Esophageal Liposarcoma Research Articles

Esophageal dedifferentiated liposarcoma resected by the cervical approach: a case report

BackgroundWhile liposarcomas tend to mainly occur in the soft tissues of the extremities and retroperitoneum, esophageal liposarcoma is rare. Herein, we report a case of a patient who underwent complete resection of an esophageal dedifferentiated liposarcoma via the cervical approach, leading to the preservation of the esophagus.Case presentationA 69-year-old man underwent an upper gastrointestinal endoscopy, as a result of which a submucosal-like tumor was observed. Upper gastrointestinal imaging showed a 12-cm tumor with a stalk arising from the esophageal entrance, extending to the middle intrathoracic esophagus, with a normal surface mucosa. Endoscopic ultrasound-fine needle aspiration biopsy showed that the nuclei of tumors cells were positive for murine double minute (MDM) and weakly positive for cyclin-dependent kinase 4 (CDK4). We diagnosed the tumor as the esophageal dedifferentiated liposarcoma, and planed tumor resection via the cervical approach. The tumor was successfully resected and the postoperative course was uneventful.ConclusionThis case report highlights the use of tumor resection via the cervical approach as a good option for esophageal liposarcoma.

Transoral resection and reconstruction of hypopharyngeal liposarcoma

Hypopharyngeal and esophageal liposarcoma are rare malignant tumors. Low grade liposarcoma have similarities to lipoma.

Giant liposarcoma of esophagus: a rare case report

BackgroundLiposarcoma is a malignant mesenchymal tumor that most commonly involves the retroperitoneum and lower extremities. However, liposarcoma of esophagus has been rarely reported in the literature.Case presentationWe report a case of a 46-year-old man with complaint of intermittent dysphagia for 6 years, accompanied with paroxysmal vomiting of pedicled tumor to the mouth. Imaging studies showed a huge mixed density lesion in the middle esophageal lumen. Surgical resection of the tumor was performed through an external cervical approach. Microscopically, the tumor was composed of mature adipocytes in normal adipose tissue prominently intersected by sparsely cellular fibrous septa containing atypical, enlarged spindle cells with hyperchromatic nuclei. Immunohistochemically, the tumor cells were positive for Vimentin, S-100, CD34 and MDM2. Besides, fluorescence in situ hybridization (FISH) analysis indicated the presence of amplification involving MDM2 gene. The patient was diagnosed as having esophageal well-differentiated liposarcoma and recovered well after the operation.ConclusionsEsophageal liposarcoma is an extremely rare tumor. Due to the nonspecific clinical manifestation and lack of experience, it is challenging to make a clear diagnosis before operation. Definite diagnosis of esophageal liposarcoma depends on histopathology, immunohistochemistry and molecular analysis.

A rare case of giant oesophageal liposarcoma treated with oesophagectomy

Background: Oesophageal sarcomas are extremely rare, constituting less than 5% of oesophageal tumours, with only 0.5% (10%) of these being liposarcomas. Aim: Presentation of a rare case of an oesophageal liposarcoma and review of the literature. Case Presentation: A 69-year-old man with a three-year history of voice changes and progressive dysphagia was referred to our department for consultation and treatment of a giant dedifferentiated oesophageal liposarcoma. The patient underwent hybrid McKeown oesophagectomy. Conclusions: Multimodal management of such patients in specialised centers may spare these patients the discomfort while offering the best chances for cure.

478. ENDOSCOPIC RESECTION OF A HUGE ESOPHAGEAL LIPOSARCOMA WITH LIGASURE INSTRUMENT

Abstract Esophageal liposarcoma is a very rare pedunculated tumor of esophagus. It may cause progressive dysphagia due to mass effect and sudden death due to tumor suffocation. Surgical resection is challenging due to peduncle usually located at the superior esophageal sphincter region. We introduce a novel approach method that can transect the tumor peduncle safe and easy. Review of the chart of Chimei Medical Center. One 70 year old man with progressive dysphagia for 5 months and body weight loss over 8 KGs. A huge tip necrotic pedunculated tumor was found with endoscopy. Cut off the tumor peduncle with ligasure instrument under endoscopic guide was performed and the tumor was removed successfully. No tumor recurrence was found in 2 years. Esophageal liposarcoma is a rare low grade malignancy of esophagus. Ligasure cut off the tumor peduncle can make a clear cut margin and avoid perforation or stenosis of esophagus.

