Superficial Myxoid Liposarcoma of the Thigh

Myxoid liposarcoma is the most common form of myxoid sarcoma. Their primary and superficial localizations are very rare compared to the deeper forms. We report the case of myxoid liposarcoma in a 57-year-old patient. The clinical picture marked by painless swelling of the right thigh had evolved over a period of about ten years. The diagnosis was guided by ultrasound and magnetic resonance imaging. A pathological examination of the surgical specimen after removal of the tumor helped to clarify the diagnosis. We discuss the clinical presentation and therapeutic management.

Massive low-grade myxoid liposarcoma of the floor of the mouth:A case report and review of literature

BACKGROUND Oral liposarcoma is an extremely rare lesion that is often clinically misdiagnosed as a benign tumor due to its asymptomatic and indolent clinical course.Here,we report a case of massive low-grade myxoid liposarcoma(MLS)of the floor of the mouth.CASE SUMMARY A 71-year-old man presented with a huge mass in the left floor of the mouth.A biopsy was performed,and a diagnosis of a myxoid tumor suspicious for lowgrade MLS or myxoma was made.Gadolinium-enhanced T1-weighted magnetic resonance imaging showed an intensely enhanced tumor lesion that occupies the left sublingual space and extends to the submandibular space.Submandibular dissection,tumor resection,and reconstruction with a radial forearm flap were performed.The surgical specimen exhibited histologically low-grade MLS.Fused in sarcoma(FUS,also known as TLS)and DNA damage-inducible transcript 3(DDIT3,also known as CHOP)break-apart was not detected in the fluorescence in situ hybridization analysis.The tumor was completely encapsulated and did not require additional treatment.Furthermore,no recurrence was reported 40 mo after surgery.CONCLUSION We experienced an extremely rare,massive,low-grade MLS emerging from the floor of the mouth.Oftentimes,an MLS of the floor of the mouth lacks significant clinical findings and is often misdiagnosed.Although no FUS-DDIT3 fusion gene was detected,a low-grade MLS was ultimately diagnosed based on the histological findings.

Case Report： Myxoid Liposarcoma in Retroperitoneum

Liposarcoma is a malignant neoplasm of mesodermal origin, which among sarcomas, 10% to 20% are located in the retroperitoneum. The case presented shows a 50-year-old male patient who initially presented weight loss and abdominal pain in the left iliac fossa. A tumor mass of hardened consistency was palpated in virtually all the abdomen. An abdominal ultrasound and a computed tomography of the abdomen were performed and confirmed the tumor mass. An exploratory laparotomy was performed, with removal of bulky abdominal mass of greasy consistency. A histopathological study of the piece reported myxoid liposarcoma. Clinical and prognostic features, as well as oncologic outcomes, are well known in this group of patients. The patient has been in the outpatient clinic for 7 years without tumor recurrence. Computed tomography is the fundamental study for the diagnosis of imaging. The treatment of choice consists in an aggressive approach aiming the complete resection, which is a major predictor of local and distant recurrence and survival.

Liposarcoma Retro-Peritoneal in the General Surgery Department of the Hospital of the District of the Commune IV

Retroperitoneal soft tissue sarcomas in the retroperitoneal/intra-abdominal regions represent 10% - 15% of all cases of soft tissue sarcoma. Liposarcomas, which are the most common histological type, account for 20% - 45% of retroperitoneal/intra-abdominal sarcoma cases, and 20% of liposarcomas cases are primary retroperitoneal liposarcomas. Surgical resection in case of malignancy remains the treatment of choice for liposarcomas, according to the guidelines of most major international companies. Our goal was to improve the management of retroperitoneal liposarcoma. This was a 65-year-old patient, with no medical or surgical history, who was referred to us for abdominal swelling, in whom clinical and paraclinical examination found retroperitoneal liposarcoma stage IV, and the surgical treatment consisted in making a tumor reduction. Conclusion: Retro-peritoneal liposarcoma is an undervalued malignant tumor, and the diagnosis is often late.

