Department of Surgery, Gastroenterological Surgery, Graduate School of Medicine, Osaka University, Osaka, Japan.
Oncol Rep. 2013 May;29(5):1751-5. doi: 10.3892/or.2013.2345. Epub 2013 Mar 13.
Intra-abdominal desmoid tumor is a life-threatening disease. Studies have shown that dacarbazine (DTIC)-doxorubicin (DOX) (D-D) therapy is the most effective treatment. However, myelosuppression is a major problem, and cardiac muscle disorders due to DOX limit the number of administration cycles, whereas it usually requires a long time to achieve tumor shrinkage. To resolve these issues, we introduced low-dose D-D therapy to 3 patients employing 50 mg/m² DOX and 600-700 mg/m² DTIC per cycle, which permits repeated administration cycles up to 10-11 times. Case 1 was a 23-year-old female with a sporadic recurrent mesenterium desmoid tumor located in the pelvis (maximum diameter, 8 cm). Cases 2 and 3 were a 33-year-old female and a 36-year-old male. Both patients had intra-abdominal mesenterium desmoid tumors (maximum diameter 9.6 and 9.0 cm, respectively) that were generated after proctocolectomy due to familial adenomatous polyposis. No severe adverse events occurred during the therapy. With the aid of sulindac and tamoxifen after low-dose D-D therapy, the first two patients achieved a complete response, and the third patient achieved a partial response and awaits further tumor shrinkage. Our experience indicates that low-dose DT-D therapy is a safe and effective regimen for patients with intra-abdominal desmoid tumors.
腹腔内韧带样瘤是一种危及生命的疾病。研究表明，氮烯咪胺（DTIC）-多柔比星（DOX）（D-D）治疗是最有效的治疗方法。然而，骨髓抑制是一个主要问题，由于 DOX 引起的心肌障碍限制了给药周期的数量，而通常需要很长时间才能实现肿瘤缩小。为了解决这些问题，我们对 3 名患者采用低剂量 D-D 治疗，每个周期给予 50mg/m² DOX 和 600-700mg/m² DTIC，允许重复给药周期多达 10-11 次。病例 1 是一名 23 岁的女性，患有散发性复发性肠系膜韧带样瘤，位于骨盆（最大直径 8cm）。病例 2 和 3 是一名 33 岁的女性和一名 36 岁的男性。两名患者均因家族性腺瘤性息肉病而行直肠结肠切除术，术后发生肠系膜间位韧带样瘤（最大直径分别为 9.6cm 和 9.0cm）。在治疗过程中没有发生严重的不良事件。在低剂量 D-D 治疗后使用舒林酸和他莫昔芬，前两名患者达到完全缓解，第三名患者达到部分缓解，等待进一步肿瘤缩小。我们的经验表明，低剂量 DT-D 治疗方案对腹腔内韧带样瘤患者是安全有效的。
