Soft tissue sarcomas are a group of rare tumors that together make up only 1 percent of all adult cancers. Mar 17, 2019 liposarcoma mixoide pdf liposarcoma is a rare cancer of connective tissues that resemble fat cells under a microscope. Since liposarcoma is known to be malignant, recurrence after resection is always a concern. They characteristically displace, rather than infiltrate adjacent. It forms one of the spectrum of fat containing retroperitoneal masses, which range from benign to frankly and aggressively malignant lesions. A 51year old man was admitted to our department because a retroperitoneal mass was seen on abdominal computed tomography at another hospital. Buecker, md also available in chinese, french, italian, japanese, portuguese and spanish. Retroperitoneal liposarcoma rls is a rare, biologically heterogeneous tumor that present considerable challenges due to its size and deep location. Pleomorphic liposarcoma is the rarest but sometimes is a very aggressive disease type. Retroperitoneal liposarcoma universitaetsspital zuerich. Retroperitoneal liposarcoma is listed as a rare disease by the office of rare diseases ord of the national institutes of health nih.
Fighting retroperitoneal sarcoma can be like fighting a rare and giant beast. We present the case of a 24yearold male who was diagnosed with. Liposarcoma mixoide genetic and rare diseases information. The threeyear overall survival rate after complete resection in the liposarcoma group was 50%3. A rare, slow growing tumor that develops in the retroperitoneal cavity of the abdomen and tends to displace organs rather than destroy. Dedifferentiated liposarcoma of the retroperitoneum.
We describe the case of a 44yearold female with a retroperitoneal myxoid liposarcoma of 22. The terms atypical lipoma and atypical intramuscular lipoma were introduced specifically to describe welldifferentiated liposarcomas occurring in the extremities, because of their better prognosis in comparison with their retroperitoneal counterparts. New author database being installed, click here for details. Surgery was 8 or 9 hours during which i also had a bowel resection, a left ureter resection, and a left ventral hernia repaired. More often than not, patients report late to the hospital due to the slow progress and few late symptoms. Primary dedifferentiated liposarcoma of the retroperitoneum. Soft tissue sarcomas represent less than 1% of all human neoplasms. My surgeon is at the hospital of the university of pennsylvania and is great. Liposarcoma is a malignancy of fat cells see pediatric liposarcoma and liposarcoma imaging. A healthy, 53yearold man presented with a small, soft, nontender mass of his right upper arm that progressively grew larger and more firm during the 6 weeks before evaluation figure 1.
Mar 24, 2016 multiple mr images demonstrate a large mass arising in the right aspect of the retroperitoneum, in roughly the vicinity of the ivc and right adrenal gland. Subtype specific prognostic nomogram for patients with primary liposarcoma of the retroperitoneum, extremity, or trunk. In the retroperitoneum, the two most common subtypes are. Recent advances in the management of liposarcoma ncbi. Comparison between retroperitoneal leiomyosarcoma and. Onethird of malignant tumors that arise in the retroperitoneum are sarcomas and liposarcoma is the most common retroperitoneal sarcoma. A clinicopatological analysys of 155 cases with a proposal for an expanded definition of dedifferentiation. Histologic subtype and margin of resection predict pattern of recurrence and survival for retroperitoneal liposarcoma.
Retroperitoneal liposarcoma university of toronto tspace. The dedifferentiated component of ddls shows an lmslike morphology in some. Retroperitoneal liposarcoma genetic and rare diseases. Retroperitoneal liposarcoma is a subtype of liposarcoma, and is a malignant tumor of mesenchymal origin that may arise in any fatcontaining region of the body. Retroperitoneal liposarcoma symptoms, diagnosis, treatments. Retroperitoneal liposarcomas are arguably much more challenging to treat from a surgical standpoint than are extremity liposarcomas. Giant dedifferentiated liposarcoma of the retroperitoneum. Giant retroperitoneal liposarcoma europe pmc article. Liposarcoma retroperitoneal gigante cirugia espanola elsevier. Liposarcoma is the most frequent histopathological variety of the retroperitoneum, surgery is the gold standard for treatment. Jun 17, 2019 liposarcoma mixoide pdf liposarcoma is a rare cancer of connective tissues that resemble fat cells under a microscope. Clinical features, evaluation, and treatment of retroperitoneal soft tissue sarcoma view in chinese cell infiltrate. Mar 18, 2020 liposarcoma is a malignancy of fat cells see pediatric liposarcoma and liposarcoma imaging.
A dedifferentiated liposarcoma of the retroperitoneum is an extremely rare tumor. Introduction liposarcoma is a malignant soft tissue tumour, which represents less than 0. Jan 09, 2019 if it forms in the retroperitoneal space, the tumor may not cause any symptoms for a long period of time, until it grows to a large size and compresses the adjacent organs in the abdomen. Giant retroperitoneal liposarcoma pubmed central pmc. Retroperitoneal liposarcomas represent a unique situation and require a more aggressive. This means that retroperitoneal liposarcoma, or a subtype of retroperitoneal liposarcoma, affects less than 200,000 people in the us population. Giant retroperitoneal liposarcoma world journal of surgical. Liposarcoma mixoide pdf liposarcoma is a rare cancer of connective tissues that resemble fat cells under a microscope. And a dedifferentiated liposarcoma is a highgrade tumor that occurs when a lowergrade tumor changes and creates new highgrade cells. The terms atypical lipoma and atypical intramuscular lipoma were introduced specifically to describe welldifferentiated. May 08, 2015 retroperitoneal liposarcoma is a rare tumor with an incidence of 2. Excision of the tumor en bloc with right radical nephrectomy and lymphadenectomy was. We present the case of a 24yearold male who was diagnosed with a giant. A 51year old man was admitted to our department because a retroperitoneal mass was seen on abdominal.
