A 42 year old female patient, with no personal history of interest, was referred by her GP to the Oral and Maxillofacial Surgery Department of the Hospital Universitario Virgen de las Nieves in Granada for presenting multiple papillomatous lesions on the upper lip which, according to the patient, had been present for approximately fifteen years. The patient's intraoral examination also revealed the presence of a scrotal tongue. Under local anaesthesia and in two surgical procedures, the lesions were completely excised. Pathological examination of the lesions classified them as fibropapillomas.

Due to the characteristic cobblestone appearance of the mucosal lesions, accompanied in the systemic exploration by cutaneous trichilemmomas in acral regions, the long period of evolution of the lesions and the presence of a direct family history (mother who died of thyroid neoplasia at the age of 49 with the presence of the same type of lesions on the labial mucosa, sister with non-filial mammary pathology, and sister with a non-filial mammary pathology), the patient was diagnosed with fibropapillomas, sister with unaffiliated breast pathology and daughter operated on for papillary thyroid carcinoma) it was decided to refer the patient to the Gynaecology Department for screening for neoplastic breast pathology, given the strong suspicion, among the differential diagnoses proposed, of Cowden's syndrome.

At the gynaecology department of our hospital, the patient was diagnosed with bilateral fibrocystic breast disease and infiltrating ductal carcinoma grade GII of the left breast, requiring lumpectomy plus homolateral axillary emptying and complementary treatment with postoperative chemo-radiotherapy. Given the clinical diagnosis of multiple hamartoma genodermatosis (the patient had one pathognomonic lesion, one major criterion and three minor criteria), a complete systemic tumour screening process was initiated.
The patient was evaluated in the Digestive Service, where the presence of asymptomatic polyps in the rectal and colonic mucosa was detected and she was referred to the Endocrinology Service for evaluation of progressive thyroid enlargement associated with clinical hyperthyroidism. After ruling out malignancy by cytohistological study, the patient was operated on by the Endocrine Surgery Service and underwent total thyroidectomy. The conclusive anatomopathological result diagnosed the lesion as a multinodular colloid goitre.
Five months after the thyroid surgery and after a routine abdominopelvic ultrasound scan, a mass was detected in the right kidney which, after a CT scan, showed radiological characteristics of malignancy. Referred to the Urology Department, it was decided to perform a radical right nephrectomy plus supracaval lymphadenectomy. Histological study of the surgical specimen diagnosed the lesion as clear cell and granular renal carcinoma with a mainly tubular component. No additional post-surgical treatment was required.
At present, the patient has a progressive decrease in visual acuity which is not being studied by the Ophthalmology Department.
All the neoplastic processes diagnosed and treated were detected in early stages. At the moment the patient is in complete remission of all the tumour pathology diagnosed and treated. Despite having a clear clinical diagnosis almost from the beginning, the patient has been studied at the National Centre for Oncological Research [Department of Human Genetics], confirming definitively that she is a carrier of the c1093lnaGGAT mutation in the PTEN gene, which ratifies the genetic diagnosis of Cowden Syndrome. The study has been extended to the rest of the first-degree relatives in order to carry out an adequate screening and initiate a close clinical follow-up if necessary.