Primary liposarcoma of the left atrium surgically treated

A case of myxoid liposarcoma of the left atrium in a 35-year old man is reported. The tumour, revealed by an atrial flutter, was excised. The patient died 13 months later, with multiple metastases. A review of the literature yielded only 7 cases of primary liposarcoma of the heart, including 4 which were surgically treated. This confirms the extreme rarity of a tumour which has a very poor prognosis due to recurrence in situ or metastatic spread.     

Rare myxoid liposarcoma metastasis to the interventricular septum of the heart

Liposarcomas are malignant tumors of the soft tissue. Myxoid liposarcoma is the second most common subtype of these tumors in adults. It accounts for approximately 20% of all malignant soft tissue tumors [1], [2]. Peak of its incidence occurs between 40 to 60 years of age with relatively indolent clinical course Matsumoto et al. (2007) [3], Cho et al. (2010) [4], Faiman et al. (2005) [5]. Typical localizations of myxoid liposarcoma comprise limbs, particularly thighs with a tendency to metastasize into extrapulmonary sites such as retroperitoneum, mediastinum, bones. Cardiac metastases are extremely rare.We present a case of a 36-year-old man with a history of recurrent myxoid liposarcoma. Primary location was in the left popliteal area. After extirpation of the tumor, metastatic tumor was subsequently revealed in the right axilla. Each surgical extirpation was followed by radiation therapy and brachytherapy. Cardiac metastasis was accidentally diagnosed with PET/CT during the staging process. The patient was asymptomatic and was admitted to our institution for further diagnostics and treatment. After confirmation of its location, the tumor was excised. Histological examination revealed myxoid liposarcoma.     

Primary pulmonary myxoid liposarcoma discovered fortuitously

We report a fortuitous discovery of primary pulmonary myxoid liposarcoma in an HIV-positive patient. Primary pulmonary localizations are uncommon. Generally, pulmonary localizations are metastatic. There is a male predominance and diagnosis is generally made around 40 years of age. The two main features of liposarcoma are the large tumor size and the complex histology that evolves over time. Pathology findings are rarely reproducible and vary from one pathologist to another. Macroscopically, liposarcomas can mimic benign tumors. The risk of recurrence is high after simple enucleation due to microscopic extracapsular extensions. Surgery remains the predominant treatment. Wide complete excision, if possible, provides long-term survival.     

Myxoid Liposarcoma in the Abdominal Wall: A Case Report

A liposarcoma is the most common type of soft tissue sarcoma, and most liposarcomas are malignant. The extremities are the most common site for liposarcomas. There are 5 histologic types of liposarcoma, as follows: well differentiated; myxoid; round cell; pleomorphic; and dedifferentiated. Myxoid liposarcomas (MLSs) represent a subgroup of liposarcomas. There has been no report of MLSs in the abdominal wall.We report a rare case of a MLS of a 43-year-old male who presented with tensile force on the abdominal wall. Computed tomography (CT) found a tumor in abdominal wall. There was no other abnormal symptom and the laboratory testing was also unusual.At last, the tumor was successfully excised, which was diagnosed MLSs in pathology. Following standard principles, after complete excision, the patient received radiotherapy. The patient was followed up for 8 month and no disease recurrence was identified.MLSs are rarely seen in the clinic, irrespective of the presenting signs, but also based on histologic features. The aim of this report was to present the differential diagnosis of an abdominal wall mass, and to remind us of MLSs.     

Giant retroperitoneal liposarcoma. One case report

INTRODUCTION: Retroperitoneal Liposarcoma is a rare and primary malignancy developed from mesenchymal tissue. It's generally unique and shows an only one histologic component. We report a case of multicentric and synchronous liposarcoma (intraperitoneal and retroperitoneal) and with different histologic types. EXEGESES: A 53-year old man presented with abdominal pain, increased abdominal girth and weight loss. Physical examination revealed two abdominal masses. Computed tomography scan showed a very large retroperitoneal mass displacing the left kidney, digestive loops and vessels. The patient underwent surgery. One enormous mass of the left renal lodge measuring 50 cm, a retroduodenal mass measuring 15 cm and a nodule of the coecum were removed. Histological examination of the mass of the left renal lodge revealed mixed type liposarcoma (dedifferentiated and myxoid). Histological examination of the retroduodenal mass and of the nodule of the coecum revealed well-differentiated liposarcoma. CONCLUSION: Pathological, therapeutic and prognostic aspects of abdominal liposarcoma will be reviewed in this article.     