Pleomorphic Nasopharyngeal Liposarcoma: An Aggressive Tumor

Nasopharyngeal liposarcoma is an extremely rare clinical entity. Less than ten cases have been reported in the literature. It mainly occurs in humans. The non-specificity of the clinical and radiological signs explains the difficulty of the diagnosis confirmed by anatomopathological and immunohistochemical examinations. Its pathogenesis remains obscure and often correlates with genetic abnormalities. Pleomorphic liposarcoma is its rarest subtype, remarkable for its aggressiveness and rapidity of growth. Complete surgical excision remains the treatment of choice. We report a case of liposarcoma whose diagnosis was made during histological examination coupled with immunohistochemistry. .

Mediastinal Liposarcoma: Case Report and Literature Review

Liposarcomas (LS) are the second most common type of soft tissue malignancies in adults;they arise from mesenchymal cells and account for 1% of all adult cancers and 15% of all pediatric cancers. The site of origin can be from anywhere there is fat in the human body. LS are classified based on the primary site of origin, and mediastinal LS are extremely rare. When mediastinal neoplasms are stratified based on histology, they represent less than 1% of all mediastinal tumors. To date, less than 150 cases have been reported in the English literature. This article aims to present an unusual case of an extremely rare malignancy and perform a systematic review of the latest literature. In this report, our group is documenting the presentation, management, and outcome of a 65-year-old male patient with a massive anterior mediastinal primary LS. The tumor was displacing the mediastinum into the right chest, occupying most of the left chest, and pushing the diaphragm into the abdomen. Mediastinal liposarcomas are extremely rare malignancies and can prove to be challenging to diagnose and treat. Aggressive surgical treatment with R0 resection is the gold standard, however, tumor biology in many cases is associated with variable growth rates and encroachment of adjacent vital structures and blood vessels. When complex anatomical structures preclude an R0 resection, there is a high incidence of local recurrence. In cases where there is a high risk of recurrence, radiotherapy is indicated and chemotherapy has a more limited role.

Primary gastric dedifferentiated liposarcoma resected endoscopically:A case report

BACKGROUND Liposarcoma is one of the most common adult mesenchymal tumors but is uncommon in the gastrointestinal tract and extremely rare in the stomach.Furthermore,the histological subtypes of liposarcoma usually reported in the stomach are well-differentiated or myxoid,and few reports have been issued on small-sized gastric liposarcomas resected endoscopically and followed up.Herein,we report a case of primary gastric dedifferentiated liposarcoma(DL)that was resected endoscopically.CASE SUMMARY A 67-year-old female Korean patient was referred to our institution for further evaluation of a gastric submucosal tumor(SMT)located in the lesser curvature of the gastric body by esophagogastroduodenoscopy.Endoscopic ultrasound revealed a well-circumscribed,slightly heterogeneous,isoechoic,17 mm×10 mm sized mass originating from the third sonographic layer.Computed tomography showed no evidence of significant lymph node enlargement or distant metastasis.Endoscopic resection was undertaken using the snare resection technique after mucosal precutting to provide a definitive histopathologic diagnosis,which proved to be consistent with DL,based on its morphology and the immunoexpressions of MDM2 and CDK4.The patient was planned for surgery because the deep resection margin was positive for malignancy.After declining any invasive procedure or adjuvant treatment,the patient was placed under close follow-up,and at one year after endoscopic resection,remained disease free.CONCLUSION This is the first reported case of a small primary gastric DL resected endoscopically and followed up.This report demonstrates that when diagnosis of a SMT is uncertain,the use of invasive techniques,including endoscopic resection,should be considered.

