Liposarcoma is a rare cancer of connective tissues that resemble fat cells under a microscope. It accounts for up to 18% of all soft tissue sarcomas. Liposarcoma. Introducción: El liposarcoma mixoide es una neoplasia maligna del mesénquima con una presentación muy rara en esófago. Los sarcomas representan 1% de. Liposarcoma is the most common soft tissue sarcoma. With its various subtypes, the natural history of this disease can vary significantly from a.
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Main Menu Learn About Sarcoma. The Sarcoma Universe 3. Clinical Trials for Sarcoma. What can one determined family do to make a difference? These photos come from around the world. Liposarcoma is one mxioide more than 50 kinds of sarcoma. Each kind is rare, yet all together sarcomas affect hundreds of thousands of people around the world.
Mixodie our Comprehensive Guide for the Newly Diagnosed. Liposarcoma is a rare cancer of connective tissues that resemble fat cells under a mixoise. Liposarcoma can occur in almost any part of the body, but more than half of liposarcoma cases involve the thigh, and up to a third involve the abdominal cavity. Liposarcoma tends to affects adults between the ages liposzrcoma 40 and When it does occur in children, it is usually during the teenage years. There are four types of liposarcoma, each with its own unique characteristics and behaviors.
Scientists do not yet know the cause of liposarcoma. Although patients sometimes notice a tumor after they experience an injury, liposarcoma is not known to be caused by trauma. It is also not known to lipsarcoma from benign lipomas, which are harmless lumps of fat. Most people with liposadcoma do not feel sick. They might notice a lump which can be soft or firm to the touch that is usually painless and slow growing. Unfortunately, tumors liposarcoms the abdomen can grow to be quite large before they are found.
A physical exam is the first step in the liposarcoma diagnosis process. Lumps that are 5cm or larger and deep-seated, firm and fixed to underlying structures are usually considered suspicious. Imaging tests are the next step and often include an X-ray and an MRI.
An experienced radiologist might suspect the diagnosis based on the results of these tests. There are two main types of biopsy: Therefore, it is essential that the biopsy is planned by a surgeon or radiologist experienced with sarcomas.
Surgery is the treatment for primary liposarcomas that have not yet spread to other organs. In most cases, a surgeon will remove mixoidr tumor, along with a wide margin of healthy tissue around kixoide tumor, with the goal of leaving the area free of disease and preventing the tumor from returning. Most tumors of the arms and legs can be successfully removed while sparing the involved limb.
Complete surgical removal of tumors within the abdomen is difficult, in part because of the difficulty in getting clear margins of normal tissue. These results vary depending on the subtype of sarcoma that is involved. Radiation therapy may be used before, during or after surgery to kill tumor cells and reduce the chance of the tumor returning in the same location.
Radiation therapy that is given before surgery may be more beneficial, but it can also make it more difficult for surgical wounds to heal. The role of chemotherapy in the treatment of liposarcoma is not clearly defined, but it may be recommended in certain situations where patients are at high risk of recurrence or already have widespread disease.
Prognosis statistics are based on the study of groups of liposarcoma ,ixoide. These statistics cannot predict the future of an individual liposarcomq, but they can be useful in considering the most appropriate treatment and follow-up for a patient.
Liposarcoma prognosis is reported based on disease subtype. Five-year disease specific survival rates chances of not dying from cancer-related causes are as follows: However, the site of disease has a strong effect on prognosis of well-differentiated and de-differentiated liposarcoma. If these liposarcomas arise within the abdomen, complete eradication of the tumor in the long term is less common.
This is probably because it is difficult for the surgeon to completely remove tumors in this location. It usually includes a physical liopsarcoma and imaging studies.
Decisions regarding the treatment and aftercare of lippsarcoma are guided by the known characteristics and behavior patterns of the various subtypes. While many of the principles governing the evaluation and management of other soft tissue sarcomas certainly apply to liposarcoma, there are many features unique to it that deserve special consideration. Management of these tumors requires a multidisciplinary team approach, and should be rendered in centers experienced in the many facets of care of sarcoma patients.
In relation to other types of cancer, soft tissue sarcomas are relatively rare.
Liposarcoma – Wikipedia
Liposarcoma itself constitutes about 9. Liposarcoma is a tumor derived from primitive cells that undergo adipose differentiation. It is largely a disease of adults, its incidence peaking between liposarcima ages of 40 and 60 years, and it shows a slight predominance toward men Enzinger When liposarcomas do occur in the pediatric population, they tend to misoide in the second decade of life Coffin Its presentation in this location is most commonly that of a slow growing, painless mass.
Often these tumors are first noticed after the patient has sustained a minor trauma to the area. Liposarcoma was originally described by R. InArthur Purdy Stout wrote “surely, one of the most bizarre and fantastic chapters in the story of oncology is furnished by the tumors of fat-forming cells.
The strange way in which they grow, their astounding size…and many other peculiar features Stout, Annals of Surgery; 1: One unique feature of liposarcoma is its tendency to occur in visceral spaces, particularly that of the retroperitoneum.
The presentation of liposarcoma in this scenario may be quite different. While a mass may be appreciated, it tends to be found much later, since the retroperitoneal space can accommodate a much larger change in volume than can the thigh, for instance.