Myxoid liposarcoma of the mediastinum

We describe a mediastinal myxoid liposarcoma case in a 47-year-old woman who complained of dyspnea. This kind of tumor is rare and becomes symptomatic by compression on mediastinal structures, especially on the respiratory tract. These neoplasms are therefore often voluminous at the time the diagnosis is made. Histopathologic examination is always necessary as much for diagnosis as prognosis. Surgery, whether radical or palliative, obtain the best therapeutic survival results in myxoid liposarcoma of the mediastinum.     

A surgical management of aortic insufficiency concomitant with mediastinal well-differentiated liposarcoma

We present a rare case of a mediastinal liposarcoma concomitant with aortic insufficiency due to myxoid degeneration of the aortic valve. Because the patient's left ventricle was in moderate dilatation and a posterolateral thoracotomy combined with median sternotomy was required in order to perform a complete resection of a mediastinal liposarcoma, it was decided to carry out aortic valve surgery and tumor excision in one operation.     

A patient with mediastinal liposarcoma showing dedifferentiation]

A 70-year-old man visited our hospital with fever and general malaise. Chest CT scanning showed a large tumor shadow in the anterior mediastinum. The tumor was resected. It consisted of a portion rich in fat on the anterior mediastinal side and a solid portion on the left thoracic cavity side. Pathological examination demonstrated liposarcoma (myxoid type). The solid portion was considered to have resulted from dedifferentiation of the cystic portion. Mediastinal liposarcoma accounts for less than 1% of all mediastinal tumors. To our knowledge, there have been no previous reports of patients with myxoid-type mediastinal liposarcoma showing dedifferentiation.     

原发性胸膜脂肪肉瘤的临床特点

目的原发性胸膜脂肪肉瘤是一种非常罕见的肿瘤，本文报道国内首例病例，并探讨其临床特点、诊断及治疗。方法分析我院近期发现的1例原发性胸膜脂肪肉瘤的临床资料并复习相关文献。结果原发性胸膜脂肪肉瘤全世界仅报道17例（包括此例）。本病好发于男性，中老年人多见，病理类型以黏液型多见；临床症状以胸痛和气促最常见；诊断主要依靠影像学和开胸探查。目前治疗推荐手术切除加辅助放疗。结论原发性胸膜脂肪肉瘤的治疗和预后的评估有待进一步积累资料。     

Giant metastatic cardiac and abdominal liposarcomas causing hemodynamic deterioration

Myxoid liposarcoma usually develops in the retroperitoneum or deep soft tissues of extremities. Synchronous cardiac and abdominal metastases are extremely rare. We describe the case of a 63-year-old man, with a history of a thigh liposarcoma, treated by resection, chemotherapy and postoperative radiotherapy 13 years ago. Patient now presented with an intracardial, a pericardial and an abdominal mass, identified as metastatic myxoid liposarcomas. He eventually underwent successful emergency sternotomy and subsequent laparotomy for tumor resection.     

