General Surgery and Organ Transplantation Unit, Department of Surgery, Sapienza University of Rome, Italy.
Thoracic Surgery Unit, Department of Surgery, Sapienza University of Rome, Rome, Italy.
Pathol Res Pract. 2019 Oct;215(10):152602. doi: 10.1016/j.prp.2019.152602. Epub 2019 Aug 17.
Carcinoma cuniculatum (CC) is a rare variant of an extremely well-differentiated squamous cell carcinoma. The most commonly involved site is the skin, with a preference for the sole. Only 15 cases of esophageal CC have been reported so far. Based on published data, the clinical behavior of CC has not been clearly defined. We describe the clinical-pathologic features of two cases of esophageal CC, and provide a review of the available literature, to shed more light on this unusual tumor.
A detailed gross and histologic analysis was performed on two cases of surgically treated esophageal CC. The patients were followed-up after surgery. A systematic search was also done concerning studies focused on esophageal CC. A search of the electronic databases MEDLINE-PubMed was conducted using the following research terms: (esophagus) AND (cuniculatum carcinoma).
Both patients were alive at last follow-up at six and nine months from surgery without any recurrence. Concerning the fifteen cases reported from the systematic review, median follow-up after surgery was very long as compared to common esophageal cancers (4.0 years), with only one recurrence observed.
CC shows an indolent clinical behavior, with a low recurrence rate after radical surgery. The diagnosis of this rare tumor is typically made after surgery. An aggressive approach is required with curative intents.
腺样囊性癌（CC）是一种非常分化良好的鳞状细胞癌的罕见变体。最常涉及的部位是皮肤，特别是脚底。迄今为止，仅有 15 例食管 CC 报道。根据已发表的数据，CC 的临床行为尚未明确界定。我们描述了两例食管 CC 的临床病理特征，并对现有文献进行了回顾，以期更深入地了解这种不常见的肿瘤。
对两例经手术治疗的食管 CC 进行了详细的大体和组织学分析。对患者进行了手术随访。还针对食管 CC 进行了系统的文献研究。使用以下研究术语在电子数据库 MEDLINE-PubMed 中进行了搜索：（食管）和（腺样囊性癌）。
两名患者在手术后的六个月和九个月的最后随访时均存活，没有任何复发。关于系统综述中报道的十五例病例，与常见的食管癌相比，手术后的中位随访时间非常长（4.0 年），仅观察到一次复发。
CC 表现出惰性的临床行为，根治性手术后复发率低。这种罕见肿瘤的诊断通常是在手术后做出的。需要采用积极的治疗方法，以达到治愈的目的。
