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Intersection of scalp melanoma and nurofibromatosis type 1 rare case report
INTRODUCTION AND IMPORTANCE: Type 1 neurofibromatosis is a multisystem disease, mainly involving the nervous system and skin. Complications of type 1 neurofibromatosis are the development of neural crest derived malignancies such as melanoma of the skin. Skin melanomas have been found in 0.1 %–5.4 %...
Autores principales: | , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Elsevier
2023
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10149260/ https://www.ncbi.nlm.nih.gov/pubmed/37094414 http://dx.doi.org/10.1016/j.ijscr.2023.108186 |
Sumario: | INTRODUCTION AND IMPORTANCE: Type 1 neurofibromatosis is a multisystem disease, mainly involving the nervous system and skin. Complications of type 1 neurofibromatosis are the development of neural crest derived malignancies such as melanoma of the skin. Skin melanomas have been found in 0.1 %–5.4 % of NF1 patients and it's far less likely to develop in Black people. CASE PRESENTATION: We present a 53 years old female known to be a case of NF-1 with multiple neurofibromas. One of them in the scalp since the age of 12. Two months prior to presentation, the scalp neurofibroma start to increase in size rapidly and multiple ulcers developed. CT scan showed soft tissue mass not attached to the bone or other cranial structure. The patient underwent excision with safety margins with a full thickness skin graft. Histopathology showed a free edge of the tumor and was initially suggestive of spindle cell sarcoma then immune-histo-chemistry requested the result showed, melanoma of the scalp. The follow-up showed a good take of skin graft and the patients was sent to oncology for further management. CLINICAL DISCUSSION: Melanoma is a rare malignancy in patients with NF1, but it can occur. It is important to be aware of the possibility of melanoma in these patients and to perform regular skin checks. Treatment of melanoma in NF1 patients follows the same guidelines as for non-NF1 patients, with wide or narrow excision margins depending on the type of melanoma. Reconstruction of the defect can be done with split-thickness or full-thickness skin grafts, depending on the size and location of the defect. CONCLUSION: Melanoma is a devastating skin malignancy and should be in suspicion for any skin lesion that present even in association with rare diseases. |
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