Medium-Sized Congenital Melanocytic Nevus with Halo Phenomenon: A Report of Two Cases

Congenital melanocytic nevi with halo phenomenon in children is a rare clinical finding. We report two cases of children who developed depigmentation within medium-size congenital melanocytic nevi. Clinicians should be suspicious when confronted with this phenomenon due to the risk of development into malignant melanoma. Herein, we review these rare cases of medium-sized halo congenital melanocytic nevi with and without associated vitiligo, with emphasis that depigmentation does not necessarily indicate malignant growth.

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Giant congenital melanocytic nevi (garment variety): a case report

International Journal of Contemporary Pediatrics ◽

10.18203/2349-3291.ijcp20213333 ◽

2021 ◽

Vol 8 (9) ◽

pp. 1625

Author(s):

Om Prakash Singh ◽

Vikas Kumar ◽

Rahul Kumar

Keyword(s):

Malignant Melanoma ◽

Psychological Intervention ◽

Skin Lesions ◽

Melanocytic Nevus ◽

Congenital Melanocytic Nevus ◽

Melanocytic Nevi ◽

Black Skin ◽

Giant Congenital Melanocytic Nevi ◽

And Migration ◽

Congenital Melanocytic Nevi

Giant congenital melanocytic nevi (GCMN) are large brown-to-black skin lesions caused due to genetic mutations which lead to defective proliferation, differentiation and migration of melanoblasts which are precursor cells of melanocytes. There is a mutation in the NRAS gene causing abnormal proliferation of embryonic melanoblasts. Congenital melanocytic nevus is primarily a clinical diagnosis. The malignant melanoma and neurocutaneous melanosis are the two major complications associated with GCMN. The risk of transformation of GCMN to malignant melanoma varies between 0 and 3.8%. About 1% of live births presents with a CMN. The incidence of GCMN is estimated at less than 1: 20,000 newborns. The variety ‘garment-like’ of GCMN is even scarcer, 1: 5,00,000. GCMN has got major psychosocial impact on the patient and his family due to its unsightly appearance. Treatment includes surgical and non-surgical procedures, psychological intervention and clinical follow-up, with special attention to changes in color, texture on the surface of the lesion. We presented a case of 1-day-old female neonate born with GCMN in our hospital.

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Congenital Melanocytic Hairy Nevi in a Child from North-Western Nigeria

Asian Journal of Medicine and Health ◽

10.9734/ajmah/2020/v18i730221 ◽

2020 ◽

pp. 28-34

Author(s):

Fatima Bello Jiya ◽

Maryam Amodu- Sanni ◽

Nma Muhammed Jiya ◽

Dada Muhammed Aquib ◽

Muhammed Umar ◽

...

Keyword(s):

Melanocytic Nevus ◽

Temporal Region ◽

Healthy Children ◽

Congenital Melanocytic Nevus ◽

Melanocytic Nevi ◽

Lower Back ◽

The Face ◽

Congenital Melanocytic Nevi ◽

North Western ◽

Apparently Healthy

Aim: To present the first report of a large congenital melanocytic nevus with satellite nevi in an apparently healthy child from Sokoto, North-Western Nigeria. Presentation of Case: A three year old girl was brought to the paediatric out-patient clinic of Paediatrics department of Usmanu Danfodiyo University Teaching Hospital (UDUTH) Sokoto with complaints of darkened skin colour on the left side of the face and scalp, the left arm, lower back, buttocks, and thighs, and excessive hair growth over the same side of the face since birth. There were no neurological symptoms Physical examination findings revealed a well-nourished, not ill looking child. She had a hyper pigmented patch on the left side of the face extending from the lower jaw to the scalp, measuring 21 cm in its longest length, with hypertrichosis on the same site, and two distinct, firm, painless nodular lesions on the left temporal region measuring 3 mm and 4mm respectively. On the lower one-third of the left arm was a hairy, velvety area of hyperpigmentation measuring 2X3 cm in diameter. Other affected sites were the lower back, the gluteal region and the thighs. Her neurologic and other systemic examinations were normal. A diagnosis of large congenital facial melanocytic hairy nevus with multiple satellite nevi was made. Discussion: Congenital melanocytic nevi are benign proliferations of melanocytic cells said to be present at birth or in the first two years of life. Large lesions are rare, they measure 20 cm or more and are said to occur more commonly on the trunk and thighs. The exact pathogenesis of congenital melanocytic nevi is yet, unknown. It is thought to occur as a result of a morphological error in the neuroectoderm during embryogenesis. Treatment of patients with large congenital melanocytic nevus may include surgical or non-surgical procedures as well as psychological interventions. Large lesions, multiple satellite lesions or paravertebral and axial locations are sometimes associated with the risk of neurological complications and malignant transformation. Conclusion: Large congenital melanocytic nevi are uncommon skin lesions that can occur in apparently healthy children. Individualization of the patients with regards to treatment options and long term monitoring are imperative.

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Giant Congenital Melanocytic Nevus (GCMN) - A New Hope for Targeted Therapy?

