Malignant melanoma in xeroderma pigmentosum patients: report of five cases
| Autor: | Figen Özgür, Yücel Güngen, K. Güler Gürsu, Yücel Erk, Oya Kocabalkan |
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| Rok vydání: | 1997 |
| Předmět: |
Adult
Male congenital hereditary and neonatal diseases and abnormalities medicine.medical_specialty Xeroderma pigmentosum Skin Neoplasms Genetic counseling Fulminant Photodermatosis Skin Diseases Disease-Free Survival Metastasis Epidemiology medicine Humans skin and connective tissue diseases Child Melanoma Pigmentation disorder Xeroderma Pigmentosum integumentary system business.industry nutritional and metabolic diseases General Medicine medicine.disease Dermatology Pedigree Cell Transformation Neoplastic Oncology Surgery business |
| Zdroj: | European journal of surgical oncology : the journal of the European Society of Surgical Oncology and the British Association of Surgical Oncology. 23(1) |
| ISSN: | 0748-7983 |
| Popis: | Xeroderma pigmentosum is a rare genetic disease transmitted via a recessive gene with an altered reaction of the epidermis to light. Fifty per cent of patients develop a skin tumour by 8 years of age. The majority of patients may have multiple tumours, but metastasis is rare. In the last 25 years we have treated 24 xeroderma pigmentosum patients in our clinic. Only five patients had developed cutaneous malignant melanoma during their follow-up. Three of the patients were from the same family, melanoma occurring in three of five affected individuals. All xeroderma pigmentosum patients with malignant melanoma had received classical treatment modalities. Except one case of fulminant pattern, all four patients had long disease-free survival. Although early detection and treatment of these cutaneous malignancies will reduce morbidity and mortality, genetic counselling remains the most important protective measure for xeroderma pigmentosum. |
| Databáze: | OpenAIRE |
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