Giant esophageal liposarcoma with squamous cell carcinoma resected via the cervical approach: a case report

BackgroundLiposarcoma is one of the most common soft tissue sarcomas, but is extremely rarely found in the esophagus. There have been no reports of esophageal liposarcoma together with superficial carcinoma of the esophagus. Here, we report a patient who underwent complete resection of esophageal liposarcoma with carcinoma via a cervical approach.Case presentationA 66-year-old man was diagnosed with an esophageal tumor 11 years ago, but he left it untreated. He presented to our hospital with progressive dysphagia and appetite loss since the previous year. Esophagogastroduodenoscopy (EGD) showed a large pedunculated submucosal tumor (SMT) originating at the esophageal entrance, extending to the gastroesophageal junction. Additionally, there was a superficial carcinoma on the surface of the SMT, 30 cm from the incisor teeth. Three-dimensional computed tomography (3D-CT) showed a giant elongated intraluminal tumor extending downwards from the cervical esophagus. We diagnosed a giant esophageal polyp accompanied by a superficial carcinoma and performed tumor resection via a cervical approach. The excised specimen consisted of a 23.0 × 8.5 cm polypoid mass. The final diagnosis by histopathological and immunohistochemical examination was well-differentiated liposarcoma and esophageal squamous cell carcinoma. He was discharged on postoperative day 14 with drastic improvement in his swallowing ability.ConclusionWe reported an extremely rare case of esophageal liposarcoma together with esophageal squamous cell carcinoma that was successfully resected through a small cervical incision.

Successful resection of a giant pedunculated pharyngeal liposarcoma by endoscopic submucosal dissection with countertraction using grasping forceps.

Hypopharyngeal and esophageal liposarcomas are very rare and are mainly diagnosed as pedunculated submucosal tumors.1 Mobile pedunculated tumors of the hypopharynx have the risk of causing airway obstruction and should hence be extracted.2,3 Here, we report a rare case of a patient in whom endoscopic submucosal dissection (ESD) with countertraction using grasping forceps was performed for a giant pedunculated pharyngeal liposarcoma.

P157 GIANT PEDUNCULATED OESOPHAGEAL LIPOSARCOMAS: A CASE AND REVIEW OF RESECTION TECHNIQUES

Abstract Aim Liposarcomas are rare causes of oesophageal tumours, accounting for <1% of tumours. We present a case of giant oesophageal liposarcoma with a discussion focusing on resection techniques. Background & Methods A 54-year-old gentleman presented to the hospital with symptoms of anaemia and weight loss for 3 months. A CT scan and oesophagogastroduodenoscopy (OGD) showed a giant, pedunculated polyp extending from the cervical oesophagus to cardioesophageal junction (CEJ) with mucosal ulceration at its distal aspect. Endoscopic ultrasound (EUS) revealed a 24 x 6 cm submucosal pedunculated mass with lipomatous regions and core biopsy only showed rare groups of spindle cells with no malignancy. Resection was advised in view of occult bleeding from polyp and endoscopic resection was deemed unsuitable in view of a highly vascular stalk and large size of polyp. He underwent surgical resection via a left cervical oesophagostomy with gastrostomy for polyp retrieval. Final histology showed a dedifferentiated liposarcoma arising within a giant fibrovascular polyp. Results Open surgery has classically been standard of treatment, but endoscopic resection is less morbid and invasive. Endoscopic techniques described include using a retraction suture followed by division of the polyp stalk using ultrasonic shears, using a snare, endoscopic submucosal dissection (ESD), and application of hemoclips following diathermy. In cases where endoscopic resection is not feasible, surgery such as oesophagostomy, oesophagectomy and laparotomy for resection and retrieval of the tumour have been described. Main reason for oesophagectomy was the presence of a submucosal (rather than polypoid, pedunculated) large tumour. For resection via oesophagostomy, stalk transection can be performed via stapling devices or suture ligation. Conclusion Giant oesophageal liposarcomas are very rare tumours. Such tumours are usually polypoid, arising from a pedicle in the proximal oesophagus. Resection techniques have shifted from oesophagectomy to less invasive means such as endoscopic resection or oesophagostomy. Decision on type of resection technique depends on tumour characteristics and location; with the guiding principle being resection with clear margins in order to prevent local recurrence.

