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侵犯前列腺的直肠肛管交界平滑肌瘤恶性退变至平滑肌肉瘤

Malignant Degeneration to Leiomyosarcoma of the Anorectal Junction Invading the Prostate.

作者信息

Perrotti Gabrielle, Van Houtte Olivier, Ayers Amanda, Lambert Erica, Lewis Robert

机构信息

General Surgery, Jefferson Abington Hospital, Abington, USA.

Colorectal Surgery, St. Francis Hospital and Medical Center, Hartford, USA.

出版信息

Cureus. 2021 Nov 1;13(11):e19196. doi: 10.7759/cureus.19196. eCollection 2021 Nov.

Abstract

While colonic leiomyomas are common, leiomyosarcomas of the GI tract are rare. Increased mitotic rate, as well as lymph node involvement, portend a worse prognosis in leiomyosarcomas. They can arise anywhere along the GI tract, but anal canal occurrence is extremely rare. We present the case of a 75-year-old male diagnosed eight years prior with leiomyoma of the anorectal junction. There was a recurrence after endoscopic resection. He was referred to colorectal surgery due to symptoms of bleeding, skin irritation, anal pruritus, and rectal pain. On exam, he had a palpable mass at the dentate line. Workup revealed a 3.5 cm mass at the anorectal junction with pathology showing a leiomyosarcoma with moderate atypia and a high mitotic rate. MRI revealed invasion into the prostate. Robotic pelvic exenteration, including cystoprostatectomy, abdominoperineal resection, and ileal conduit, was performed. Final pathology results showed a grade 2 leiomyosarcoma invading the prostate and skeletal muscle and incidentally found Gleason 3+4 prostate cancer, pT2.  A very small percentage of anorectal leiomyosarcoma cases were located in the anal canal. Surgery remains the best curative option, as chemotherapy and radiation data are limited. This rare tumor, which previously has not been documented to have degenerated from a benign rectal leiomyoma, was diagnosed as a result of close monitoring after previous local resections and cured by local resection and radiation.

摘要

虽然结肠平滑肌瘤很常见,但胃肠道平滑肌肉瘤却很罕见。有丝分裂率增加以及淋巴结受累预示着平滑肌肉瘤的预后更差。它们可发生于胃肠道的任何部位,但肛管发生极为罕见。我们报告一例75岁男性,8年前被诊断为直肠肛管交界平滑肌瘤。内镜切除后复发。因出血、皮肤刺激、肛门瘙痒和直肠疼痛症状,他被转诊至结直肠外科。检查时,在齿状线处可触及肿块。检查发现直肠肛管交界处有一个3.5 cm的肿块,病理显示为中度异型性和高有丝分裂率的平滑肌肉瘤。MRI显示肿瘤侵犯前列腺。实施了机器人盆腔脏器清除术,包括膀胱前列腺切除术、腹会阴联合切除术和回肠造口术。最终病理结果显示为2级平滑肌肉瘤,侵犯前列腺和骨骼肌,偶然发现Gleason 3+4前列腺癌,pT2。肛管直肠平滑肌肉瘤病例中只有很小一部分位于肛管。手术仍然是最佳的治愈选择,因为化疗和放疗的数据有限。这种罕见的肿瘤之前没有文献记载是由良性直肠平滑肌瘤退变而来,是通过对先前局部切除术后的密切监测而诊断出来的,并通过局部切除和放疗治愈。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/5c9a/8642126/423ba6d8e399/cureus-0013-00000019196-i01.jpg

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