Giant Congenital Melanocytic Nevi, Neurocutaneous Melanosis and Neurological Alterations

Dermatology ◽  
1997 ◽  
Vol 195 (2) ◽  
pp. 125-128 ◽  
Author(s):  
R. Ruiz-Maldonado ◽  
M. del Rosario Barona-Mazuera ◽  
L.R. Hidalgo-Galván ◽  
V. Medina-Crespo ◽  
C. Duràn-Mckinster ◽  
...  
Keyword(s):  
Neurocutaneous Melanosis ◽  
Melanocytic Nevi ◽  
Giant Congenital Melanocytic Nevi ◽  
Neurological Alterations ◽  
Congenital Melanocytic Nevi

scholarly journals Multiple giant congenital nevi – a case report / Multipli gigantski kongenitalni nevusi – prikaz slučaja

2011 ◽  
Vol 3 (2) ◽  
pp. 69-76
Author(s):  
Vesna Mikulić
Keyword(s):  
Body Surface ◽  
Psychosocial Problems ◽  
The Body ◽  
Skin Tumor ◽  
Whole Body ◽  
Neurocutaneous Melanosis ◽  
Melanocytic Nevi ◽  
Increased Risk ◽  
Giant Congenital Melanocytic Nevi ◽  
Congenital Melanocytic Nevi

Abstract Giant congenital melanocytic nevi are benign nevomelanocytic proliferations of 20 cm or more in diameter, present at birth. They are primarily found on the posterior trunk, but they may arise on any other part of the body, covering more than 2% of the body surface. Giant congenital nevi are major risk factors for the development of melanoma, and the risk has been estimated to be as high as 5-7%. Persons with giant congenital melanocytic nevi on the head, neck and along the midline of the back are at increased risk for leptomeningeal melanocytic lesions. Most patients with neurocutaneous melanosis present with neurologic manifestations of the disease in the first 2 years of life. Melanoma occurs in 62-80% of cases, but even without neoplasms, symptomatic neurocutaneous melanosis has a poor prognosis. This is a report of a 23-year-old female patient who presented with multiple congenital pigmented and pilous nevi covering over 2% of her total body surface, without malignant alterations or association with other abnormalities. At birth, a nevus covered her neck, shoulders and the upper left arm, whereas several nevi over 5cm in diameter were present in the gluteal region, on the abdomen and legs. During the first 2 years of life, the existing nevi increased in size and progressed into darker brown. New, smaller pigmented changes appeared on the whole body and the face, while at the age of 17 they reached their current size and layout. At puberty, nevi over 10cm in size grew dark hairs. There were neither melanoma nor skin tumor cases in the family. Nuclear magnetic resonance imaging was not performed in the childhood or later in life, but other parameters - neurologic and ophthalmologic findings were in normal range all the time, as was growth and development. A complete photo-documentation was made, including macroscopic and dermoscopic images and regular follow-ups continue. Giant congenital melanocytic nevi may cause considerable esthetic and psychosocial problems. Due to their high malignant potential, association with other abnormalities, no consensus on the treatment, and monitoring problems, giant congenital melanocytic nevi represent a therapeutic problem as well.

Neurocutaneous Melanosis with Giant Congenital Melanocytic Nevi

2016 ◽  
Vol 14 (01) ◽  
pp. 047-050
Author(s):  
Shaukat Ali ◽  
Saifullah Khalid ◽  
Kashif Azmi ◽  
Murad Ahmad ◽  
Shaad Abqari
Keyword(s):  
Neurocutaneous Melanosis ◽  
Melanocytic Nevi ◽  
Giant Congenital Melanocytic Nevi ◽  
Congenital Melanocytic Nevi

scholarly journals Giant Congenital Melanocytic Nevi and Neurocutaneous Melanosis

2015 ◽  
Vol 2015 ◽  
pp. 1-5 ◽  
Author(s):  
Catarina Araújo ◽  
Cristina Resende ◽  
Francisco Pardal ◽  
Celeste Brito
Keyword(s):  
Multiorgan Failure ◽  
Melanocytic Nevus ◽  
Congenital Melanocytic Nevus ◽  
Cerebral Magnetic Resonance Imaging ◽  
Neurocutaneous Melanosis ◽  
Melanocytic Nevi ◽  
Giant Congenital Melanocytic Nevi ◽  
Congenital Melanocytic Nevi ◽  
Leptomeningeal Infiltration ◽  
Expansive Lesion

