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Persistent Intracranial Hypertension and Severe Hypoglycorrhachia in an Adult With Giant Congenital Melanocytic Naevi.

Cureus

December 2023

Department of Internal Medicine (Neurology), School of Medical Sciences, Universiti Sains Malaysia, Kubang Kerian, MYS.

A 29-year-old female, with giant congenital melanocytic naevi (GCMN) presented with a-year history of symptoms and signs of intracranial hypertension. Investigations revealed raised cerebrospinal fluid (CSF) pressure and severe hypoglycorrhachia (low CSF glucose) without pleocytosis. Initial contrast-enhanced brain MRI was normal, but a repeat MRI after a year showed meningeal enhancement with mild communicating hydrocephalus.

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Missense mutation of NRAS is associated with malignant progression in neurocutaneous melanosis.

Acta Neuropathol Commun

January 2024

Department of Neurosurgery, Brain Research Institute, Niigata University, 1-757 Asahimachi, Chuo-ku, 951-8585, Niigata, Japan.

Neurocutaneous melanosis (NCM) is a rare congenital neurocutaneous syndrome characterized by congenital melanocytic nevus of skin and abnormal proliferation of leptomeningeal melanocytes. Early acquisition of post-zygotic somatic mutations has been postulated to underlie the pathogenesis of NCM. The pathogenesis of NCM remains to be fully elucidated, and treatment options have not been established.

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Introduction: Melanosis peritonei is an exceptionally benign condition of uncertain origin marked by the deposition of dark pigments on the peritoneal cavity. It's usually associated with other abnormalities and must be differentiated from metastatic melanoma.

Case Presentation: We report this case of a 67-year-old female presented for abdominal distension for 16 months.

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Melanosis tubae et ovarii: An overlooked entity?

Indian J Pathol Microbiol

February 2022

Department of Gynecology and Obstetrics, Maulana Azad Medical College, New Delhi, India.

Melanosis is a rare benign entity defined by the deposition of brownish-black pigment in a wide variety of organs especially the gastrointestinal tract, peritoneum, fallopian tubes and ovaries. Melanosis of the fallopian tube is widely known as pigmentosis tubae. Literature has mentioned ovarian teratomas and serous cystadenomas to be associated with the ovarian and peritoneal melanosis.

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