A Case of Giant Mediastinal Liposarcoma of Thymic Origin: A Rare Clinical Entity

Thymoliposarcoma is an exceedingly rare tumor of thymus with a very few cases reported till date. This case study presents a 45-year male with rare type of thymoma. On the contrast-enhanced CT images, there was a large mass lesion of predominantly fat attenuation in the pre-vascular compartment of the mediastinum insinuating on both sides of the visceral compartment of the mediastinum, and extending upto the bilateral cardio phrenic and anterior costophrenic angles, anterior to the right ventricle with loss of fat plane with the pericardium, with few sub-centimetric lymph nodes in the right paratracheal and AP window and a calcified right hilar lymph node, suggestive of well-differentiated liposarcoma/thymoliposarcoma. Initial CT guided tru-cut tissue biopsy was inconclusive, and the repeat biopsy revealed as fibro-collagenous tissue with area of necrosis, focal myxoid changes in the background with presence of cells which are spindle to oval in shape with mild nuclear pleomorphism and negative for S100, Cytokeratin, CD34, desmin. The entire tumor was resected en masse after meticulous dissection without the support of cardiopulmonary bypass (CPB) with an intact pericardium. Final histopathology report of the surgical biopsy specimens is consistent with dedifferentiated thymoliposarcoma with focal ganglionic cell differentiation. Postoperative follow-up CECT of thorax revealed no evidence of residual mass in the pre-vascular compartment. The patient is disease-free and asymptomatic after 6-month and he is under routine follow-up under Radiotherapy department since he received 30 Gy of postoperative radiotherapy (PORT).

Giant retroperitoneal liposarcoma treated with radical conservative surgery: A case report and review of literature

BACKGROUND Retroperitoneal liposarcoma(RLPS)is a rare malignant tumor of the connective tissue and usually grows to a large size,undetected.Diagnosis is currently based on collective findings from clinical examinations and computed tomography(CT)and magnetic resonance imaging,the latter of which show a fat density mass and possible surrounding organ involvement.Surgical resection is the main therapeutic strategy.The efficacy and safety of further therapeutic choices,such as chemotherapy and radiotherapy,are still controversial.CASE SUMMARY A 61-year-old man presented with complaint of a large left inguinal mass that had appeared suddenly,after a slight exertion.Ultrasonography revealed an omental inguinal hernia.During further clinical examination,an enormous palpable abdominal mass,continuing from the left inguinal location,was observed.CT revealed a giant RLPS,with remarkable mass effect and wide visceral dislocation.After multidisciplinary consultation,surgical intervention was performed.Subsequent neoadjuvant chemotherapy and radiotherapy were precluded by the mass’large size and retroperitoneal localization,features typically associated with non-response to these types of treatment.Instead,the patient underwent conservative treatment via radical surgical excision.After 1 year,his clinical condition remained good,with no radiological signs of recurrence.CONCLUSION Conservative treatment via surgery resulted in a successful outcome for a large RLPS.

Giant liposarcoma of the esophagus: A case report

Liposarcomas rarely develop in the aerodigestive tract.Here,we present a primary esophageal liposarcoma that was discovered between the T3 and T7 levels of the esophagus during right pleural exploration of a 51-year-old male patient.The patient had presented with non-specific symptoms,including progressive dysphagia over the previous 6 mo,without complaints of chest or epigastric pain,regurgitation,or weight loss.A radical three-hole esophagectomy was performed.The tumor was extremely large(14 cm × 7.0 cm × 6.5 cm),but completely encapsulated.Upon histological examination,the tumor was diagnosed as a giant,well-differentiated esophageal liposarcoma with a dedifferentiated component.Non-specific radiological and endoscopic results during the clinical work-up delayed diagnosis until post-operative histology was performed.In this report,the clinical,radiological and endoscopic diagnostic challenges specific to the case are discussed,as well as the surgical and pathological findings.

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.

Clinicopathological Features of Extraskeletal Myxoid Chondrosarcoma:An Analysis of 9 Cases