Introduction. The major medical concern with giant congenital melanocytic nevi CMN is high risk of developing cutaneous melanoma, leptomeningeal melanoma, and neurocutaneous melanocytosis.Case Report. A 30-year-old woman with a giant congenital melanocytic nevus covering nearly the entire right thoracodorsal region and multiple disseminated melanocytic nevi presented with neurological symptoms. Cerebral magnetic resonance imaging revealed a large expansive lesion in the left frontal region. Postsurgically pathological diagnosis revealed characteristics of melanoma. Immunohistochemical examination showed S100(+), HMB45(+), MelanA(+), and MiTF(+). She received radiotherapy with temozolomide followed by two more chemotherapy cycles with temozolomide. She followed a rapidly progressive course, reflecting widespread leptomeningeal infiltration, and she died of multiorgan failure seven months after diagnosis of cerebral melanoma.Discussion. This patient was diagnosed as having a neurocutaneous melanosis with malignant widespread leptomeningeal infiltration. Diffuse spinal involvement is unusual and is described in only another patient.

scholarly journals Giant Congenital Melanocytic Nevi: An Update and Emerging Therapies

2018 ◽  
Vol 10 (1) ◽  
pp. 24-28 ◽  
Author(s):  
Girish Gulab Meshram ◽  
Neeraj Kaur ◽  
Kanwaljeet Singh Hura
Keyword(s):  
Hydrostatic Pressure ◽  
Malignant Melanoma ◽  
Rare Occurrence ◽  
Review Of The Literature ◽  
Neurocutaneous Melanosis ◽  
Melanocytic Nevi ◽  
Emerging Therapies ◽  
Giant Congenital Melanocytic Nevi ◽  
Congenital Melanocytic Nevi

Giant congenital melanocytic nevi (GCMN) are a rare occurrence. Gain-of-function mutation in the NRAS gene is found to be associated with GCMN, causing abnormal proliferation of embryonic melanoblasts. The two major complications associated with GCMN are malignant melanoma and neurocutaneous melanosis. Treatment of GCMN has conventionally been surgical. However, the role of NRAS inhibitors and inactivation of nevus tissue by high hydrostatic pressure are being explored. We present a case of a 1-day-old neonate born with GCMN, along with a review of the literature.

Neurocutaneous melanosis in children with giant congenital melanocytic nevi

2014 ◽  
Vol 38 (2) ◽  
pp. 79-84 ◽  
Author(s):  
Monika Bekiesinska-Figatowska ◽  
Orest Szczygielski ◽  
Maria Boczar ◽  
Jaroslaw Madzik ◽  
Teresa Klepacka ◽  
...  
Keyword(s):  
Neurocutaneous Melanosis ◽  
Melanocytic Nevi ◽  
Giant Congenital Melanocytic Nevi ◽  
Congenital Melanocytic Nevi

Giant Congenital Melanocytic Nevi: The Significance of Neurocutaneous Melanosis in Neurologically Asymptomatic Children

2001 ◽  
Vol 107 (4) ◽  
pp. 933-941 ◽  
Author(s):  
Robert D. Foster ◽  
Mary L. Williams ◽  
Anthony J. Barkovich ◽  
William Y. Hoffman ◽  
Stephen J. Mathes ◽  
...  
Keyword(s):  
Neurocutaneous Melanosis ◽  
Melanocytic Nevi ◽  
Asymptomatic Children ◽  
Giant Congenital Melanocytic Nevi ◽  
Congenital Melanocytic Nevi

scholarly journals Neurocutaneous melanoma in association with giant congenital melanocytic nevi in a child (Touraine syndrome) – A dermatoradiological correlation

2020 ◽  
Vol 0 ◽  
pp. 1-3
Author(s):  
Juvaina Puthiyakam ◽  
Najeeba Riyaz ◽  
Faiz Riyaz Arakkal ◽  
Riyaz Arakkal
Keyword(s):  
Melanocytic Nevus ◽  
Neurocutaneous Melanosis ◽  
Melanocytic Nevi ◽  
Mri Brain ◽  
The Face ◽  
Giant Congenital Melanocytic Nevi ◽  
The Right ◽  
Congenital Melanocytic Nevi

Touraine syndrome or neurocutaneous melanosis/melanoma is a rare melanophakomatosis characterized by extensive/multiple congenital melanocytic nevi associated with cerebral/meningeal melanosis or melanoma. We report a 12-year-old boy with a congenital giant melanocytic nevus on the bathing trunk distribution with scattered lesions on the face, neck, and legs. MRI brain revealed a melanoma in the right amygdala.

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