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Cowden syndrome. Managing multiple skeletal metastases of different origin: a case report
BACKGROUND: Cowden Syndrome is a rare autosomal dominant multiple hamartomatous condition, characterised by both benign and malignant tumours affecting multiple systems. CASE PRESENTATION: We present a 47-year-old female patient with thigh pain that was diagnosed with Cowden syndrome 20 years ago an...
Autores principales: | , , , , , |
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Formato: | Texto |
Lenguaje: | English |
Publicado: |
BioMed Central
2008
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2584651/ https://www.ncbi.nlm.nih.gov/pubmed/18947411 http://dx.doi.org/10.1186/1757-1626-1-265 |
Sumario: | BACKGROUND: Cowden Syndrome is a rare autosomal dominant multiple hamartomatous condition, characterised by both benign and malignant tumours affecting multiple systems. CASE PRESENTATION: We present a 47-year-old female patient with thigh pain that was diagnosed with Cowden syndrome 20 years ago and developed multiple and different skeletal metastases which became resistant to radio-chemotherapy. A percutaneous plate fixation of the distal femur with an intralesional excision and cementoplasty of the metastasis was performed initially. This was combined with a cemented total hip arthroplasty using an Exeter long revision stem and a cementoplasty of the femoral canal for the proximal lesions. CONCLUSION: A meticulous approach to her complex metastatic disease resulted in successful palliative prophylactic reconstructive surgery that improved her quality of life, allowing her independent pain free walking for 12 months. |
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