Esophageal Liposarcoma with Cervical and Abdominal Approach: A Case Report

Esophageal Liposarcoma with Cervical and Abdominal Approach: A Case Report

Polypoid fibroadipose tumors of the esophagus: ‘giant fibrovascular polyp’ or liposarcoma? A clinicopathological and molecular cytogenetic study of 13 cases

Giant fibrovascular polyp of the esophagus is a descriptive diagnostic term intended to encompass rare, large, polypoid esophageal masses composed of fibroadipose tissue. Despite sometimes dramatic clinical presentations, they have historically been considered to represent reactive, non-neoplastic proliferations. Recently, however, a small number of reports have described well-differentiated liposarcomas of the esophagus, mimicking giant fibrovascular polyps. In order to clarify the relationship between esophageal liposarcoma and giant fibrovascular polyp, we retrieved esophageal cases coded as 'giant fibrovascular polyp,' 'lipoma' and ‘liposarcoma’ from our archives and re-examined their clinicopathologic features and MDM2 amplification status. Thirteen cases were identified (lipoma (n=1), giant fibrovascular polyp (n=5), well-differentiated liposarcoma (n=3), dedifferentiated liposarcoma (n=3)). The tumors ranged from 5.2 to 19.5 cm and arose predominantly in the cervical esophagus. All consisted chiefly of mature adipose tissue, with a variable component of fibrous septa. In all cases, close inspection of these fibrous septa showed them to contain an increased number of slightly enlarged spindled cells with irregular, hyperchromatic nuclei, similar to those seen in some well-differentiated liposarcomas. Three cases, all previously classified as dedifferentiated liposarcoma, showed in addition solid zones of non-lipogenic spindle cell sarcoma. By fluorescence in situ hybridization (FISH), all cases showed MDM2 amplification, confirming diagnoses as well-differentiated (N=10) and dedifferentiated (N=3) liposarcoma. Clinical follow-up (8 cases, range 22–156 months, median 33 months) showed 3 patients with local recurrences (1 well-differentiated and 2 dedifferentiated liposarcomas), 1 patient with liver metastases (dedifferentiated liposarcoma) and 2 deaths from disease (both dedifferentiated liposarcomas). These results suggest that the great majority of large, polypoid, fat-containing masses of the esophagus represent well and dedifferentiated liposarcoma, rather than 'giant fibrovascular polyps.' We suggest that the diagnosis of 'giant fibrovascular polyp' should be made with great caution in the esophagus, and only after careful morphological study and MDM2 FISH has excluded the possibility of liposarcoma.

