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Liposarcoma

Updated : April 17, 2024





Background

Liposarcoma is an uncommon mesenchymal cancer that affects deep soft tissues such as the popliteal fossa, esophageal tract, and an anatomical space located behind the abdominal or peritoneal cavity (retroperitoneum). It is a tumor of lipoblasts.

The tumor subtypes determine the intensity ratio of liposarcoma at distinct body locales. Dedifferentiated liposarcoma, for example, is more usual in the retroperitoneum, whereas myxoid liposarcoma is more common in the lower appendage of the body.

In the esophageal tract, liposarcoma Is a relatively uncommon esophageal liposarcoma that is a quietly spreading tumor that affects the upper portion of the neck. Another good form’s predominant region is localized to the esophagus, with a low chance of metastasis. It has a high percentage of local refractory (10%), even up to twenty-five years after surgery.

Epidemiology

Liposarcoma accounts for fewer than a quarter of all soft tissue sarcomas in the US. Although case reports of juvenile-onset have been observed, the median age of diagnosis is fifty years old. Liposarcoma is the most frequent soft tissue sarcoma in the world, with reference to the American cancer society.

There is no other known risk factor for liposarcoma. Ethnicity or sex has no impact on this. Some investigations have revealed a modest male predominance. Esophageal tract liposarcoma is quite uncommon. Adenocarcinoma is the most prevalent type of esophageal neoplasm in the US, while squamous cell carcinomas are the most common type worldwide.

Sarcoma makes up just above 0.5 percent of all esophageal tumors. Only forty cases of esophageal liposarcoma tumor have been diagnosed among them. Esophageal liposarcoma is more common in middle-aged to adult male patients (age range thirty-eight to eighty-three years, with a mean of sixty-two years; men to female ratio = 4 to 1), including these documented instances.

Anatomy

Pathophysiology

The pathogenesis of liposarcoma can best be explained by splitting it into 3 subgroups (from the World Health Organization’s (WHO) categorization dated 2002). These contain WDLPS/DDLPS (well-differentiated liposarcoma neoplasm and dedifferentiated liposarcoma neoplasm), MLS, RCL (myxoid liposarcoma neoplasm and round cell liposarcoma neoplasm), and pleomorphic liposarcoma (PLS).

In WDLPS/DDLPS, a diagnostic big marker and ringed chromosome will be expressed, causing unregulated cell growth. Reversible displacement across chromosomes 12 and 16 happens in MCL and RCL, resulting in the union of genes DDIT3 and FUS, which then activates descending pathways such as PPARgamma2 and C/EBPalpha, boosting cell cycle progression.

The most difficult and poorly appreciated of these is pleomorphic liposarcoma. The destructive character of this cancer is point mutations in different tumor suppressor mechanisms such as p53, NF1, and RB1.

Etiology

Liposarcoma has yet to be identified. Although the American cancer society has found several obvious risk factors for soft tissue sarcomas, there are also examples when patients have no known risk factors. The genetic mutation that causes these cancers is currently being researched.

Radiation (particularly radiation therapy used to treat other malignancies), certain family cancer syndromes, lymphatic system damage/trauma, and toxic chemical exposure are all risk factors for liposarcoma, according to the American cancer society. Liposarcomas do not originate from lipomas, which are harmless.

Genetics

Prognostic Factors

Several variables determine the prognosis of liposarcoma. Histologic subtypes, tumor grade, tumor region, and surgical margin status are linked to survival. It has been reported that a well-differentiated liposarcoma has a 50% recurrence rate with no danger of distant metastasis and an excellent 5-year survival rate (75 percent to 100 percent).

Myxoid and pleomorphic liposarcomas, on the other hand, have a greater probability of recurrence (up to 80%) and a poor to intermediate survival rate (ranging from 4 to 107 months). Distal metastasis is more likely in undifferentiated liposarcoma.

A positive surgical resection margin is linked to a high rate of local recurrence and poor prognosis. Although there have been some case reports of adjuvant radiation therapy being used to reduce recidivism, the role of adjuvant chemotherapy and radiation therapy is still being debated.