Objective： To investigate the Clinicopathological （EMC）. Methods： Nine cases of extraskeletal characteristics of extraskeletal myxoid chondrosarcoma myxoid chondrosarcoma were studied. Extensive immunohistochemical analysis was performed in all the cases and ultrastructural studies were done in 2 extraskeletal myxoid chondrosarcomas. Follow-up information was available for seven patients. Results： There were 7 males and 2 females whose ages ranged from 31 to 69 years （median 52.78 years）. Local pain or tenderness and the presence of a palpable mass were the main complaints of the patients. The tumors were located mainly in the lower extremities （66.7%）. Most tumors were deep-seated. They usually had a distinct multinodular configuration delineated by fibrous connective tissue. The tumor cells were arranged in delicate intersecting strands, rings, and garlands for the most part. The myxoid matrix was abundant in most cases. Immunohistochemical analysis was performed in all the cases and ultrastructural studies were done in 2 extraskeletal myxoid chondrosarcomas. EMC expressed vimentin （100%, 9/9）, neuron-specific enolase （77.8%, 7/9）, S-100 protein （66.7%, 6/9）, synaptophysin and chromogranin A （22.2%, 2/9）. None of the tumors expressed EMA and desmin. Ultrastructurally： EMC was characterized by distinct cords of cells immersed in a glycosaminoglycan rich matrix. The cells were rich in mitochondria, had well-developed Golgi apparatus and there were numerous smooth vesicles. In many cells, there were also prominent glycogen deposits and lipid droplets. Some tumor cells had intracisternal microtubules. In one of the 2 extraskeletal myxoid chondrosarcomas there were 140-180 nm diameter membrane-bound dense-core secretory granules in cell bodies. Conclusion： Extraskeletal myxoid chondrosarcoma （EMC） is a rare soft tissue sarcoma characterized by distinctive morphological and cytogenetical features. However, the chondroid nature has been a subject of controversy, and its line of differentiation remains to be determined. A substantial proportion of EMC shows immunophenotypic and/or ultrastructural evidence of neuroendocrine differentiation. EMC has high potential of local recurrence and metastasis, and a high disease-associated death rate.

Extraskeletal myxoid chondrosarcoma metastatic to the pancreas: A case report

Extraskeletal myxoid chondrosarcoma (EMC) is a lowgrade sarcoma characterized by developing metastases and local recurrence in high rate. It is mainly deep seated in the proximal extremities. The most common metastatic sites are the lungs, soft tissues, lymph nodes, bones and the brain. To our knowledge, no case of clearly defined EMC has been reported to date developing a metastasis in the pancreas. We describe a case of a man suffering from EMC who developed a single pancreatic metastasis 20 years after the initial diagnosis. A 49-year-old man was submitted to surgical excision of an EMC, in left thigh, 20 years ago. Fourteen years after the initial diagnosis a local recurrence in left thigh occurred. Multiple lesions of metastatic origin, in both lungs, were excised via thoracotomies until the time being. In 2003, as a part of a periodically performed imaging control, an abdominal CT scan was performed revealing a solid lesion in the pancreas. Distal pancreatectomy was performed. The histopathology of the excised specimen proved to be the one of metastatic lesion of EMC. The above-mentioned case of EMC is, as far as we know, the first one described developing a certain pancreatic metastasis.

Dedifferentiated liposarcoma of the small bowel mesentery presenting as a submucosal mass

Dedifferentiated liposarcoma(DDLPS) is a variant of liposarcoma but with a more aggressive course.It occurs most commonly in the retroperitoneum and rarely in any other anatomical location.We describe a case of DDLPS arising from the small bowel mesentery presenting as submucosal mass of the small bowel.The current case is unusual as the tumor originated from the small bowel mesentery and a dedifferentiated component transmurally invaded the small bowel wall,including the small bowel submucosa.

Giant exophytic renal angiomyolipoma masquerading as a retroperitoneal liposarcoma: A case report and review of literature

A 42-years-old lady, presented with a large retroperitoneal mass which was preoperatively diagnosed as a retroperitoneal liposarcoma following an image guided core biopsy. She underwent a margin-negative resection of the retroperitoneal mass(multi visceral resection-enbloc excision of the retroperitoneal mass with a left nephrectomy and a segmental descending colectomy). The final histopathological examination of the resected specimen confirmed an exophytic renal angiomyolipoma(AML) which was extending into the retroperitoneum. AML is a rare benign tumor arising most commonly from the kidney. It can sometimes present as a diagnostic challenge as it mimics a retroperitoneal liposarcoma or a fat-containing renal cell carcinomas closely. We present this case to share our experience of managing a case of giant exophytic AML which resembled retroperitoneal liposarcoma closely and resulted into an aggressive surgery.

