Hoshi Minako, Ikeda Kimimasa, Higashiguchi Kimiya, Kobayashi Teruyuki, Sakai Kenji, Koyama Taichi, Doi Takasi, Taniguchi Hirokazu, Murakami Masakazu, Kurokawa Eiji, Nakamichi Itsuko
Dept. of Surgery, Minoh City Hospital.
Gan To Kagaku Ryoho. 2015 Nov;42(12):1944-6.
The patient was a 22-year-old woman with FAP, who had undergone laparoscopic total colectomy 3 years previously. She presented to our hospital with a high fever and abdominal pain. Large hard tumors were palpated in the right lower abdomen and pelvis. Blood examination showed an inflammatory response. CT scan revealed 17 cm diameter solid tumors. At surgery, 2 tumors arising from the mesentery of the small intestine were found, neither of which invaded any organs. We performed tumor extirpation with partial resection of the duodenum, ileum, right fallopian tube and rectum. A jejunal stoma was formed, leaving a length of remnant intestine of about 120 cm. The histopathological diagnosis was given as desmoid tumor with infection. The patient was discharged from the hospital on the 9th postoperative day. Desmoid tumor is the second most common cause of death in FAP patients. Although desmoids can also occur in the extremities, most FAP patients develop intra-abdominal tumors. Despite being histologically benign, they are locally infiltrative and can cause death through invasion and destruction of adjacent vital structures and organs. Here, we report a case of desmoid tumors with FAP with reference to the literature.
该患者为一名22岁患有家族性腺瘤性息肉病(FAP)的女性,3年前接受了腹腔镜全结肠切除术。她因高热和腹痛前来我院就诊。在右下腹和盆腔可触及巨大坚硬的肿瘤。血液检查显示有炎症反应。CT扫描显示直径为17厘米的实性肿瘤。手术中,发现2个起源于小肠系膜的肿瘤,均未侵犯任何器官。我们进行了肿瘤切除,并部分切除了十二指肠、回肠、右侧输卵管和直肠。形成了空肠造口,剩余肠管长度约为120厘米。组织病理学诊断为韧带样瘤伴感染。患者术后第9天出院。韧带样瘤是FAP患者的第二大常见死因。尽管韧带样瘤也可发生于四肢,但大多数FAP患者会发生腹腔内肿瘤。尽管在组织学上为良性,但它们具有局部浸润性,可通过侵犯和破坏相邻的重要结构和器官而导致死亡。在此,我们结合文献报道一例FAP合并韧带样瘤的病例。