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+A 19-year-old woman presented with a lump in her right breast.
+She had macrocephaly and milimetric papules on the tongue giving a cobble stone appearance.
+The breast ultrasonography (US) showed more than twenty circumscribed, oval masses in each breast.
+An US-guided core biopsy was performed for the palpable masses which were confirmed as juvenile fibroadenomas.
+After the biopsy, a breast US was done as follow-up every 6 or 12 months for 3 years.
+During the surveillance period, core biopsies were performed six times, followed by US-guided vacuum-assisted core needle excisions or surgical excisions of lesions with increased sizes.
+Pathologic results were fibroadenoma, tubular adenoma or atypical ductal hyperplasia (ADH) involving fibroadenoma.
+At the age of 19 years, the patient underwent a neck US for a palpable mass in the left neck.
+Several indeterminate nodules were revealed in both thyroid glands.
+A follow-up US revealed that some of those nodules increased in size.
+The patient underwent a total thyroidectomy and the pathologic result revealed an invasive follicular carcinoma in the left thyroid and follicular adenoma in the right thyroid (Fig.1A).
+At the age of 21 years, she visited the hospital due to a swelling of her left cheek.
+Head and neck CT scan revealed a vascular mass in the left parotid gland extended into the left forehead.
+An external carotid angiography confirmed the diagnosis of an AVM with feeder vessel arising from the left internal maxillary artery (Fig.1B).
+A partial embolization decreased the blood flow through the AVM, alleviating the patient's symptom.
+She was referred to genetic counseling under the suggestion of a Cowden syndrome and the PTEN DNA sequencing test of her blood sample revealed a frameshift mutation, c.301dupA (p.I101NfsX6).
+At the age of 22 years, the patient presented with a mass with increased size and increased vascularity in the left breast.
+An US-guided vacuum-assisted core needle excision revealed a ductal carcinoma in situ (DCIS) of non-comedo type and a low grade involving fibroadenoma (Fig.1C).
+The contrast-enhanced breast MRI for the preoperative evaluation demonstrated multiple, well-circumscribed, enhancing masses in both breasts (Fig.1D).
+All masses showed high or intermediate signal intensity on T2-weighted images (Fig.1E).
+Several masses showed an early rapid enhancement with washout kinetic pattern on time-signal intensity curve evaluation, which tends to be associated with malignancy (Fig.1F).
+A bilateral prophylactic mastectomy with immediate breast reconstruction was performed in view of multiple bilateral breast masses with suspicious kinetic features on breast MRI and a high risk for breast cancer of Cowden syndrome.
+The surgical histopathology revealed ADH involving tubular adenoma without residual carcinoma in the left breast and multiple tubular adenomas, fibroadenomas and intraductal papillomas in both breasts.
+The patient had no family history of breast cancer or Cowden syndrome.