Zhao Xiang-Rong, Gao Chao, Zhang Yong, Yu Yong-Hua
From the School of Medical and Life Sciences, Shandong Academy of Medical Sciences, Jinan University, Jinan, China (XRZ, CG); Department of Radiation Oncology II, Shandong Cancer Hospital and Institute, Jinan, China (XRZ, CG, YZ, YHY).
Medicine (Baltimore). 2015 Dec;94(49):e2253. doi: 10.1097/MD.0000000000002253.
Retrorectal cystic hamartomas are rare congenital presacral lesions and malignancy is extremely rare. Although surgical excision is the essential for treatment, a unique feature of our case compared with previously reported tailgut cysts is that this patient's blood irregular antibodies are positive with higher operational risks.A 44-year-old woman presented to our department complaining of pelvic and perineal pain for 6 months. Computed tomography (CT) scan of the abdomen and pelvis demonstrated a well-demarcated hypodense, multilocular cystic lesion, 10 cm in size, in the presacral region of the right of the midline. We found her blood irregular antibodies were positive in the preoperative examination. So she quitted surgery. Exploratory laparotomy and incision and drainage of pelvic tumor were operated. Postoperative routine pathology showed: (retroperitoneal tumors) moderately differentiated adenocarcinoma. Combined with clinical symptom and imaging, malignant transformation of retrorectal cystic hamartomas (tailgut cysts) was diagnosed. Taking into account that cyst is not sensitive to radiotherapy, so tumor necrosis factor (TNF) and raltitrexed were infused into the cysts and 3 cycles oxaliplatin (130 mg/m) were completed. Now although the lesion is shrink, but yellow, viscous mucus still secrete constantly, 100 ml/w.Given surgical excision is the essential for treatment, complete surgical excision should be implemented as far as possible. But if surgery cannot be carried out like the presented case, systemic chemotherapy and local radiotherapy are also available, which can alleviate the symptoms of oppression and improve the quality of life partly.
直肠后囊性错构瘤是罕见的先天性骶前病变，恶变极为罕见。虽然手术切除是治疗的关键，但与先前报道的尾肠囊肿相比，我们这个病例的一个独特之处在于该患者血液中的不规则抗体呈阳性，手术风险较高。一名44岁女性因骨盆和会阴疼痛6个月前来我院就诊。腹部和骨盆的计算机断层扫描（CT）显示在中线右侧的骶前区域有一个边界清晰的低密度、多房囊性病变，大小为10厘米。我们在术前检查中发现她的血液不规则抗体呈阳性。所以她放弃了手术。进行了剖腹探查和盆腔肿瘤切开引流术。术后常规病理显示：（腹膜后肿瘤）中分化腺癌。结合临床症状和影像学检查，诊断为直肠后囊性错构瘤（尾肠囊肿）恶变。考虑到囊肿对放疗不敏感，因此向囊肿内注入肿瘤坏死因子（TNF）和雷替曲塞，并完成了3个周期的奥沙利铂（130mg/m）治疗。目前虽然病变有所缩小，但仍持续分泌黄色、粘稠的黏液，每周100毫升。鉴于手术切除是治疗的关键，应尽可能实施完整的手术切除。但如果像本病例一样无法进行手术，全身化疗和局部放疗也是可行的，这可以部分缓解压迫症状并提高生活质量。
