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Cowden病的主要表现有口腔皮肤错构瘤和胃肠道弥漫性息肉病,息肉多属良性,恶变率低,其它表现有乳腺癌和甲状腺肿瘤.作者报道一例Cowden病患者具有甲状旁腺腺瘤和胃癌的表现.病人为一66岁女性,以往曾患有双侧乳腺浸润性导管癌,分别在19年前和13年前作左侧乳腺癌根治术和右侧改良式乳腺癌根治术.入院前9年.病人发现胃肠道隐血,胃镜和活检提示增生性胃息肉,其中1个显示有原位癌变化,即作胃部分切除.入院时检查发现胫骨前多个5mm大小的皮垂,舌和口腔粘膜有100个以上的2～3mm大小纤维瘤,神经系统正常.实验室检查示血钙和甲状旁腺激素明显增高,颈部探查发现右下甲状旁腺明显增大,约1850mg,手术切除;同时发现甲状腺右叶增大,作右叶切除,组织学检查证实为甲状旁腺腺瘤和甲状腺滤泡型腺瘤.术后每年复查,胃内增生型息肉和结肠内多达100个以上的非腺瘤性息肉尚无癌变证据.
The main manifestations of Cowden’s disease are oral skin hamartoma and diffuse polyposis of the gastrointestinal tract. Polyps are mostly benign with low rates of malignant transformation. Other manifestations are breast and thyroid tumors. The authors report a case of Cowden’s disease with parathyroid adenomas. And the performance of gastric cancer. The patient is a 66-year-old woman, previously had bilateral breast invasive ductal carcinoma, respectively, left and right modified breast cancer radical surgery 19 years ago and 13 years ago. During the first 9 years, the patient had found occult blood in the gastrointestinal tract, gastroscopy and biopsy suggesting hyperplastic gastric polyps, of which one showed changes in the carcinoma in situ, ie partial partial excision of the stomach. On examination at admission, multiple 5 mm skin flaps were found in front of the sacrum. There are more than 100 2~3mm fibroids in the tongue and oral mucosa. The nervous system is normal. Laboratory examination shows that serum calcium and parathyroid hormone are significantly increased. Neck exploration revealed a significant increase in the right lower parathyroid gland, approximately 1850 mg. Surgical resection; also found that the right thyroid enlargement, right lobectomy, histological examination confirmed as parathyroid adenoma and thyroid follicular adenoma. Postoperative review every year, up to 100 intracellular hyperplasia polyps and colon More than non-adenomatous Meat is no evidence of cancer.