Huge primary dedifferentiated pancreatic liposarcoma mimicking carcinosarcoma in a young female: A case report

BACKGROUND Pancreatic liposarcoma is a rare tumor. According to a literature review, the patient described in this study is the seventh case of pancreatic liposarcoma reported in the English literature and the third case of dedifferentiated liposarcoma. Furthermore, this case had the largest primary tumor volume, and a primary pancreatic liposarcoma was diagnosed based on sufficient evidence. CASE SUMMARY We here report a rare case of a 28-year-old female with a huge dedifferentiated liposarcoma in the pancreatic tail. In June 2015, the patient underwent distal pancreatectomy with splenectomy. During the operation, a huge liposarcoma of approximately 28.0 cm × 19.0 cm × 8.0 cm was found, which had a yellow and white fish-like incisal surface. Based on both pathology and MDM2 gene amplification, the tumor was diagnosed as a dedifferentiated liposarcoma. The patient was treated with surgery but declined postoperative chemotherapy. She was well at the 26-mo follow-up, and no relapse was observed. CONCLUSION Pancreatic liposarcoma has a low incidence. Chemotherapy should be included in the treatment regimens. Complete resection is the only effective treatment.

Primary liposarcoma of esophagus:A case report

Liposarcoma is the most common soft tissue sarcoma in adult life while esophageal liposarcoma is an extremely rare tumor. In the world literature, only 14 cases of esophageal liposarcomas have been described. We report a 72-year old male patient who was urgently admitted to our hospital for acute epigastric pain with a burning retrosternal sensation, persistent nausea, vomiting and dysphagia. Barium swallow, upper gastrointestinal （GI） endoscopy, esophageal manometry and CT scan, failed to accurately diagnose the lesion. After surgical resection of an esophageal polypoid tumor, the histological examination revealed a well-differentiated grade Ⅰ liposarcoma. Diagnostic and therapeutic tools were discussed and the results of literature were reviewed.

Liposarcoma of the breast arising in a malignant phyllodes tumor:A case report and review of the literature

Liposarcoma of the breast is a very rare malignant tumor. It can clinically manifest as a palpable breast mass and mimic primary breast cancer. We report an unusual case of a 51-year-old female who presented with an asymptomatic right breast mass, which was histologically diagnosed as well differentiated liposarcoma arisen within malignant phyllodes tumor. The patient underwent breast conserving surgery, received no adjuvant treatment and is disease-free after 2 years. Radiological and histopathological features are presented and described in detail. Data from the literature are presented and therapy recommendations discussed.

Successful outcome following resection of a pancreatic liposarcoma with solitary metastasis

Liposarcomas are rare soft tissue tumors, commonly affecting the lower limbs and less commonly the retroperitoneum. Although other organs can be affected,the pancreas is one of the rarest, and metastasis at presentation has never been reported. We describe the case of a 76-year-old gentleman presenting with abdominal pain and an abdominal mass. Imaging confirmed a primary tumor in the body and tail of the pancreas, with a metastatic deposit in the mesentery adjacent to the second part of the duodenum. Biopsy confirmed a liposarcoma, and subsequently a complete surgical excision was achieved. He then received adjuvant radiotherapy and has remained disease free for the next 26 mo.

Dedifferentiated liposarcoma of the rectum:A case report

Liposarcoma is one of the most common soft tissue sarcomas found in adults,and it usually occurs in the retroperitoneum and the extremities.Here,we describe a case of dedifferentiated liposarcoma originating from a well-differentiated liposarcoma of the mesorectum that presented as a protruding mass in the rectal lumen.Hartmann's operation with total mesorectal excision was performed and the tumor was removed radically.No management guidelines are currently available for liposarcoma of the rectum.We propose that complete surgical resection be required for the treatment of rectal liposarcoma and that a long-term detailed follow up is necessary.