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 explain...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. .展开更多
AIM:To explore the clinical and pathological characteristics of thirteen patients with orbital liposarcoma.METHODS:The clinical history data of thirteen patients diagnosed as orbital liposarcoma at Beijing Tongren Hos...AIM:To explore the clinical and pathological characteristics of thirteen patients with orbital liposarcoma.METHODS:The clinical history data of thirteen patients diagnosed as orbital liposarcoma at Beijing Tongren Hospital,from 2006 to 2021 were collected and analyzed.The data includes age,gender,affected orbital side,course of disease,status of disease(primary or recurrent),clinical manifestations,preoperative visual acuity,operative treatment,the relations between liposarcoma and surrounding tissue,longest diameter of liposarcoma,histological subtype,immunohistochemical indicators,follow-up treatment and prognosis.RESULTS:The initial symptoms are diverse.Proptosis is the most frequent chief complaint and the others included vision loss,epiphora,diplopia,and eyelid palpable mass.Results of imaging examination[computed tomography(CT)or magnetic resonance imaging(MRI)]showed orbital mass.In terms of treatment,10 patients received tumor resection,and the mean longest diameter of the tumor was 3.39±1.36 cm.The other 3 patients had optic nerve invaded,so they received orbital exenteration.Pathological examination results confirmed the diagnose of liposarcoma for 13 patients.Six patients displayed as myxoid type,and three patients in each type of dedifferentiated and welldifferentiated type.One patient was verified as pleomorphic,which was a rare type of liposarcoma.All of the patients showed Vimentin positive,and most showed CD34 and S-100 positive.Besides,four patients showed smooth muscle actin positive.All thirteen patients were alive.CONCLUSION:Orbital liposarcoma is a rare disease and it has no specific clinical manifestation.The diagnosis of liposarcoma should be considered when proptosis and orbital mass occurred in orbit.It is recommended to perform pathological examination to achieve early detection and early treatment.展开更多
BACKGROUND Dedifferentiated liposarcoma(DDLS)has a worse prognosis and occurs most commonly in the retroperitoneal region and rarely in the intraperitoneal region.Histological diagnosis was revolutionized by the combi...BACKGROUND Dedifferentiated liposarcoma(DDLS)has a worse prognosis and occurs most commonly in the retroperitoneal region and rarely in the intraperitoneal region.Histological diagnosis was revolutionized by the combined contributions of histoimmuno-chemistry and molecular biology.Aside from surgery,there is no consensus on the optimal treatment for this chemoresistant cancer.CASE SUMMARY A thirty-year-old black female presented with a large painful abdominal mass occupying nearly the entire abdomen and progressive weight loss was admitted for surgery.Abdominal computed tomography showed a large heterogeneous mass of the mesentery that was sized 18 cm×16 cm in size and had heterogeneous contrast enhancement.During laparotomy,en bloc excision of the large and multilobulated gastrocolic ligament mass was performed.The initial postoperative histopathological diagnosis was undifferentiated sarcoma.Finally,the results of immunohistochemistry and molecular biology allowed us to confirm the diagnosis of DDLS.The tumour followed an aggressive evolution with diffuse metastasis,causing the death of the patient less than 5 mo after the operation.CONCLUSION Dedifferentiated liposarcomas are rare tumours that typically originate in the retroperitoneum but may arise in unexpected locations.展开更多
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 ca...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 </span><span style="font-family:Verdana;">mediastinal tumors. To date, less than 150 cases have been reported in the</span><span style="font-family:Verdana;"> 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 </span><span style="font-family:Verdana;">this report, our group is documenting the presentation, management, and</span><span style="font-family:Verdana;"> 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.展开更多
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 re...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.展开更多
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 examination...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.展开更多
<span style="font-size:12px;">Thymoliposarcoma is an exceedingly rare tumor of thymus with a very few cases reported till date. </span>This case study presents a 45-year male with rare type of th...<span style="font-size:12px;">Thymoliposarcoma is an exceedingly rare tumor of thymus with a very few cases reported till date. </span>This case study presents a 45-year male with rare type of thymoma. <a name="_Hlk35733991"></a><span>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.</span><a name="_Hlk35738313"></a><span> 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.</span><a name="_Hlk40534514"></a><span> Postoperative follow-up CECT of thorax revealed no evidence of residual mass in the pre-vascular compartment. The patient is disease-free an</span><span><span>d</span></span><span><span> asymptomatic after 6-month and he is under routine follow-up under Radiotherapy department</span></span><span style="font-family:;" "=""> <a name="_Hlk41419004"></a><span>since he received 30 Gy of postoperative radiotherapy (PORT).</span></span>展开更多
