de Souza Ana Beatriz Pereira, Lima Amanda Pereira, Genaro Lívia Moreira, Geiger Carla Peres Fingerhut, Ayrizono Maria de Lourdes Setsuko, Leal Raquel Franco
Colorectal Surgery Unit, School of Medical Sciences, University of Campinas, Campinas, São Paulo, Brazil.
Department of Radiology, School of Medical Sciences, University of Campinas, Campinas, São Paulo, Brazil.
Int J Surg Case Rep. 2022 Jun;95:107211. doi: 10.1016/j.ijscr.2022.107211. Epub 2022 May 18.
Crohn's disease (CD) is a chronic bowel disease that, due to exacerbated inflammation, can lead to complications such as the development of perianal fistulas. The development of mucinous adenocarcinoma in perianal fistulas in patients with CD is rare and, consequently, few reports exist in the literature.
We report the case of a 71-year-old man diagnosed 22 years ago with CD with perineal involvement, who came with complaints of intense perianal pain, a gluteal mass, and local bleeding. Tomography of his abdomen showed an expansive, heterogeneous, and solid perianal mass on the right, with interspersed necrotic/liquefied areas and possible mucinous content. The patient was referred to the surgery department for an incisional biopsy, which confirmed mucinous adenocarcinoma. The patient underwent extra levator abdominoperineal rectal resection (APR) with partial prostatectomy.
Perineal mucinous adenocarcinoma arising in a fistula associated with CD is very rare. Since the symptoms overlap, early diagnosis of malignancy is difficult. Histological analysis is the gold standard for its diagnosis. Surgical resection through APR is well-established and, despite being a complex procedure with potential complications, tends to have good results. However, the locoregional and inguinal lymph node involvement was related to a worse progression in this case.
The diagnostic hypothesis of mucinous adenocarcinoma should be suspected in CD patients who present long-term perineal involvement with fistulas. Biopsies and imaging exams should be performed to aid the diagnosis of the condition and thus contribute to the surgical plan.
克罗恩病（CD）是一种慢性肠道疾病，由于炎症加剧，可导致诸如肛周瘘管形成等并发症。CD患者肛周瘘管发生黏液腺癌的情况罕见，因此文献报道较少。
我们报告一例71岁男性患者，22年前被诊断为伴有会阴受累的CD，因肛周剧痛、臀部长肿块及局部出血前来就诊。其腹部断层扫描显示右侧有一个膨胀性、异质性实性肛周肿块，伴有散在的坏死/液化区域以及可能的黏液成分。该患者被转诊至外科进行切开活检，结果证实为黏液腺癌。患者接受了经腹会阴联合直肠切除术（APR）加部分前列腺切除术。
CD相关瘘管中发生的会阴黏液腺癌非常罕见。由于症状重叠，恶性肿瘤的早期诊断困难。组织学分析是其诊断的金标准。通过APR进行手术切除是公认的方法，尽管这是一个复杂的手术且有潜在并发症，但往往效果良好。然而，在该病例中，局部和腹股沟淋巴结受累与病情进展较差有关。
对于长期存在会阴瘘管受累的CD患者，应怀疑黏液腺癌的诊断假设。应进行活检和影像学检查以辅助诊断病情，从而有助于制定手术方案。