A novel germline mutation of MSH2 in a hereditary nonpolyposis colorectal cancer patient with liposarcoma

BACKGROUND: One of the clinical features of hereditary nonpolyposis colorectal cancer (HNPCC) is a high incidence of multiple primary neoplasms arising in various organs including the gastrointestinal and genitourinary tracts. Among extracolonic tumors, a limited number of soft tissue sarcomas associated with HNPCC have been reported, and the mechanism underlying liposarcoma in HNPCC patients remains unclear. AIM: We herein report the case of a HNPCC patient with liposarcoma, with the goal of elucidating the involvement of a mismatch repair deficiency in the tumor. METHODS AND RESULTS: A 40-yr-old Japanese patient, who had a past history of adenocarcinoma of the rectum and transitional cell carcinoma of the urinary bladder, developed a liposarcoma in his left thigh. Although his family history did not fulfill the revised Amsterdam criteria, his blood sample was subjected to genetic testing. Direct sequencing of the genomic DNA from the blood identified an AT deletion at codon 677 in exon 13 of hMSH2, a pathogenic mutation that has not been reported before. The expression of MSH2 in the liposarcoma and rectal cancer of the patient was analyzed by immunohistochemistry, which revealed loss of MSH2 expression in the tumors. To investigate whether the loss of MSH2 was a common feature of liposarcoma, we examined the MSH2 expression in an additional two sporadic liposarcomas, both of which were stained with anti-MSH2 antibody. CONCLUSION: We identified a novel pathogenic germline mutation of MSH2 in an HNPCC patient. Since an immunohistochemical analysis showed no nuclear staining for MSH2 protein in the liposarcoma as well as the rectal cancer, the loss of wild-type MSH2 protein was thus considered to possibly play a role in the development of liposarcoma in HNPCC patients.     

胸膜弥漫性黏液性恶性纤维组织细胞瘤一例并文献复习

目的探讨胸膜弥漫性黏液性恶性纤维组织细胞瘤（MFH）的临床和病理特点。方法分析我院收治的1例胸膜弥漫性黏液性MFH患者的临床和病理资料，并分析国内外文献报道的7例胸膜MFH病例。结果患者为男性，65岁，表现为咳嗽、胸痛、气促伴大量血性胸腔积液。胸部CT示右侧脏壁层胸膜弥漫性肿块影。开胸探查见脏壁层胸膜表面弥漫性灰白色半透明肿块，直径为1～8cm。光镜下可见黏液性基质中瘤细胞弥漫分布，呈梭形和多形性。波形蛋白、CD68、溶菌酶免疫组化染色瘤细胞呈阳性反应，细胞角蛋白、上皮膜抗原、结蛋白呈阴性反应。术后24d，因上腔静脉梗阻、循环衰竭死亡。与文献报道的非弥漫性胸膜MFH相比，本例为胸膜弥漫性多灶性MFH，起病急，病情进展快。结论胸膜弥漫性黏液性MFH是非常罕见的胸膜原发性肿瘤，恶性程度高，预后极差。     

Liposarcoma of the stomach： A rare case report

Liposarcoma is the most common soft tissue sarcoma and accounts for 15%-20% of all mesenchymal malignancies. The tumor occurs most frequently in the limbs, retroperitoneum and rarely has a visceral location. We report a case of a gastric liposarcoma in a male patient. A 68 years old male patient was admitted to hospital for abdominal discomfort and fullness lasting for a month. He reported rare episodes of vomiting. The CT examination revealed a large epigastric mass （8 cm × 4 cm） involving the lesser curvature of the stomach, in contact with the pancreas and gallbladder. Fatty areas within the mass were evident. A total gastrectomy together with cholecystectomy was performed. The histopathological diagnosis was a well differentiated liposarcoma. The patient did not undergo any adjuvant treatment, he is under close follow up and two years later he is disease free. We report this case due to the rarity of this tumor in the stomach （nine cases reported in the literature）.     

Large-cell lymphoma. An unusual late relapse

We report a case of a 40-year-old man with a stage 4, anaplastic, large-cell lymphoma. He had been diagnosed 13 years before as having a liposarcoma, at which point he was treated with combination chemotherapy, which included anthracycline. On review of the histopathology from 13 years before, the original diagnosis of liposarcoma was revised to that of an anaplastic large-cell lymphoma. A diagnosis of relapsed anaplastic large-cell lymphoma was made. A MUGA scan showed a reduced ejection fraction of 46%. Our patient responded initially to combination chemotherapy, which included anthracycline, without further reduction in his ejection fraction. This was followed by high-dose chemotherapy and peripheral blood stem-cell transplantation. Twenty months later he is well and remains in complete remission.     