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-o...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.展开更多
AIM: To report the abnormal type of the orbital liposarcoma-dedifferentiated subtype in a patient. METHODS: A case report. RESULTS: A 23 years old Chinese woman with a recurrence of right-sided proptosis was evaluated...AIM: To report the abnormal type of the orbital liposarcoma-dedifferentiated subtype in a patient. METHODS: A case report. RESULTS: A 23 years old Chinese woman with a recurrence of right-sided proptosis was evaluated. Ocular examination revealed proptosis of the right eye with chemosis, hyperemia and limitation of eye movements. Magnetic resonance imaging scanning showed an irregular shaped tumor in the right orbit. The tumor resection was done with a clinical diagnosis of malignant tumor. Histopathological findings revealed the diagnosis of dedifferentiated liposarcoma. CONCLUSION: The rare occurrence of this tumour should be kept in mind while dealing with orbital tumours.展开更多
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 ...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.展开更多
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 ...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.展开更多
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 abdomin...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.展开更多
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 c...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.展开更多
BACKGROUND Pancreatic lipomas are thought to be very rare.Lipomas are usually easy to identify on imaging,particularly via computed tomography (CT).But sometimes it’s quite difficult to distinguish a lipoma from a we...BACKGROUND Pancreatic lipomas are thought to be very rare.Lipomas are usually easy to identify on imaging,particularly via computed tomography (CT).But sometimes it’s quite difficult to distinguish a lipoma from a well-liposarcoma without histologic result.CASE SUMMARY Here,we present a case of pancreatic lipoma in a 59-year-old female.She was asymptomatic and had no medical history of note.CT and magnetic resonance imaging revealed a mass like well-differentiated liposarcoma in the pancreatic head,positron emission tomography/CT showed a low fluorodeoxyglucose uptake and laboratory tests revealed elevated transaminase and carbohydrate antigen-199 levels.Finally,the patient underwent a pancreaticoduodenectomy.Histologically,mature adipocytes were noted in the bulk of the tumor.Accordingly,the pathologic diagnosis of the pancreatic neoplasm was lipoma.To our knowledge,this case is the first example of a suspected well-differentiated liposarcoma that was actually a pancreatic lipoma.We also highlight the radiological features distinguishing a pancreatic lipoma from a pancreatic liposarcoma and briefly review the literature.CONCLUSION Pancreatic lipomas show no obvious gender bias and most commonly occur in the head of the pancreas,of which the maximum diameters are often less than 5 cm,and small,asymptomatic non-compressed lipomas require follow-up only.Surgical excision should be considered when the tumor has compressed important tissues or is difficult to distinguish from a liposarcoma,the choice of surgery depends on the intraoperative presentation.展开更多
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 a...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.展开更多
BACKGROUND Esophageal liposarcoma is a rare malignant tumor and an esophageal dedifferentiated liposarcoma(DDL)is extremely rare.There are no reports on the treatment of DDL by thoracoscopic surgery.CASE SUMMARY A 38-...BACKGROUND Esophageal liposarcoma is a rare malignant tumor and an esophageal dedifferentiated liposarcoma(DDL)is extremely rare.There are no reports on the treatment of DDL by thoracoscopic surgery.CASE SUMMARY A 38-year-old woman presented with dysphagia and dyspnea.Imaging examination showed a large mass in the posterior mediastinum.The patient also developed respiratory failure and it was unclear whether this was caused by a mass from inside or outside the esophagus.We decided to perform thoracoscopic exploration to relieve the obstruction caused by tracheal compression.The upper segment of the esophagus was split longitudinally,and most of the mass could be removed from the esophageal lumen to the thoracic cavity.The pedicle was excised by linear cutting closers under mirrors.Little residual mass was visualized by gastroscopy.The mucous and muscular layers were closed by interrupted sutures.Pathological examination showed that the mass was a DDL.The patient did not have any dysphagia or dyspnea 2 wk postoperatively and refused any further treatment.Computed tomography and esophagoscopy did not find any recurrence at up to 20 mo postoperatively.CONCLUSION Thoracoscopy can be used to treat large esophageal masses.展开更多