A case of giant primary liposarcoma of esophagus

A case of giant primary liposarcoma of esophagus

Pseudoachalasia due to an Unusual Esophageal Tumor

There have been cases of patients who are diagnosed with achalasia when they actually have pseudoachalasia. Many times the pseudoachalasia is due to an esophageal squamous cell carcinoma or adenocarcinoma. This is a case of pseudoachalasia with an unusual tumor finding. Case Report: A 51 year old male with a history of achalasia presented with fatigue, melena, intermittent fevers, and weight loss for the past 3 weeks. Labs were notable for a Hgb of 5.7, WBCs 11.1, and albumin 1.8. He had an esophageal manometry a year prior at another hospital, read as a gastroesophageal outflow obstruction likely representing achalasia. There, he also underwent an EGD showing mild esophagitis. Barium esophagram on admission to Tampa General showed likely stricture of the proximal and distal esophagus (less likely mass) with dilation of rest of the esophagus. EGD revealed a proximal pouch in the upper esophagus. The esophageal mucosa appeared normal, but was not distending well with air. A 5-6 cm hard, necrotic mass was found in the cardia, which appeared to be on a stalk originating from the gastroesophageal junction. Biopsy of the mass showed fragments of ischemic fibrinous necrotic debris with scattered inflammation, but no malignant cells. CT chest with contrast showed a large intraluminal esophageal mass, measuring 20.2 cm, extending from the level of the clavicles to the gastroesophageal junction. EUS revealed a longitudinal mass from 20-40 cm which originated from the muscularis mucosa (layer 2). FNA showed scattered squamous cells. The decision was made to proceed with surgery. The tumor was completely excised from the submucosal layer without an esophagectomy. The final pathology revealed well-differentiated liposarcoma with myxoid change/atypical lipomatous tumor which formed a 16.9 cm mass- stage pT2b pNX. The patient did well post operatively, without chemo or radiation, and returned to a regular diet with no dysphagia. This case highlights the importance of continuing work up after a diagnosis of possible achalasia when there are still uncertainties. It is unclear if the tumor was missed on the original EGD or obvious signs were not present yet. A review of the literature found no cases of pseudoachalasia due to esophageal liposarcoma. Large esophageal liposarcomas are exceedingly rare, often resulting in esophagectomies. It is unique that this tumor could be fully resected from the submucosal layer, avoiding an esophagectomy.Figure: Esophageal liposarcoma after surgical resection.Figure: CT chest with arrow pointing to esophageal liposarcoma.Figure: Liposarcoma extending from the gastroesophageal junction on EGD.

Successful resection of giant esophageal liposarcoma by endoscopic submucosal dissection combined with surgical retrieval: a case report and literature review.

Liposarcoma of the esophagus is very rare. We experienced a huge (27.5 × 11.6 cm) liposarcoma of the esophagus. A 73-year-old man presented with severe dyspnea requiring emergency tracheal intubation. Computed tomography and esophagogastroduodenoscopy showed a large submucosal tumor arising from the esophageal entrance and extending intraluminally to the lower esophagus. We successfully performed endoscopic submucosal dissection (ESD) and esophagotomy to remove the tumor, which preserved swallowing and phonation. The final diagnosis by histopathologic and immunohistologic examination was well-differentiated liposarcoma of the esophagus. Treatment by the combination of ESD and esophagotomy can be performed even for a very large tumor. This method preserves deglutition with a lower risk of recurrent laryngeal nerve paralysis than that with esophagectomy.

Liposarcoma of Hypopharynx and Esophagus: a Unique Entity?

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. 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. 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. 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.

壁外性に発育した食道脂肪肉腫の1例

壁外性に発育した食道脂肪肉腫の1例

Huge Liposarcoma of Esophagus Resected by Endoscopic Submucosal Dissection: Case Report with Video

Liposarcoma is one of the most common soft tissue sarcomas occurring in adults, but it rarely occurs in the gastrointestinal tract and more uncommonly in the esophagus. To the best of our knowledge, there are only 19 reported cases of esophageal liposarcoma in the literature published in English language up to the year 2008, and they were all treated by surgical methods. Here, we report a case of primary liposarcoma of the esophagus which was treated with endoscopic submucosal dissection (ESD). ESD was well tolerated in this patient, suggesting that it may be a therapeutic option for primary esophageal sarcomas.

Transoral Endoscopic Resection of Esophageal Liposarcoma

A 63-year-old man presented with a 1-month history of dysphagia. Endoscopy showed the patient had a mass in the cervical esophagus. Multiple biopsy specimens were taken, which were inconclusive for a diagnosis. A positron-emission tomography scan showed a hypermetabolic lesion. A transoral endoscopic approach was used to resect the tumor. Final histologic examination revealed a completely resected low-grade liposarcoma.

