Cowden syndrome. Managing multiple skeletal metastases of different origin: a case report
1 Academic Orthopaedic Unit, Leeds General Infirmary, Leeds School of Medicine, Leeds, LS1 3EX, UK
2 Institute of Psychiatry, King's College London, London, UK
Cases Journal 2008, 1:265 doi:10.1186/1757-1626-1-265Published: 23 October 2008
Cowden Syndrome is a rare autosomal dominant multiple hamartomatous condition, characterised by both benign and malignant tumours affecting multiple systems.
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.
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.