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Open Access Case Report

Cowden syndrome. Managing multiple skeletal metastases of different origin: a case report

Antonios Angoules1, Evangelia M Tsapakis2, Ioannis Polyzois1, Zakareya Gamie1, James J Rankine1 and Eleftherios Tsiridis1*

Author Affiliations

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

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Cases Journal 2008, 1:265 doi:10.1186/1757-1626-1-265

Published: 23 October 2008

Abstract

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.