Giant liposarcoma of the esophagus with Li-Fraumeni-like syndrome

Primary esophageal liposarcoma is a rare malignancy, with only 25 cases reported in the English literature. In this report, we present a new case of giant esophageal liposarcoma in a 49-year-old woman with a family history of upper gastrointestinal tract cancer. This was a transmural tumor located in the lower esophagus. Subtotal esophagectomy was successfully performed, and histopathology revealed a well-differentiated liposarcoma. The patient was followed-up for 20 months without recurrence. However, asynchronous ovarian serous adenocarcinoma was found 1 year after the esophageal surgery, and Li-Fraumeni-like syndrome was diagnosed.

Well-Differentiated Liposarcoma of the Oesophagus: Clinicopathological, Immunohistochemical and Array CGH Analysis

Liposarcoma develops extremely rarely in the oesophagus. Microscopically, it exhibits subtle atypia of H&E-stained features. Accordingly, immunohistochemical features and chromosomal alterations are used for its confirmatory diagnosis. However, cytogenetic analysis has not been performed for oesophageal liposarcoma. We studied chromosomal alterations using array comparative genomic hybridization (CGH), as well as endoscopic, radiological, H&E-stained and immunohistochemical features in the oesophageal well-differentiated liposarcoma of a 67-year-old man. Array CGH analysis revealed the presence of high-level amplifications at chromosomal locations 1p12-1q21.2, 12q13.2-12q15 and 12q21.33-12q23.1. At least 29 genes were highly amplified (log(2) ratio >2), among which CDK4 and MDM2 were the most highly amplified (log(2) ratio >4) and were accepted as major target genes. Moreover, the amplification of AMDHD1, HAL and LTA4H (log(2) ratio = 3.153) was a novel finding. This case suggests the presence of a characteristic profile of gene amplification in well-differentiated liposarcoma of the oesophagus. The amplified genes may be of pathogenic importance for primary oesophageal well-differentiated liposarcoma.

Giant Esophageal Liposarcoma: A Rare Case Report

Purpose: Introduction: Esophageal liposarcoma is an extremely rare finding with only 18 reported cases. Our case describes a rare malignant tumor of the esophagus of substantial size. Case: A 75 year-old Hispanic male was referred for evaluation of dysphagia. He reports a 2 month history of progressive dysphagia to solids and now liquids, poor appetite, 11-lb weight loss, globus and rare melena. He has a 30-pack year of smoking. Exam was only remarkable for clubbing and pallor without lymphadenopathy. Labs revealed mild anemia. CT imaging revealed a heterogeneous, well-circumscribed mass, 18 cm long from the proximal esophagus to the fundus. EGD revealed a thick, smooth, tubular mass just below the UES extending the length of the esophagus into the fundus. The distal portion was nodular with an ulcerated tip. The patient underwent a total esophagectomy and was discharged in a few days without complications. Pathology revealed a 17x7x3cm encapsulated neoplasm weighing 120 grams. Lymphovascular and perineural invasion were absent. Immunohistochemical studies were positive for MDM2 and vimentin with a 5% proliferation rate seen with Ki-67 and negative for S-100 and actin. Diagnosis was consistent with a well-differentiated esophageal liposarcoma. Discussion: Liposarcomas are the most common soft tissue malignancy, but rarely located in the GI tract. Esophageal liposarcomas are least common comprising ˜1.5% of all GI cases. First reported in 1983; only 18 cases have been reported since then. Median age at presentation is 58 with a M:F ratio of 1.16:1. 84.6% originated in the upper esophagus, 34% were myxoid histology, while 53% were well-differentiated. One case was pleiomorphic. They usually don't cause symptoms until they reach a substantial size. Metastatic disease has not been documented. Treatment consists of endoscopic resection or esophagectomy with very high 10-yr survival rates. Liposarcomas just distal to the UES may be difficult to access for endoscopic resection. EUS should be performed to rule out a large vessel within the lesion to avoid excessive hemorrhage prior to endoscopic resection.Figure