Peek D F, Beets G L
Department of Surgery, University Hospital Maastricht, The Netherlands.
Dis Colon Rectum. 1999 Jan;42(1):113-5; discussion 115-6. doi: 10.1007/BF02235194.
Pseudomyxoma peritonei is a rare disease characterized by mucinous ascites and associated with ruptured mucocele, cystadenoma, and low-grade carcinoma arising from the appendix, ovaries, or colon. Metastases and extraperitoneal involvement are extremely rare events.
This is a case report of a patient with pseudomyxoma peritonei with pleural involvement.
A 38-year-old male patient with a pseudomyxoma peritonei from appendiceal origin underwent an extensive cytoreduction procedure. During the operation pleural involvement was noted. This was later confirmed by thoracoscopy. An expectant policy was followed until the patient became symptomatic with progressive disease in the abdomen and both pleural cavities. With systemic chemotherapy (5-fluorouracil and leucovorin), a good clinical response was obtained, and the patient was alive with stable disease 2.5 years after the first diagnosis.
Involvement of the pleural cavity by pseudomyxoma peritonei is rare and carries an unfavorable prognosis. Whenever possible, the same guidelines as for intra-abdominal disease should be followed: extensive cytoreductive procedures with local and/or systemic chemotherapy. In our patient we hope to achieve a prolonged palliation with systemic chemotherapy.
腹膜假黏液瘤是一种罕见疾病，其特征为黏液性腹水，与阑尾、卵巢或结肠来源的黏液囊肿破裂、囊腺瘤及低级别癌相关。转移及腹膜外受累极为罕见。
本文是一例腹膜假黏液瘤合并胸膜受累患者的病例报告。
一名38岁阑尾源性腹膜假黏液瘤男性患者接受了广泛的肿瘤细胞减灭术。术中发现胸膜受累，随后经胸腔镜检查得以证实。采取观察等待策略，直至患者出现腹部及双侧胸腔进行性病变的症状。通过全身化疗（5-氟尿嘧啶和亚叶酸钙），获得了良好的临床反应，患者在首次诊断后2.5年存活且病情稳定。
腹膜假黏液瘤累及胸腔罕见且预后不良。只要有可能，应遵循与腹腔疾病相同的治疗原则：进行广泛的肿瘤细胞减灭术并联合局部和/或全身化疗。对于我们的患者，我们希望通过全身化疗实现长期姑息治疗。
