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食管原发性黑色素瘤的各种临床情况:来自两个大学胸外科中心的回顾性 20 年分析。

Various clinical scenarios of primary melanoma of the esophagus: A retrospective 20-year analysis from two university thoracic surgery centers.

机构信息

Department of Thoracic Surgery, Jagiellonian University Medical College, Faculty of Health Sciences, John Paul II Hospital, Kraków, Poland.

Department of Thoracic Surgery and Transplantation, Pomeranian Medical University in Szczecin, Poland.

出版信息

Adv Clin Exp Med. 2022 Mar;31(3):337-344. doi: 10.17219/acem/146797.

Abstract

BACKGROUND

Primary melanoma of the esophagus (PME) represents a rare type of gastrointestinal malignancy with an exceptionally poor diagnosis. So far, only few descriptions of PME which satisfactorily summarize their clinical characteristics and prognosis have been published.

OBJECTIVES

The aim of our study was to summarize our experience with PME patients.

MATERIAL AND METHODS

In a group of 1387 patients who underwent esophagectomy due to neoplastic process in the years 2000-2020 in 2 high-volume university thoracic surgery centers, we identified those with confirmed PME diagnosis. Subsequently, their clinical characteristics, imaging and histopathological results were compared. The data regarding the long-term survival were obtained from the Polish National Death Registry.

RESULTS

The PME was identified in 4 (0.29%) patients. Three of them (75%) were males. The mean age on admission was 64.3 ±17.5 years. The main symptom in all patients was dysphagia. In 1 patient with the most advanced PME, the clinically relevant weight loss was noted. In 3 patients, Ivor Lewis esophagectomy was performed, and 1 patient underwent McKeown resection. Histopathologic examination revealed a metastasis of lymph nodes only in 1 patient. The average maximum size of tumor was 6.9 ±4.7 cm and all tumors were located in distal part of the esophagus. Two out of those 4 patients are still alive and the longest survival time is 17 years. One patient died due to postoperative massive gastrointestinal bleeding complicated with cardiac arrest and the other one due to progression of PME systemic dissemination 6 months after surgical treatment.

CONCLUSION

The PME is an extremely rare diagnosis. A long-term survival can be achieved with the complete resection. Clinical scenarios of surgically treated PME patients may significantly differ.

摘要

背景

食管原发性黑色素瘤(PME)是一种罕见的胃肠道恶性肿瘤,诊断极为困难。迄今为止,仅有少数关于 PME 的描述能够充分总结其临床特征和预后。

目的

本研究旨在总结我们在 PME 患者中的经验。

材料和方法

在 2000 年至 2020 年期间,在 2 个高容量大学胸外科中心,对 1387 例因肿瘤接受食管切除术的患者进行了研究,我们确定了那些确诊为 PME 的患者。随后,比较了他们的临床特征、影像学和组织病理学结果。长期生存数据来自波兰国家死亡登记处。

结果

在 4 例患者(0.29%)中发现了 PME。其中 3 例(75%)为男性。入院时的平均年龄为 64.3±17.5 岁。所有患者的主要症状均为吞咽困难。在 1 例 PME 最晚期的患者中,出现了明显的体重减轻。3 例患者行 Ivor Lewis 食管切除术,1 例行 McKeown 切除术。组织病理学检查仅在 1 例患者中发现淋巴结转移。肿瘤的平均最大直径为 6.9±4.7cm,所有肿瘤均位于食管远端。这 4 例患者中有 2 例仍存活,最长生存时间为 17 年。1 例患者因术后大量胃肠道出血合并心脏骤停死亡,另 1 例患者因手术治疗 6 个月后 PME 全身播散进展而死亡。

结论

PME 是一种极其罕见的诊断。完全切除可实现长期生存。接受手术治疗的 PME 患者的临床情况可能有很大差异。

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