • Childhood neoplasia
  • Melanocytosis
  • Melanoma
  • Neural crest
  • Neurocutaneous melanosis
  • Nevus

ASJC Scopus subject areas

  • Anatomy
  • Surgery
  • Pathology and Forensic Medicine

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    Cajaiba, M. M., Benjamin, D., Halaban, R., & Reyes-Múgica, M. (2008). Metastatic peritoneal neurocutaneous melanocytosis. American Journal of Surgical Pathology, 32(1), 156-161. https://doi.org/10.1097/PAS.0b013e3181238cd2