Open Access Macedonian Journal of Medical Sciences ◽

10.3889/oamjms.2017.121 ◽

2017 ◽

Vol 5 (4) ◽

pp. 549-550 ◽

Cited By ~ 1

Author(s):

Georgi Tchernev ◽

Ilia Lozev ◽

Ivan Pidakev ◽

Torello Lotti ◽

Uwe Wollina ◽

...

Keyword(s):

Melanocytic Nevus ◽

Whole Body ◽

Occurrence Rate ◽

Lateral Part ◽

Congenital Melanocytic Nevus ◽

Older Children ◽

Melanocytic Nevi ◽

Pigmented Lesion ◽

Surgical Methods ◽

Congenital Melanocytic Nevi

We present a 6-month-old male patient, who was consulted with dermatologist by his parents, because of a pigmented lesion, present since birth, covering almost the all skin of the back and buttocks. A sharply bordered, unequally coloured congenital pigmented nevus, measuring approximately 21 cm in diameter was observed in the whole body skin examination. The lesion was affecting the lower 2/3 of the skin of the back and the top half of the gluteus area, extending to the lateral part of the tors, forward the abdomen and the upper lateral part of the hips, composed by multiple darker-pigmented nests and several lighter areas, with single depigmented zones, hairy surface, irregularly infiltrated on palpation. Congenital melanocytic nevi are presented in approximately 1% of newborns, while giant congenital melanocytic nevi (GCMN) are the most uncommon subtype of them; with occurrence rate 1 in 50,000 births. They affect 2% of a total body surface or presenting in a diameter larger than 20 cm in older children. Although not common, the possible malignant transformation remains one of the most important considerations related to them, as the related lifetime risk of melanoma is 4% to 10%. Treatment recommendations include non-surgical methods as dermabrasion only within the first two weeks of life, for prevention the possible melanocytic deeper migration, while serial surgical excisions or tissue expanders could be useful treatment tool even in later stages. Nevertheless, cosmetic result is not always satisfactory, and the risk of malignant changes remains, in cases of previous melanocytic migration in deeper layer. Recent article suggests the potential role in the treatment of GCMN with NRAS inhibitor trametinib, approved for treatment of advanced melanoma, associated with underlying NRAS mutations. Although promising, the drug could be useful in paediatric patients, only with associated NRAS gene mutation. It is still unclear whether it could be helpful, independent of the NRAS status.

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Nonpigmented Metastatic Melanoma in a Two-Year-Old Girl: A Serious Diagnostic Dilemma

Case Reports in Oncological Medicine ◽

10.1155/2015/298273 ◽

2015 ◽

Vol 2015 ◽

pp. 1-3

Author(s):

Gulden Diniz ◽

Hulya Tosun Yildirim ◽

Selcen Yamaci ◽

Nur Olgun

Keyword(s):

Differential Diagnosis ◽

Malignant Melanoma ◽

Metastatic Melanoma ◽

Subcutaneous Tissue ◽

Melanocytic Nevus ◽

Congenital Melanocytic Nevus ◽

Diagnostic Dilemma ◽

Flow Cytometric ◽

Increased Risk ◽

Congenital Melanocytic Nevi

Although rare, malignant melanoma may occur in children. Childhood melanomas account for only 0.3–3% of all melanomas. In particular the presence of congenital melanocytic nevi is associated with an increased risk of development of melanoma. We herein report a case of malignant melanoma that developed on a giant congenital melanocytic nevus and made a metastasis to the subcutaneous tissue of neck in a two-year-old girl. The patient was hospitalized for differential diagnosis and treatment of cervical mass with a suspicion of hematological malignancy, because the malignant transformation of congenital nevus was not noticed before. In this case, we found out a nonpigmented malignant tumor of pleomorphic cells after the microscopic examination of subcutaneous lesion. Nonpigmented metastatic melanoma was diagnosed by several immunohistochemical and flow cytometric studies. She was offered palliative chemotherapy; however, her parents did not accept treatment. The patient died within 9 months of diagnosis. We emphasized here that the possibility of malignant melanoma in the differential diagnosis of childhood tumors should be kept in mind.

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Combination of preoperative embolization and surgery in the treatment of a giant congenital neuroid nevus of the forehead and scalp in a child

Journal of Neurosurgery Pediatrics ◽

10.3171/2008.11.peds08146 ◽

2009 ◽

Vol 3 (3) ◽

pp. 215-219

Author(s):

Alexander Margulis ◽

Benjamin Z. Koplewitz ◽

Alexander Maly ◽

José Cohen ◽

Ravit Yanko-Arzi ◽

...

Keyword(s):

Male Infant ◽

Large Mass ◽

Melanocytic Nevus ◽

Preoperative Embolization ◽

Congenital Melanocytic Nevus ◽

Complete Excision ◽

Melanocytic Nevi ◽

Giant Congenital Melanocytic Nevi ◽

Congenital Melanocytic Nevi ◽

Staged Reconstruction

Congenital melanocytic nevi of neuroectodermal origin are composed primarily of melanocytes and occasional neural elements. A subset of large/giant congenital melanocytic nevi is characterized by neuroidal differentiation resembling the histological pattern of neurofibroma (neuroid congenital melanocytic nevi). The authors report the case of a male infant born with a neuroid congenital melanocytic nevus presenting as a large mass covering extensive portions of his scalp and forehead. The atypical feeding arteries to the nevus were larger in diameter then the patient's internal carotid arteries and formed a rich network of large blood vessels in the nevus. Selective preoperative embolization using calibrated particles was followed by a complete excision and staged reconstruction with tissue expanders. The authors believe that this multidisciplinary approach allowed for a safe surgical procedure with minimal blood loss for this most unusual pediatric head and neck tumor.