Few cases of Liposarcoma of the spermatic cord have been reported in the literature. This rare tumor represents 7% of malignant tumors of the spermatic cord. We report a case of an unusual liposarcoma of the spermatic...Few cases of Liposarcoma of the spermatic cord have been reported in the literature. This rare tumor represents 7% of malignant tumors of the spermatic cord. We report a case of an unusual liposarcoma of the spermatic cord, with respect to its sclerosing variant, occurring in a healthy looking 42 years old man. A wide local excision of the tumor was performed. Adjuvant radiotherapy may sometimes be effective on local recurrence. Even though this tumor evolves slowly, a prolonged follow up is required because of the risk of late recurrence.展开更多
A 61-year-old female patient with chronic hepatitis B virus infection was diagnosed with liposarcoma in a community hospital. Fine needle aspiration biopsy confirmed the diagnosis of well-differentiated liposarcoma. A...A 61-year-old female patient with chronic hepatitis B virus infection was diagnosed with liposarcoma in a community hospital. Fine needle aspiration biopsy confirmed the diagnosis of well-differentiated liposarcoma. Abdominal computed tomographic angiography(CTA) showed that the mass adhered to and constricted the main trunk and branch of the superior mesenteric vein(SMV), especially the ileocolic vein, and collateral circulation was observed during the vascular reconstruction scan. The abdominal liposarcoma was resected. Because of the collateral circulation, devascularization of the SMV was attempted, and we resected the eroded SMV. The condition of the blood vessels was evaluated 20 d after surgery using CTA, which showed that the SMV had disappeared. Significant improvements in SMV collateral circulation and the inferior mesenteric vein were observed after vascular reconstruction. The patient had an uneventful postoperative course except for transient gastroplegia. Twenty months after surgery, the patient had a recurrence of liposarcoma. She underwent tumor resection to remove the distal small intestine and right hemicolon. We learned that(1) direct devascularization of the main SMV trunkwithout a vein graft is possible. The presence of collateralcirculation can increase the success rate of patientsundergoing radical surgery and prevent the occurrence ofserious postoperative complications. In addition,(2) thiscase demonstrated the clinical value of 3 D reconstruction.展开更多
Primary cardiac liposarcoma is exceedingly rare and its metastatic potential varies based on the actual tumor subclass. Intestinal intussusception is also an uncommon cause of abdominal pain and bowel obstruction in a...Primary cardiac liposarcoma is exceedingly rare and its metastatic potential varies based on the actual tumor subclass. Intestinal intussusception is also an uncommon cause of abdominal pain and bowel obstruction in adults and it usually generates at a malignant lead point in this age group. We report a case of a primary cardiac dedifferentiated liposarcoma in a pregnant woman causing small bowel seeding leading to bowel intussusception.展开更多
Sarcoidosis is a systemic inflammatory condition in which noncaseating epithelioid cell granulomas appear within one or several body sites. Sarcoid reaction(also referred to as sarcoidal or sarcoid-like reaction) occu...Sarcoidosis is a systemic inflammatory condition in which noncaseating epithelioid cell granulomas appear within one or several body sites. Sarcoid reaction(also referred to as sarcoidal or sarcoid-like reaction) occurs in patients who do not fulfill the diagnostic criteria for systemic sarcoidosis but present with similar clinical and histological features. As sarcoma-associated sarcoid reactions are rare, we describe the features of sarcoid reaction that developed in a man with liposarcoma and summarize reports of other oncology patients with sarcoma-associated sarcoid reactions. A 68-yearold man with retroperitoneal liposarcoma presented for evaluation of erythematous dermal plaques on his left leg. Microscopic examination of a tissue specimen revealed multiple epithelioid granulomas in the superficial and mid-reticular dermis. Correlation of the clinical presentation and histopathologic findings established a diagnosis of liposarcoma-associated cutaneous sarcoid reaction. Sarcoid reactions have been described in only seven individuals with sarcoma, including two patients with leiomyosarcoma and one patient with either carcinosarcoma, Kaposi sarcoma, liposarcoma, malignant peripheral nerve sheath tumor, rhabdosarcoma, or synovial sarcoma. Sarcoidal granulomas most commonly develop within the locoregional draining lymph nodes. Sarcoid reactions may also affect other organs, such as the lungs, skin, and spleen.展开更多
文摘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. .
