Giant congenital melanocytic naevus with proliferative nodules mimicking congenital malignant melanoma: a case report and review of the literature of congenital melanoma (original) (raw)
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European Journal of Plastic Surgery, 2006
A 17-month-old girl presented with a mediumsized congenital melanocytic naevus on the dorsum of the right foot. Histological examination of the excised specimen revealed a malignant melanoma (Breslow thickness 3.2 mm, Clark level IV) in a compound naevus. Wider excision with a 20-mm margin, according to protocol, followed. No metastases were discovered on complete systemic investigation. The patient remains tumour-free, 5 years later. The clinical dilemma posed by the mediumsized melanocytic naevus, in relation to small and large congenital melanocytic naevi is discussed.
Metastatic Melanoma in Association With a Giant Congenital Melanocytic Nevus in an Adult
The American Journal of Dermatopathology, 2015
Giant congenital melanocytic nevi (GCMNs) represent a distress to patients for 2 reasons: one is disfigurement, and the other is the increased risk of developing secondary melanocytic tumors, such as benign proliferative nodules (BPNs) and malignant melanoma (MM). BPN present as a rapid growth nodule arising within a congenital melanocytic nevus (CMN) that often ulcerates, occurs in children younger than 2 years of age. BPNs arising within a CMN are exceedingly rare after childhood, and very few cases have been described in adults. Despite the worrisome clinical and histologic findings of BPN, most laboratory investigations seem to support their benignity. The distinction between MM and BPN is extremely important, but the histopathology of BPN of GCMN can be a challenge to differentiate from MM. In the recent years, molecular tests that investigate DNA copy number alterations such as fluorescence in situ hybridization and comparative genomic hybridization have shown promise to help guide the diagnosis of ambiguous melanocytic proliferations arising within CMNs. We report the case of a 22-year-old woman with a nodule arising in a GCMN and with an axillary mass suggesting a nodal metastasis of melanoma, and discuss the unusual clinical, histopathologic, and molecular findings that make this case particularly interesting.
Melanoma on Congenital Nevi: Case Report
Introduction: Congenital nevi occur in about 1% of newborns. The most used classification is based on their size; small congenital naevus when the largest diameter is less than 1.5cm, intermediate naevi for those between 1.5 and 19.9cm, wide naevus when the diameter is greater than 20cm, and giant naevus when the size is greater than 40cm, with frequent satellite lesions. The risk of transformation into melanoma is estimated at 6.3% for giant nevi, it usually occurs before the age of 20, hence the indication of early excision. This risk is even lower as the size of the nevus is small, and the transformation is usually late. We report the case of a patient with melanoma occurring on a congenital melanocytic naevi of intermediate size. Case Presentation: We report the case of a 54-year-old patient, with no pathological antecedents, who was born with a congenital nevus of the right shoulder with onset 7 months ago of a nodule within the old lesion, increasing rapidly of which the histological study had concluded to a melanoma. Discussion: Melanoma can also occur in congenital nevi less than 10cm in size. They can be considered as precursors of melanoma. Because of their large number and frequency, prophylactic excision of all these nevi is not feasible. However, they should be removed as soon as possible when clinical or dermoscopic changes are observed.
Australasian Journal of Dermatology, 2012
Large congenital melanocytic naevi (LCMN) are associated with an increased risk of malignancy and neurocutaneous melanocytosis (NCM). This Australian study aims to assess both the clinical characteristics of LCMN and the risks associated with it. The authors reviewed medical records of the Sydney Children's Hospital Dermatology Outpatient Clinic for the past 10 years and identified 31 eligible patients. A total of 14 boys and 17 girls with a median age of 0.13 years were assessed; 18 lesions were on the trunk, five were on the head, five were on the lower limbs and three were on the upper limbs. In all 20 patients had satellite naevi (the median number of the satellite naevi was 7.5). The patients were followed up for a median duration of 12 months. Central nervous system magnetic resonance imaging was performed on 19 patients and two (6.5%) were found to have NCM. Biopsies were performed on five patients; one patient (3.2%) was found to have benign proliferative nodules of undifferentiated spindle cells but no patient (0%) was found to have a malignancy. The clinical characteristics for the two patients with NCM and the patient with benign proliferative nodules suggest that the risk of both NCM and benign proliferative nodules may be greater with an increased number of satellite naevi and with the LCMN being larger in size.
Melanoma arising in a Giant congenital melanocytic nevus: two case reports
Diagnostic Pathology, 2019
Background: A giant congenital melanocytic nevus (GCMN) is found in 0.1% of live-born infants. If present, the lesion has a chance of about 6% to develop into malignant melanoma. Both children and adults can be affected by malignant melanoma arising in a giant congenital nevus. Up to 95% of GCMNs harbor NRAS mutations, and mutations in the BRAF, MC1R, TP53, and GNAQ genes have also been described. The individualization of therapy is required, but diagnostic and prognostic criteria remain controversial. Case presentations: We report two cases: 1) melanoma arising in a giant congenital nevus during the first month of life complicated with neurocutaneous melanosis (NCM), and 2) melanoma arising in a giant congenital nevus during the first 6 months of life. Pathology, immunohistochemistry, and genetic analyses of tumor tissue were performed. The first case revealed only a non-pathogenic P72R polymorphism of the TP53 gene in the homozygote condition. For the second case, a Q61K mutation was detected in the NRAS gene. Conclusion: Malignant melanoma associated with GCMN is rare and therefore poorly understood. Outcomes have been linked to the stage at diagnosis, but no additional pathological prognostic factors have been identified. The most frequent genetic event in giant CMNs is NRAS mutations, which was discovered in one of our cases. To accumulate evidence to improve disease prognosis and outcomes, children with congenital melanocytic nevus should be included in a systemic follow-up study from birth.
A Rare Case of Giant Congenital Melanocytic Nevi: A Case Report
Bioscientia Medicina : Journal of Biomedicine and Translational Research
Background: Giant congenital melanocytic nevi are often characterised as melanocytic lesions present at birth. It is thought that it affects 1 in 20,000 births. The lesion is significant because it may be associated with serious consequences, such as malignant melanoma, and may also result in neurological deficits, such as neurocutaneous melanocytosis. This study aimed to present the case of giant congenital melanocytic nevus as a rare case. Case presentation: This study reported a 14-year-old female reported with large blackish rough spots on the trunk that were neither itchy or painful since birth. The patient had no previous history of convulsions, headaches, or vomiting. Plaque hyperpigmentation, macula hyperpigmentation, and hypertrichosis were found on the trunk. Dermoscopy show reveals brown-black homogeneity. Histopathology examination reveals brown-pigmented nevus cells distributed diffusely, with some nevus cells surrounding adnexa skin. Conclusion: Regular examination is ...
Oxford Medical Case Reports
Melanoma is a malignancy that develops from pigment-producing cells known as melanocytes. Although it is considered one of the most fatal tumors in the world, its early diagnosis is associated with a better prognosis and overall survival. A 49-year-old man was admitted to the dermatology department with a 15 cm lesion on the left arm. It was growing on a congenital nevus to reach an enormous size. Surgical excision was not possible and the treatment was controversial as the patient showed unpredicted resistance to immunotherapy. Further evaluations revealed false-negative BRAF mutation, which completely changed the course of treatment. Cutaneous melanoma is a rare malignancy, accounting only for 1% of skin cancer cases, and having it arising on a pre-existing congenital nevus is even much rarer. Although there is no decisive definition of giant melanoma, some authors define it as a lesion of more than 10 cm in diameter. Through the literature, only a few cases of giant melanoma on t...