Upper Aerodigestive Tract Liposarcoma: Four Cases of a Rare Entity and a Review of the Literature

IF 2.2 3区 医学 Q1 OTORHINOLARYNGOLOGY Head and Neck-Journal for the Sciences and Specialties of the Head and Neck Pub Date : 2025-02-17 DOI:10.1002/hed.28114
Uma Ramesh, Antonio Bon Nieves, Maria Feucht, Naomi Wang, Rahul Alapati, Chelsea S. Hamill, Shannon Kraft, James Garnett, Devin Shrock, Omar Karadaghy, Ethan Craig, Andrés M. Bur
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Abstract

Background

Liposarcomas involving the upper aerodigestive tract (UADT) are extremely rare. Consequently, consensus regarding the treatment approach and surveillance is not well established.

Methods

We present four cases of UADT liposarcoma. All patients presented with either dysphagia or respiratory difficulty and underwent endoscopic examination as part of their diagnostic workup. Surgical excision, either endoscopic or open resection, was performed for all patients.

Results

All patients had a pathologic diagnosis of well-differentiated liposarcoma without nodal or distant disease involvement. One patient underwent complete resection with no recurrences. Another patient experienced several recurrences, and his tumor ultimately underwent rapid disease progression and pathologic transformation into dedifferentiated liposarcoma, necessitating total laryngectomy. Of the two patients with positive surgical margins at their initial resections, one was lost to follow-up from our institution. The other three patients, including the second patient with positive margins, continue to follow up with endoscopic surveillance at our institution with no evidence of residual disease.

Conclusions

UADT liposarcoma is a locally aggressive tumor that may frequently recur. Stringent surveillance is necessary to monitor disease progression, particularly if positive margins are noted during surgical excision. The role of adjuvant therapy has yet to be established.

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上消化道脂肪肉瘤:四例罕见病例及文献综述
背景:脂肪肉瘤累及上气消化道(UADT)极为罕见。因此,关于治疗方法和监测的共识尚未很好地建立。方法:报告4例UADT脂肪肉瘤。所有患者均表现为吞咽困难或呼吸困难,并接受内窥镜检查作为诊断工作的一部分。所有患者均行内镜或切开手术切除。结果:所有患者病理诊断为高分化脂肪肉瘤,无淋巴结或远处病变累及。1例患者接受了完全切除,无复发。另一位患者多次复发,其肿瘤最终迅速进展并病理转化为去分化脂肪肉瘤,需要全喉切除术。在最初切除时手术切缘呈阳性的两例患者中,有一例失去了我们机构的随访。其他三名患者,包括第二名切缘阳性的患者,继续在我们机构进行内窥镜监测,未发现残留疾病的证据。结论:UADT脂肪肉瘤是一种易复发的局部侵袭性肿瘤。严格的监测是必要的,以监测疾病的进展,特别是如果在手术切除时发现阳性边缘。辅助治疗的作用尚未确定。
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来源期刊
CiteScore
7.00
自引率
6.90%
发文量
278
审稿时长
1.6 months
期刊介绍: Head & Neck is an international multidisciplinary publication of original contributions concerning the diagnosis and management of diseases of the head and neck. This area involves the overlapping interests and expertise of several surgical and medical specialties, including general surgery, neurosurgery, otolaryngology, plastic surgery, oral surgery, dermatology, ophthalmology, pathology, radiotherapy, medical oncology, and the corresponding basic sciences.
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