基金Supported by National Natural Science Foundation of China(No.82000862,No.82201214)Shaanxi Key Research and Development Program(No.2021SF-156).
文摘AIM:To explore the clinical and pathological characteristics of thirteen patients with orbital liposarcoma.METHODS:The clinical history data of thirteen patients diagnosed as orbital liposarcoma at Beijing Tongren Hospital,from 2006 to 2021 were collected and analyzed.The data includes age,gender,affected orbital side,course of disease,status of disease(primary or recurrent),clinical manifestations,preoperative visual acuity,operative treatment,the relations between liposarcoma and surrounding tissue,longest diameter of liposarcoma,histological subtype,immunohistochemical indicators,follow-up treatment and prognosis.RESULTS:The initial symptoms are diverse.Proptosis is the most frequent chief complaint and the others included vision loss,epiphora,diplopia,and eyelid palpable mass.Results of imaging examination[computed tomography(CT)or magnetic resonance imaging(MRI)]showed orbital mass.In terms of treatment,10 patients received tumor resection,and the mean longest diameter of the tumor was 3.39±1.36 cm.The other 3 patients had optic nerve invaded,so they received orbital exenteration.Pathological examination results confirmed the diagnose of liposarcoma for 13 patients.Six patients displayed as myxoid type,and three patients in each type of dedifferentiated and welldifferentiated type.One patient was verified as pleomorphic,which was a rare type of liposarcoma.All of the patients showed Vimentin positive,and most showed CD34 and S-100 positive.Besides,four patients showed smooth muscle actin positive.All thirteen patients were alive.CONCLUSION:Orbital liposarcoma is a rare disease and it has no specific clinical manifestation.The diagnosis of liposarcoma should be considered when proptosis and orbital mass occurred in orbit.It is recommended to perform pathological examination to achieve early detection and early treatment.
文摘BACKGROUND Dedifferentiated liposarcoma(DDLS)has a worse prognosis and occurs most commonly in the retroperitoneal region and rarely in the intraperitoneal region.Histological diagnosis was revolutionized by the combined contributions of histoimmuno-chemistry and molecular biology.Aside from surgery,there is no consensus on the optimal treatment for this chemoresistant cancer.CASE SUMMARY A thirty-year-old black female presented with a large painful abdominal mass occupying nearly the entire abdomen and progressive weight loss was admitted for surgery.Abdominal computed tomography showed a large heterogeneous mass of the mesentery that was sized 18 cm×16 cm in size and had heterogeneous contrast enhancement.During laparotomy,en bloc excision of the large and multilobulated gastrocolic ligament mass was performed.The initial postoperative histopathological diagnosis was undifferentiated sarcoma.Finally,the results of immunohistochemistry and molecular biology allowed us to confirm the diagnosis of DDLS.The tumour followed an aggressive evolution with diffuse metastasis,causing the death of the patient less than 5 mo after the operation.CONCLUSION Dedifferentiated liposarcomas are rare tumours that typically originate in the retroperitoneum but may arise in unexpected locations.
文摘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 </span><span style="font-family:Verdana;">mediastinal tumors. To date, less than 150 cases have been reported in the</span><span style="font-family:Verdana;"> 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 </span><span style="font-family:Verdana;">this report, our group is documenting the presentation, management, and</span><span style="font-family:Verdana;"> 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.
文摘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.
文摘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.
