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继发于肿瘤的贲门失弛缓症:一种具有不断变化的鉴别诊断的疾病。

Achalasia secondary to neoplasia: a disease with a changing differential diagnosis.

机构信息

Department of Gastroenterology, Mayo Clinic, Rochester, MN, USA.

出版信息

Dis Esophagus. 2012 May;25(4):331-6. doi: 10.1111/j.1442-2050.2011.01266.x. Epub 2011 Oct 3.

DOI:10.1111/j.1442-2050.2011.01266.x
PMID:21967574
Abstract

Achalasia secondary to neoplasia is an uncommon entity, but recognition is paramount given the concern of missing a cancer diagnosis. Most case series of secondary achalasia occurred in prior decades raising the question of whether the underlying neoplastic causes have changed. All cases of achalasia secondary to neoplasia were reviewed at the Mayo Clinic from 2000 to the present. Cases were assessed for underlying cause of achalasia, whether achalasia was the primary presentation and demographic and clinical factors. Seventeen patients with achalasia secondary to neoplasia were identified. This was 1.5% of all patients with achalasia seen. The most common causes were adenocarcinoma of the esophagus, followed by breast and non-small cell lung cancer. No cases of gastric cancer were identified. Most patients had weight loss and rapid onset of symptoms but could not clearly be distinguished from primary achalasia. Nine patients presented with achalasia, whereas eight patients had known neoplasia. Five of these patients had a positive paraneoplastic panel suggestive of a paraneoplastic syndrome. Prognosis was generally poor except for patients with esophageal leiomyomatosis. This case series demonstrates a changing differential diagnosis for achalasia secondary to neoplasia with a higher number of patients presenting with a known primary and with a paraneoplastic syndrome. Awareness of secondary achalasia and its differentiation from primary causes is still essential.

摘要

继发于肿瘤的贲门失弛缓症并不常见,但鉴于存在漏诊癌症的风险,认识到这一点至关重要。大多数继发于贲门失弛缓症的病例系列发生在前几十年,这引发了一个问题,即潜在的肿瘤病因是否已经发生了变化。本研究回顾了梅奥诊所 2000 年至目前所有继发于肿瘤的贲门失弛缓症病例。评估了贲门失弛缓症的潜在病因、贲门失弛缓症是否为首发表现以及人口统计学和临床因素。确定了 17 例继发于肿瘤的贲门失弛缓症患者。这占所有贲门失弛缓症患者的 1.5%。最常见的病因是食管腺癌,其次是乳腺癌和非小细胞肺癌。未发现胃癌病例。大多数患者有体重减轻和症状迅速发作,但与原发性贲门失弛缓症无法明确区分。9 例患者表现为贲门失弛缓症,而 8 例患者有已知肿瘤。其中 5 例患者的副肿瘤标志物阳性,提示存在副肿瘤综合征。除了食管平滑肌瘤病患者外,预后通常较差。本病例系列表明,继发于肿瘤的贲门失弛缓症的鉴别诊断正在发生变化,更多的患者以已知的原发性疾病和副肿瘤综合征为首发表现。认识到继发于贲门失弛缓症及其与原发性病因的区别仍然至关重要。

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