Melanoma arising in a Giant congenital melanocytic nevus: two case reports

Abstract 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

Clinical Features of Cutaneous Acute Graft-versus-Host Disease after Allogeneic Hematopoietic Stem Cells Transplantation in Children with Hemato-Oncological Diseases

The graft-versus-host disease (GvHD) is frequent complication, it occurs in 50% of patients after allogeneic hematopoietic stem cell transplantation (HSCT) and it is one of the major causes of mortality not associated with disease recurrence. Skin lesion in the symptom complex of acute GvHD develops within the first 100 days after HSCT, and it is complicated diagnostic and therapeutic problem. Significant immunosuppressive status of children during the posttransplant period enhances and changes the course of dermatoses, infections, drug toxicity. Finally, it can lead to immunoallergic processes with possible development to generalized life-threatening diseases of the skin and mucous membranes. Meanwhile, toxic, allergic and infectious skin lesions can be present simultaneously or develop sequentially. The description of the clinical picture of skin lesions in acute GvHD is really crucial and has scientific and practical significance due to relatively small frequency of HSCT in children with oncology diseases. The article summarizes data on etiology, pathogenesis, clinical forms, diagnostic and treatment methods of cutaneous complications of the early post-transplantation period after HSCT

Дерматологическая токсичность высоких доз тиотепы у детей. Описание клинического случая

Hematopoietic stem cell transplantation (HSCT) is a treatment modality for a number of severe malignant and non-neoplastic diseases. Autologous hematopoietic stem cell transplantation (auto-HSCT) improves outcomes in patients with solid and hematological malignancies. Skin lesions at the auto-HSCT stage are quite common and represent an important diagnostic and therapeutic problem. The most significant causes of skin lesions in auto-HSCT are drug toxicity, infectious and viral lesions. Each of the complications can manifest itself to varying degrees as well as combine with others, having a significant negative on the patient’s condition, posing a threat to the patient’s life in severe cases.Трансплантация гемопоэтических стволовых клеток (ТГСК) является методом терапии ряда тяжелых злокачественных и неопухолевых заболеваний. Аутологичная трансплантация гемопоэтических стволовых клеток (ауто-ТГСК) улучшает исходы у пациентов со злокачественными новообразованиями солидной и гематологической природы. Поражения кожи на этапе ауто-ТГСК встречаются достаточно часто и представляют собой важную диагностическую и терапевтическую проблему. Наиболее значимыми причинами поражений кожи при ауто-ТГСК являются медикаментозная токсичность, инфекционные и вирусные поражения. Каждое из осложнений может проявляться в различной степени, а также сочетаться с другими, оказывая значимое отрицательное влияние на состояние пациента, в тяжелых случаях представляя угрозу для жизни пациента