Also, obstructive urinary and bowel symptoms may predominate as the mass impinges on these structures. The management of liposarcoma in this location can be particularly difficult. Mesoderm comprises the middle germ cell muxoide and gives rise to cells that comprise cardiac and skeletal muscle, hematopoitic cells and cells producing bone, cartilage and connective tissue throughout the body.
In addition this germ cell layer ultimately gives rise to the cells that make up the genitourinary system. The term “ALT” for atypical lipomatous tumor was introduced in Fibrous septae are often present. However, it is unequivocally established that DD constitutes a high grade lesion with increased cellularity that is prone to disseminate and is associated with a much worse prognosis than WD which has minimal metastatic potential.
Moreover, grade is frequently not included in multivariate liposarcoma models that include histologic subtype. It has no specific delineating anatomical structures. Organs are retroperitoneal if they only have peritoneum on their anterior side.
Although different liposarcoma histologic subtypes can be observed in soft tissue sarcomas that arise in the retroperitoneum, histologic subtype per se is usually not incorporated into retroperitoneal disease management strategies. Complete surgical resection is a difficult challenge as these large tumors are often surrounded by a thin fibrous capsule which is frequently indistinguishable from surrounding retroperitoneal adipose tissue.
Critical structures in the retroperitoneum can also pose a challenge to resectability. Accordingly, it is often difficult to obtain a margin of normal tissue around the tumor. The importance of lipsarcoma macroscopic resection is well established and resection of contiguous organs in the hope of decreasing the rate of local failure is a common practice.
However, evidence that a more extensive resection impacts survival is very limited. Among the factors that significantly influence clinical outcome, histologic subtype is recognized as an independent predictor of local recurrence LRdistant metastasis DM and disease specific survival DSS.
Tumors without kixoide component of dedifferentiation, referred to as either atypical lipomatous tumors or well-differentiated liposarcoma WDare associated with a less aggressive clinical course.
WD tumors are characterized by repetitive local recurrence without the potential for metastasis. In contrast, dedifferentiated tumors DD also recur at a high rate, but have the potential to metastasize and represent an aggressive clinical phenotype. Accordingly, at MDACC we recognize these tumors as distinct with differing tumor biology and clinical outcome.
Utilizing this general strategy for patients with WD tumors has resulted in less aggressive surgery without liposarcomx addition of chemotherapy or radiotherapy.
For lipisarcoma with retroperitoneal liposarcomas with evidence of dedifferentiation, we adopt a more involved treatment approach that begins with neoadjuvant chemo-radiotherapy followed by aggressive surgical resection.
In this experience, completeness of resection significantly impacted clinical outcome: In this series and others from the MSKCC group an aggressive surgical approach was applied based on the demonstrated prognostic significance of a complete macroscopic resection.
Unlike extremity liposarcoma where tumor size is known to significantly affect outcome the size of retroperitoneal liposarcomas is typically much larger; in this series lipisarcoma mean tumor maximum dimension was 26 cm. As an indication of aggressive surgical resection, over two-thirds of patients in this series underwent resection of contiguous organs, including nephrectomy.
On multivariate analysis of prognostic factors associated with local recurrence, distant metastasis, and disease-specific survival, only the presence of contiguous organ resection and the DD histologic subtype were significant adverse factors.
It is possible that the patients receiving contiguous organ resection simply had more extensive tumor burden rather than worse biology per se. Underlying tumor biology, rather than extensive surgical resection appears to be more important determinant of clinical outcome. We hypothesize that the histologic subtype of retroperitoneal sarcomas may serve as a surrogate for tumor biology and mixoids affect clinical outcome more than extent of initial resection if a gross total excision is performed.
The treatment ,ixoide for a retroperitoneal liposarcoma patient presenting to the MDACC Sarcoma Center is formulated upon multidisciplinary consensus review prior to initiation of therapy. This approach is in contrast to traditional tumor board review of patients who typically undergo resection and are then considered by the multidisciplinary review panel to determine the utility of adjuvant therapy based on final review of the resected specimen pathology.
Our practice is to utilize validated diagnostic imaging criteria and selective image guided biopsy of suspected foci of DD to make a preoperative diagnosis of WD versus DD in patients presenting with retroperitoneal mmixoide. WD patients generally undergo less aggressive surgery avoiding resection of contiguous organs if possible, while patients with DD are treated with neoadjuvant chemotherapy and radiation therapy followed by aggressive surgical resection.
Using this approach, patients may realize benefits from neoadjuvant therapies that would otherwise not be demonstrable were resection performed as the initial treatment strategy. The major theoretical benefits to neoadjuvant chemotherapy are to identify patients who respond to the systemic therapy as measured by the pathologic response, and to initiate treatment of possible occult metastatic disease at the earliest possible time after diagnosis.
These potential advantages are offset by several disadvantages of neoadjuvant therapy, namely that definitive resection is delayed and wound healing may be compromised.
What is liposarcoma?
However, there is evidence to suggest that response to front line sarcoma chemotherapeutic agents may be dependent on histologic liposarcoma subtype, with DD having greater response rates than WD. We have recently reviewed our experience utilizing this approach to patients with retroperitoneal sarcoma, demonstrating that WD patients usually benefit from a less aggressive surgical approach that avoids unnecessarily extensive resection.
This strategy was developed based on liposarcomw anecdotal observations of the generally indolent clinical behavior of WD tumors in contrast to the metastasis prone phenotype of DD tumors.