Zhou Feng, Lv Bingjian, Dong Lifeng, Wan Fang, Qin Jiale, Huang Lili
Department of Pathology, Women's Hospital, School of Medicine, Zhejiang University Hangzhou, Zhejiang Province, China.
Department of Surgery, Women's Hospital, School of Medicine, Zhejiang University Hangzhou, Zhejiang Province, China.
Int J Clin Exp Pathol. 2014 Jun 15;7(7):4448-53. eCollection 2014.
We report a very rare case of Peutz-Jeghers syndrome (PJS) composed of multiple genital tract tumors and mucinous adenocarcinoma. A 46-year-old woman presented to our hospital with lower abdominal pain resulting from PJS involves sex cord tumor with annular tubules (SCTAT), ovarian mucinous tumor, ovarian serous tumor, mucinous adenocarcinoma of colon. The CEA concentration is high before surgery, and decreases after the surgery and subsequent chemoradiotherapy. This case demonstrates a classic clinical presentation of a patient with PJS. PJS patients have increased risk of malignancy and early detection and regular surveillance of the high-risk patients with PJS is crucial. Surgery may be required for obstructive gastrointestinal lesions as well as those exhibiting malignant degeneration.
我们报告了一例极为罕见的伴有多种生殖道肿瘤和黏液腺癌的黑斑息肉综合征(PJS)病例。一名46岁女性因PJS累及环状小管性索肿瘤(SCTAT)、卵巢黏液性肿瘤、卵巢浆液性肿瘤、结肠黏液腺癌而出现下腹痛前来我院就诊。术前癌胚抗原(CEA)浓度较高,术后及后续放化疗后降低。该病例展示了PJS患者的典型临床表现。PJS患者发生恶性肿瘤的风险增加,对PJS高危患者进行早期检测和定期监测至关重要。对于梗阻性胃肠道病变以及发生恶变的病变可能需要进行手术。