文摘<span style="font-size:12px;">Thymoliposarcoma is an exceedingly rare tumor of thymus with a very few cases reported till date. </span>This case study presents a 45-year male with rare type of thymoma. <a name="_Hlk35733991"></a><span>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.</span><a name="_Hlk35738313"></a><span> 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.</span><a name="_Hlk40534514"></a><span> Postoperative follow-up CECT of thorax revealed no evidence of residual mass in the pre-vascular compartment. The patient is disease-free an</span><span><span>d</span></span><span><span> asymptomatic after 6-month and he is under routine follow-up under Radiotherapy department</span></span><span style="font-family:;" "=""> <a name="_Hlk41419004"></a><span>since he received 30 Gy of postoperative radiotherapy (PORT).</span></span>
文摘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.
文摘AIM: To report the abnormal type of the orbital liposarcoma-dedifferentiated subtype in a patient. METHODS: A case report. RESULTS: A 23 years old Chinese woman with a recurrence of right-sided proptosis was evaluated. Ocular examination revealed proptosis of the right eye with chemosis, hyperemia and limitation of eye movements. Magnetic resonance imaging scanning showed an irregular shaped tumor in the right orbit. The tumor resection was done with a clinical diagnosis of malignant tumor. Histopathological findings revealed the diagnosis of dedifferentiated liposarcoma. CONCLUSION: The rare occurrence of this tumour should be kept in mind while dealing with orbital tumours.
文摘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.
文摘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.
基金Supported by Beijing Municipal Science and Technology Commission,No.Z161100000116045National Natural Science Foundation of China,No.81772642
文摘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.
基金Supported by the Liaoning Provincial Department of Education Science Research Project,No.L2014299NSFC Molecular mechanism of aberrant expression of JDP2 and the regulation by JDP2 of TGF-betainduced epithelial to mesenchymal transition in human pancreatic carcinoma,No.81572360(2016.1-2019.12)
文摘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.
文摘BACKGROUND Pancreatic lipomas are thought to be very rare.Lipomas are usually easy to identify on imaging,particularly via computed tomography (CT).But sometimes it’s quite difficult to distinguish a lipoma from a well-liposarcoma without histologic result.CASE SUMMARY Here,we present a case of pancreatic lipoma in a 59-year-old female.She was asymptomatic and had no medical history of note.CT and magnetic resonance imaging revealed a mass like well-differentiated liposarcoma in the pancreatic head,positron emission tomography/CT showed a low fluorodeoxyglucose uptake and laboratory tests revealed elevated transaminase and carbohydrate antigen-199 levels.Finally,the patient underwent a pancreaticoduodenectomy.Histologically,mature adipocytes were noted in the bulk of the tumor.Accordingly,the pathologic diagnosis of the pancreatic neoplasm was lipoma.To our knowledge,this case is the first example of a suspected well-differentiated liposarcoma that was actually a pancreatic lipoma.We also highlight the radiological features distinguishing a pancreatic lipoma from a pancreatic liposarcoma and briefly review the literature.CONCLUSION Pancreatic lipomas show no obvious gender bias and most commonly occur in the head of the pancreas,of which the maximum diameters are often less than 5 cm,and small,asymptomatic non-compressed lipomas require follow-up only.Surgical excision should be considered when the tumor has compressed important tissues or is difficult to distinguish from a liposarcoma,the choice of surgery depends on the intraoperative presentation.
文摘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.
文摘BACKGROUND Esophageal liposarcoma is a rare malignant tumor and an esophageal dedifferentiated liposarcoma(DDL)is extremely rare.There are no reports on the treatment of DDL by thoracoscopic surgery.CASE SUMMARY A 38-year-old woman presented with dysphagia and dyspnea.Imaging examination showed a large mass in the posterior mediastinum.The patient also developed respiratory failure and it was unclear whether this was caused by a mass from inside or outside the esophagus.We decided to perform thoracoscopic exploration to relieve the obstruction caused by tracheal compression.The upper segment of the esophagus was split longitudinally,and most of the mass could be removed from the esophageal lumen to the thoracic cavity.The pedicle was excised by linear cutting closers under mirrors.Little residual mass was visualized by gastroscopy.The mucous and muscular layers were closed by interrupted sutures.Pathological examination showed that the mass was a DDL.The patient did not have any dysphagia or dyspnea 2 wk postoperatively and refused any further treatment.Computed tomography and esophagoscopy did not find any recurrence at up to 20 mo postoperatively.CONCLUSION Thoracoscopy can be used to treat large esophageal masses.
