Cowden syndrome, a rare genetic disorder estimated to occur in 1 in 200,000 live births and inherited as an autosomal dominant mutation in PTEN gene, is part of the PTEN hamartoma tumor syndrome. These patients are at risk of breast cancer, as well as cancers of the digestive tract, thyroid, uterus and ovaries. Often identified by their dentist due to characteristic papillomatosis in the gingival mucosa, they have an estimated lifetime risk of up to 81% of developing breast cancer. This article describes a relatively uncommon procedure of bi-pedicle nipple-sparing mastectomy, a modified Letterman technique, used in the setting of immediate implant based reconstruction in a patient with Cowden syndrome.
Breast cancer is one of the commonest cancers affecting women and oncoplastic breast surgery has been firmly established as the mainstay of modern surgical treatment, replacing the traditional two-operation approach. Careful patient selection, relevance of effective communication, patient education and navigating the complex decision-making process, are some of the topics covered in this chapter. Preoperative planning, implant selection, patient marking, importance of scar placement, marking and measuring the patient preoperatively, good theatre practice, technical tips for good cosmesis, and after care; are also discussed. A brief section on revision surgery following implant reconstruction, lipomodelling and a brief overview about breast implant associated anaplastic large cell lymphoma (BIA-ALCL) and multidisciplinary approach to modern management of breast cancer. It aims to serve as a guide to surgeons on current practice and achieving the ideal balance between oncological clearance of the cancer combined with good cosmesis and high levels of patient satisfaction.
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