Clinical History

Physical Examination

Age group

Associated comorbidity

Associated activity

Acuity of presentation

Differential Diagnoses

Laboratory Studies

Imaging Studies

Procedures

Histologic Findings

Staging

Treatment Paradigm

by Stage

by Modality

Chemotherapy

Radiation Therapy

Surgical Interventions

Hormone Therapy

Immunotherapy

Hyperthermia

Photodynamic Therapy

Stem Cell Transplant

Targeted Therapy

Palliative Care

Medication

Media Gallary

References

https://www.ncbi.nlm.nih.gov/books/NBK538265/

Liposarcoma

Updated : April 17, 2024




Liposarcoma is an uncommon mesenchymal cancer that affects deep soft tissues such as the popliteal fossa, esophageal tract, and an anatomical space located behind the abdominal or peritoneal cavity (retroperitoneum). It is a tumor of lipoblasts.

The tumor subtypes determine the intensity ratio of liposarcoma at distinct body locales. Dedifferentiated liposarcoma, for example, is more usual in the retroperitoneum, whereas myxoid liposarcoma is more common in the lower appendage of the body.

In the esophageal tract, liposarcoma Is a relatively uncommon esophageal liposarcoma that is a quietly spreading tumor that affects the upper portion of the neck. Another good form’s predominant region is localized to the esophagus, with a low chance of metastasis. It has a high percentage of local refractory (10%), even up to twenty-five years after surgery.

Liposarcoma accounts for fewer than a quarter of all soft tissue sarcomas in the US. Although case reports of juvenile-onset have been observed, the median age of diagnosis is fifty years old. Liposarcoma is the most frequent soft tissue sarcoma in the world, with reference to the American cancer society.

There is no other known risk factor for liposarcoma. Ethnicity or sex has no impact on this. Some investigations have revealed a modest male predominance. Esophageal tract liposarcoma is quite uncommon. Adenocarcinoma is the most prevalent type of esophageal neoplasm in the US, while squamous cell carcinomas are the most common type worldwide.

Sarcoma makes up just above 0.5 percent of all esophageal tumors. Only forty cases of esophageal liposarcoma tumor have been diagnosed among them. Esophageal liposarcoma is more common in middle-aged to adult male patients (age range thirty-eight to eighty-three years, with a mean of sixty-two years; men to female ratio = 4 to 1), including these documented instances.

The pathogenesis of liposarcoma can best be explained by splitting it into 3 subgroups (from the World Health Organization’s (WHO) categorization dated 2002). These contain WDLPS/DDLPS (well-differentiated liposarcoma neoplasm and dedifferentiated liposarcoma neoplasm), MLS, RCL (myxoid liposarcoma neoplasm and round cell liposarcoma neoplasm), and pleomorphic liposarcoma (PLS).

In WDLPS/DDLPS, a diagnostic big marker and ringed chromosome will be expressed, causing unregulated cell growth. Reversible displacement across chromosomes 12 and 16 happens in MCL and RCL, resulting in the union of genes DDIT3 and FUS, which then activates descending pathways such as PPARgamma2 and C/EBPalpha, boosting cell cycle progression.

The most difficult and poorly appreciated of these is pleomorphic liposarcoma. The destructive character of this cancer is point mutations in different tumor suppressor mechanisms such as p53, NF1, and RB1.

Liposarcoma has yet to be identified. Although the American cancer society has found several obvious risk factors for soft tissue sarcomas, there are also examples when patients have no known risk factors. The genetic mutation that causes these cancers is currently being researched.

Radiation (particularly radiation therapy used to treat other malignancies), certain family cancer syndromes, lymphatic system damage/trauma, and toxic chemical exposure are all risk factors for liposarcoma, according to the American cancer society. Liposarcomas do not originate from lipomas, which are harmless.

Several variables determine the prognosis of liposarcoma. Histologic subtypes, tumor grade, tumor region, and surgical margin status are linked to survival. It has been reported that a well-differentiated liposarcoma has a 50% recurrence rate with no danger of distant metastasis and an excellent 5-year survival rate (75 percent to 100 percent).

Myxoid and pleomorphic liposarcomas, on the other hand, have a greater probability of recurrence (up to 80%) and a poor to intermediate survival rate (ranging from 4 to 107 months). Distal metastasis is more likely in undifferentiated liposarcoma.

A positive surgical resection margin is linked to a high rate of local recurrence and poor prognosis. Although there have been some case reports of adjuvant radiation therapy being used to reduce recidivism, the role of adjuvant chemotherapy and radiation therapy is still being debated.

https://www.ncbi.nlm.nih.gov/books/NBK538265/