Early diagnosis is difficult as there is an absence of specific clinical presentations. If it forms in the retroperitoneal space, the tumor may not cause any symptoms for a long period of time, until it grows to a large size and compresses the adjacent organs in the abdomen. Liposarcoma mixoide retroperitoneal con metaplasia osea cirugia. Liposarcoma mixoide retroperitoneal scielo cuba infomed. Liposarcoma is a malignant tumor that have an embryologic origin from mesodermal tissuedepending on fatty tissue. Pubmed is a searchable database of medical literature and lists journal articles that discuss retroperitoneal liposarcoma.
Click on the link to view a sample search on this topic. Although these tumors can occur anywhere in the body, about 20 percent 0. A case report m sandra jacob, shirali patel, harvey sasken, yomayra perez, valerie katz, mark ingram abstract introduction. Histological analysis revealed a mixedtype liposarcoma consisting of welldifferentiated, myxoid, and pleomorphic components, and lymph node involvement. It is one of the most common primary retroperitoneal neoplasms 2. A case report, abstract a 66yearold man, with abdominal distention, was referred to our department for the treatment of an.
It is important to distinguish between leiomyosarcoma lms and dedifferentiated liposarcoma ddls in the retroperitoneum. Lipossarcoma causas, sintomas, diagnostico e tratamento. It is thought that the high grade nonlipomatous elements. Retroperitoneal sarcomas are rare neoplasms that account for only 1%2% of all solid tumors and liposarcomas represent the most frequent histological type. Retroperitoneal liposarcoma is a rare tumor with an incidence of 2. It has been reported that 20% of the tumors are 10 cm at the time of diagnosis. Onethird of malignant tumors that arise in the retroperitoneum are sarcomas and liposarcoma is the most common retroperitoneal. A 67yearold female was referred to the surgery clinic by the gynecology service for symptomatic cholelithiasis. Tumor size was 10 x 10 x 9 cm, weight of tumor was just under 2 lbs. This is one of the reasons why retroperitoneal tumors remain undetected during their early development stage. We present the case of a 24yearold male who was diagnosed with a giant retroperitoneal liposarcoma.
We report an interesting case of a 67yearold female presented with symptomatic cholelithiasis and was found to have an occult retroperitoneal sarcoma on work up. Apr 03, 2020 a healthy, 53yearold man presented with a small, soft, nontender mass of his right upper arm that progressively grew larger and more firm during the 6 weeks before evaluation figure 1. We decided to perform surgery after no benefit was received with. A case of myxoid liposarcoma of the retroperitoneum. Oct 23, 2009 my husband had a retroperitoneal liposarcoma 6 month ago it was completed resected and his left kidney was removed too, but the doctors told us that everything should be ok and he didnt need anything else no radiotherapy or chemo. In adults, it is the most common soft tissue sarcoma. Dedifferentiated liposarcoma and pleomorphic liposarcoma in particular appear to be more sensitive to eribulin than to trabectedin, although surgical resection of retroperitoneal sarcoma welldifferentiated and dedifferentiated liposarcoma, whereas distant metastasis is more common in leiomyosarcoma. My husband 32 years old has diagnosed with retroperitoneal liposarcoma on november 2015. Patients with welldifferentiated liposarcoma can survive for decades, but recurrence is a problem. Approximately 20% of liposarcomas arise in the retroperitoneum. Retroperitoneal liposarcoma radiology reference article. Multiple mr images demonstrate a large mass arising in the right aspect of the retroperitoneum, in roughly the vicinity of the ivc and right adrenal gland. Without the administration of gadolinium contrast material, the tumor may even mimic a cystic tumor.
Retroperitoneal liposarcoma managed by laparoscopy nomura int surg 20. They represent the lowest grade lesions in the spectrum of liposarcoma. Although myxoid liposarcoma is a subtype of liposarcoma, it may be difficult to establish the correct diagnosis with magnetic resonance mr imaging due to the lack of fat signal intensity. The patient received palliative treatment due to nonresectability on the basis of chemotherapy. Volume 69, issue 6, novemberdecember 2017, pages 498501. Dedifferentiated liposarcomas represent heterogeneous tumors with lipomatous and nonlipomatous elements starkly juxtaposed. The term liposarcoma refers to a spectrum of neoplastic processes ranging from lesions that are essentially benign to those that are malignant, more aggressive, and likely to recur andor metastasize spread.
Giant retroperitoneal liposarcoma world journal of. The present case study reports a patient diagnosed with retroperitoneal. Mar 31, 2010 a dedifferentiated liposarcoma of the retroperitoneum is an extremely rare tumor. Excision of the tumor en bloc with right radical nephrectomy and lymphadenectomy was performed. Thus, the tumor is known to grow to enormous sizes. Now, in his first control exams we discovered that he has a recurrence at the same place. A unique dedifferentiated tumor of the retroperitoneum. Laparoscopic resection of a retroperitoneal liposarcoma. Liposarcoma is the most frequent histological type of retroperitoneal sarcoma, corresponding to 41% of these tumors 4,5. Whenever a multiorganic resection is feasible, a complete macroscopic resection should be contemplated. Liposarcoma normally appears as a slowly enlarging, painless, nonulcerated submucosal mass in a middleaged person, but some lesions grow rapidly and become ulcerated early. It contains both fatty and soft tissue elements and is often sizeable before presentation. And a dedifferentiated liposarcoma is a highgrade tumor that occurs when a lowergrade tumor. A 71yearold woman presented with a giant retroperitoneal liposarcoma with lymph node invasion.