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Congenital melanocytic nevus of the oral mucosa: report of a rare pigmented lesion and review of the literature

Clinics and Practice ◽

10.4081/cp.2011.e17 ◽

2011 ◽

Vol 1 (1) ◽

pp. 17 ◽

Cited By ~ 4

Author(s):

Melanie Louise Gilbert ◽

Weddad Hanna ◽

Danny Ghazarian ◽

Dean Dover ◽

Hagen Benjamin Edward Klieb

Keyword(s):

Oral Mucosa ◽

Melanocytic Nevus ◽

Congenital Melanocytic Nevus ◽

Review Of The Literature ◽

Melanocytic Nevi ◽

Pigmented Lesions ◽

Pigmented Lesion ◽

Congenital Melanocytic Nevi

Oral pigmented lesions are uncommon and congenital melanocytic nevi are especially rare. We report a case of a patient with multiple congenital melanocytic nevi including a palatal lesion. This is reported to add to the scant literature that exists on this subject. Prognosis and management are discussed.

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Giant Congenital Melanocytic Nevi and Neurocutaneous Melanosis

Case Reports in Medicine ◽

10.1155/2015/545603 ◽

2015 ◽

Vol 2015 ◽

pp. 1-5 ◽

Cited By ~ 4

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.

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Late Onset Achromatic Melanoma Arising in a Giant Congenital Melanocytic Nevus

Open Access Macedonian Journal of Medical Sciences ◽

10.3889/oamjms.2017.077 ◽

2017 ◽

Vol 5 (4) ◽

pp. 533-534 ◽

Cited By ~ 1

Author(s):

Georgi Tchernev ◽

James W. Patterson ◽

Ilko Bakardzhiev ◽

Torello Lotti ◽

Jacopo Lotti ◽

...

Keyword(s):

Late Onset ◽

Surgical Excision ◽

Melanocytic Nevus ◽

Secondary Development ◽

Surgical Removal ◽

Congenital Melanocytic Nevus ◽

Melanocytic Nevi ◽

Giant Congenital Melanocytic Nevi ◽

Congenital Melanocytic Nevi ◽

Giant Congenital Melanocytic Nevus

A 61-year-old woman, with a lifelong history of a giant congenital melanocytic nevus in the occipital region with secondary development of giant melanoma is presented. Surgical excision was performed, and the histopathological evaluation confirmed the diagnosis of Giant Malignant Melanoma (GMM) with a maximum tumour thickness of 16 mm. Nowadays, there is tremendous uncertainty regarding how giant congenital melanocytic nevi (GCMN) should be treated. The standard approach to patients with late onset giant congenital melanocytic nevi (GCMN is based on two main considerations: (1) obtain an acceptable cosmetic results with the purpose to decrease the psychosocial inconvenience to each patient, and (2) to attempt to minimise the risk of development of malignant transformation. Unfortunately complete surgical removal of the GCMN is usually difficult and very often impossible without subsequent functional or cosmetic mutilations.

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Giant congenital melanocytic nevus: a rare case report

International Journal of Contemporary Pediatrics ◽

10.18203/2349-3291.ijcp20204407 ◽

2020 ◽

Vol 7 (11) ◽

pp. 2236

Author(s):

Arty Tyagi ◽

Bipin Kumar ◽

Mani Kant Kumar

Keyword(s):

Rare Case ◽

Melanocytic Nevus ◽

Melanocytic Lesion ◽

Congenital Melanocytic Nevus ◽

Melanocytic Nevi ◽

Rare Case Report ◽

Giant Congenital Melanocytic Nevi ◽

Magnetic Resonance Imaging Mri ◽

Congenital Melanocytic Nevi ◽

Giant Congenital Melanocytic Nevus

Giant congenital melanocytic nevus is usually defined as a melanocytic lesion present a birth. Its incidence is estimated in <1 in 20,000 newborns. Despite its rarity, this lesion is important because it may be associate with severe complications such as malignant melanoma and may also cause neurological deficit including neurocutaneous melanocytosis. Giant congenital melanocytic nevus generally present as a brown lesion with flat or mammilated surface, well-demarcated borders and hypertrichiosis. Congenital melanocytic nevus is primarily a clinical diagnosis. Recently, we encountered a 3 day old baby girl in whom giant congenital melanocytic nevi was noted at birth. She presented with large nevus over posterior trunk, head and extremities. Magnetic resonance imaging (MRI) brain was normal. Parents were counselled regarding possible future course and were asked to be in regular follow-up. Here, we report this rare case with giant congenital melanocytic nevi.

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