Liposarcoma of Hypopharynx and Esophagus: a Unique Entity?
Liposarcoma of Hypopharynx and Esophagus: a Unique Entity?
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DOI：
10.1007/s12029-016-9808-6
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发表时间：
2016-06-01
影响因子：
1.6
通讯作者：
Pecorari, Giancarlo
中科院分区：
文献类型：
--
作者：
Riva, Giuseppe;Sensini, Matteo;Pecorari, Giancarlo
目的：脂肪肉瘤是成人最常见的软组织肉瘤。它约占所有间充质恶性肿瘤的20%。最常累及腹膜后腔、躯干和四肢。下咽及食管脂肪肉瘤的发生是非常罕见的。方法：我们进行了系统的文献回顾，并报告26例下咽及33例食管脂肪肉瘤。我们分析了自然病史、影像学特征、组织学、治疗和预后，特别关注下咽部和食管肿瘤之间的相似之处和差异。结果：下咽部和食管脂肪肉瘤的相似之处多于差异。发病高峰在第6和第7个十年。诊断程序是吞钡、内窥镜检查和CT/MR成像。分化良好的脂肪肉瘤是最常见的组织学亚型。手术切除是主要治疗手段。内镜下切除可用于下咽部和颈段食管有蒂肿瘤。下咽和食管脂肪肉瘤之间的差异表现为局部复发率（下咽肿瘤更高），巨大肿瘤的数量和复发时间（食管肿瘤更高）。最后，远端食管脂肪肉瘤需要更多的扩展approaches.CONCLUSIONS：下咽部和颈段食管脂肪肉瘤可以被认为是一个独特的病理实体，具有相似的功能和治疗方案。存活率取决于组织学类型和位置。局部复发是常见的，尤其是下咽脂肪肉瘤，而淋巴结或远处转移的风险很低。患者应定期进行检查，以排除局部复发，也是长期的，特别是食管肿瘤。
PURPOSE: Liposarcoma is the most common soft tissue sarcoma in adults. It represents approximately 20% of all mesenchymal malignancies. It most frequently involves retroperitoneum, trunk, and extremities. Hypopharyngeal and esophageal localization of liposarcoma is extremely rare.METHODS: We performed a systematic review of literature and reported 26 and 33 cases of hypopharyngeal and esophageal liposarcoma. We analyzed natural history, imaging features, histology, treatment, and prognosis, with a specific focus to similarities and differences between tumors of hypopharynx and esophagus.RESULTS: Hypopharyngeal and esophageal liposarcomas have more similarities than differences. Incidence has a peak at 6th and 7th decades. The diagnostic procedures are barium swallow, endoscopic examination, and CT/MR imaging. Well-differentiated liposarcoma represents the most frequent histological subtype. Surgical excision is the main treatment. Endoscopic resection can be useful for pedunculated tumors of hypopharynx and cervical esophagus. Differences between hypopharyngeal and esophageal liposarcoma are represented by local recurrence rate (greater for hypopharyngeal tumors), number of giant tumors, and time to recurrence (greater for esophageal tumors). Finally, liposarcomas of distal esophagus need more extended approaches.CONCLUSIONS: Liposarcomas of hypopharynx and cervical esophagus could be considered a unique pathological entity, with similar features and treatment options. Survival rate is dependent on histological type and location. Local recurrence is common, especially for hypopharyngeal liposarcoma, while the risk of lymph node or distant metastasis is very low. Patients should undergo regular examinations to rule out local recurrence, also for a long time, especially for esophageal tumors.