文摘Few cases of Liposarcoma of the spermatic cord have been reported in the literature. This rare tumor represents 7% of malignant tumors of the spermatic cord. We report a case of an unusual liposarcoma of the spermatic cord, with respect to its sclerosing variant, occurring in a healthy looking 42 years old man. A wide local excision of the tumor was performed. Adjuvant radiotherapy may sometimes be effective on local recurrence. Even though this tumor evolves slowly, a prolonged follow up is required because of the risk of late recurrence.
基金Supported by the National Natural Science Foundation of China,No.81773128 and No.81472247Project of provincial key basic Research,No.2017ZDJC-08 and No.2016SF-204Clinical Research Project of The First Affiliated Hospital of Xi’an Jiaotong University,No.XJTU1AF-CRF-2015-003 and No.XJTU1AF-CRF-2015-011
文摘A 61-year-old female patient with chronic hepatitis B virus infection was diagnosed with liposarcoma in a community hospital. Fine needle aspiration biopsy confirmed the diagnosis of well-differentiated liposarcoma. Abdominal computed tomographic angiography(CTA) showed that the mass adhered to and constricted the main trunk and branch of the superior mesenteric vein(SMV), especially the ileocolic vein, and collateral circulation was observed during the vascular reconstruction scan. The abdominal liposarcoma was resected. Because of the collateral circulation, devascularization of the SMV was attempted, and we resected the eroded SMV. The condition of the blood vessels was evaluated 20 d after surgery using CTA, which showed that the SMV had disappeared. Significant improvements in SMV collateral circulation and the inferior mesenteric vein were observed after vascular reconstruction. The patient had an uneventful postoperative course except for transient gastroplegia. Twenty months after surgery, the patient had a recurrence of liposarcoma. She underwent tumor resection to remove the distal small intestine and right hemicolon. We learned that(1) direct devascularization of the main SMV trunkwithout a vein graft is possible. The presence of collateralcirculation can increase the success rate of patientsundergoing radical surgery and prevent the occurrence ofserious postoperative complications. In addition,(2) thiscase demonstrated the clinical value of 3 D reconstruction.
文摘Primary cardiac liposarcoma is exceedingly rare and its metastatic potential varies based on the actual tumor subclass. Intestinal intussusception is also an uncommon cause of abdominal pain and bowel obstruction in adults and it usually generates at a malignant lead point in this age group. We report a case of a primary cardiac dedifferentiated liposarcoma in a pregnant woman causing small bowel seeding leading to bowel intussusception.
文摘Sarcoidosis is a systemic inflammatory condition in which noncaseating epithelioid cell granulomas appear within one or several body sites. Sarcoid reaction(also referred to as sarcoidal or sarcoid-like reaction) occurs in patients who do not fulfill the diagnostic criteria for systemic sarcoidosis but present with similar clinical and histological features. As sarcoma-associated sarcoid reactions are rare, we describe the features of sarcoid reaction that developed in a man with liposarcoma and summarize reports of other oncology patients with sarcoma-associated sarcoid reactions. A 68-yearold man with retroperitoneal liposarcoma presented for evaluation of erythematous dermal plaques on his left leg. Microscopic examination of a tissue specimen revealed multiple epithelioid granulomas in the superficial and mid-reticular dermis. Correlation of the clinical presentation and histopathologic findings established a diagnosis of liposarcoma-associated cutaneous sarcoid reaction. Sarcoid reactions have been described in only seven individuals with sarcoma, including two patients with leiomyosarcoma and one patient with either carcinosarcoma, Kaposi sarcoma, liposarcoma, malignant peripheral nerve sheath tumor, rhabdosarcoma, or synovial sarcoma. Sarcoidal granulomas most commonly develop within the locoregional draining lymph nodes. Sarcoid reactions may also affect other organs, such as the lungs, skin, and spleen.
