Squamous Cell Carcinoma of the Vulva in Turner Syndrome

Abstract

Turner syndrome (gonadal dysgenesis) is an important cause of short stature in girls and primary amenorrhoea, which is usually caused by loss of part or all the X-chromosome. It is well known that women with Turner syndrome are liable to develop various diseases such as autoimmune diseases like thyroiditis, and cardiovascular, urologic and bone structural diseases in addition to hypertension and diabetes 1 However the risk of cancer, with the exception being in the case of colonic and rectal cancers, does not seem to be elevated in Turner syndrome 1. Carcinoma of the vulva is a rare gynaecological cancer and in Turner syndrome it is extremely rare. We report a case of squamous cell carcinoma (SCC) of the vulva in a 61 year old Caucasian woman diagnosed with Turner syndrome at the age of 15. Radical vulvectomy with en-block inguinal femoral lymphadenectomy was carried out. Histopathological examination of the specimen confirmed complete excision of the tumour with absence of metastasis to the lymph nodes. Conclusion: This seems to be the first reported case of SCC of the vulva in a patient with Turner syndrome.