An unusual late relapse

We report a case of a 40‐year‐old man with a stage 4, anaplastic, large‐cell lymphoma. He had been diagnosed 13 years before as having a liposarcoma, at which point he was treated with combination chemotherapy, which included anthracycline. On review of the histopathology from 13 years before, the original diagnosis of liposarcoma was revised to that of an anaplastic large‐cell lymphoma. A diagnosis of relapsed anaplastic large‐cell lymphoma was made. A MUGA scan showed a reduced ejection fraction of 46%. Our patient responded initially to combination chemotherapy, which included anthracycline, without further reduction in his ejection fraction. This was followed by high‐dose chemotherapy and peripheral blood stem‐cell transplantation. Twenty months later he is well and remains in complete remission.     

肺转移性脂肪肉瘤1例及文献复习

目的 探讨肺转移性脂肪肉瘤的临床和病理特点,减少误诊、漏诊.方法 对收治的1例肺转移性脂肪肉瘤患者的临床资料进行回顾性分析.结果 本例为55岁男性,因“咳嗽伴气促1个月,加重1d”入院.CT和胸水B超示右侧包裹性胸腔积液,肿瘤声学造影示右肺占位.胸外科手术切除右肺肿块,术后病理提示圆细胞型脂肪肉瘤.术后患者出现四肢、臀部、腹部巨大肿块,且生长迅速,术后给予放化疗及对症支持治疗.结论 肺原发性和转移性脂肪肉瘤临床均少见,症状隐匿、缺乏特异性,易误诊,B超肿瘤声学造影有助于早期鉴别.除手术切除外,生长迅速、多发转移者可考虑术前、术后联合放化疗.     

Liposarcoma of the stomach: Report of two cases and review of the literature

Liposarcoma of the stomach is extremely rare, and only 37 cases have been reported worldwide. We herein report two cases of liposarcoma of the stomach. The first patient was referred to our hospital with upper abdominal discomfort. The endoscopic examination revealed a tumor mass about 3 cm in diameter. The patient underwent a partial gastrectomy and had an uneventful recovery. The histopathological examination revealed a welldifferentiated liposarcoma. The second patient had symptoms of upper abdominal discomfort combined with nausea and anorexia. Several palpable masses were found with endoscopy. Endoscopic submucosal dissection was the treatment used, and the postoperative course was uneventful. The histopathological diagnosis was a well-differentiated liposarcoma. The two patients did not undergo any adjuvant therapy. They are both currently in good condition without recurrence. Therefore, we believe that the outcome of liposarcoma of the stomach is positive, and surgical resection may be the first choice for treatment at present.     

Primary myxoid sarcoma of the pleura: 5 years follow-up

Primary sarcomas of the pleura are rare intrathoracic tumours. We describe a case of a recurring myxoid pleural sarcoma in a 51-year-old male patient with coronary heart disease, who initially was admitted for coronary revascularization surgery. Tumour diagnosis was based on pathological examination of a biopsy after radiological detection of a nodule in the right upper lobe. The nodule was excised. The first recurrence was diagnosed after 15 months. Pleuropneumonectomy was done. Four years after the first excision further tumour masses appeared above the diaphragm and were surgically removed and adjuvant irradiation carried out. A year later the patient is doing well.     

Myxoid liposarcoma of the supraclavicular fossa

Liposarcomas generally originate most often in the extremities or retroperitoneum, less frequently in the head and neck, and rarely in the thorax. We describe a particularly rare presentation of myxoid liposarcoma originating in the supraclavicular fossa. The mass was resected and has not recurred. We searched our pathology database for other soft-tissue tumors of the supraclavicular fossa and found no other case of sarcoma originating in this site. In addition, we performed a literature review of thoracic and neck liposarcomas to identify similar cases and discuss their clinical course.