Soft tissue sarcoma are tumors that affect individuals of any age and originate from any body part. These tumors range in clinical presentation from benign lipomas to aggressive metastatic angiosarcomas.
They can affect skeletal muscle, blood, lymphatic system, adipose tissue, peripheral nerves, and connective tissue.
Epidemiology
Soft tissue sarcomas are rare, with 4740 fatalities and 12,020 new cases reported in the United States. They estimate about 1% of all cancer incidence and 2% of cancer-related fatalities in the United States.
In terms of the extremities, tumors occur more commonly in the proximal limb, particularly the thigh accounting for 44% of occurrences. Trunk sarcomas occur more frequently than retroperitoneal and intraperitoneal sarcomas.
Anatomy
Pathophysiology
Sarcomas are connective tissue tumors; thus, they develop in bone, cartilage, fat, muscle, or vascular or hematopoietic tissues. Sarcomas are substantially less common than carcinomas.
Most develop locally and infect neighboring tissues. Most patients report a hard mass or discomfort caused by pressure on adjacent nerves and soft tissues.
Etiology
There are several factors causative factors.
Radiation
The effects of radiation are dose-dependent and often occur near the radiation field’s periphery. Disease-specific survival is shorter as compared to spontaneous alternatives. Children acquire soft tissue sarcoma after radiation at 11.8 years later on average and in a dose-dependent manner.
Carcinogens
Patients exposed to Thorotrast, a thorium-IV-based contrast in the period 1930 to 1955, developed hepatic angiosarcoma after 20 to 30 years.
Von Recklinghausen Disease
Mutations in the NF1 gene, which codes for a protein known as neurofibromin, cause the autosomal dominant disorder.
Ras oncogene signaling pathway tumor suppressor
Multiple cutaneous neurofibromas are caused by mutations in the NF1 gene.
Genetics
Prognostic Factors
Curative excision for circumscribed and early-stage lesions is possible with acceptable long-term endurance; however, recurrences are prevalent. The risk of recurrence exists even after 10 to 15 years, and patients should be monitored for a prolonged time.
Most recurrences occur within the first five years of treatment. A cure is not achievable for patients with a severe condition, and the median survival time ranges from 12 to 18 months, based on the subtype.
Chemotherapy has a minimal response rate of about 10% to 50%. The response is also affected by the grade and histological subtype.
pazopanib is indicated in patients who have received chemotherapy in the past for soft tissue sarcoma
A dose of 800 mg orally, each day, is administered on an empty stomach (1 hour before meals or 2 hours after meals)
Dose Adjustments
In case of renal impairment
All grades: No dosage adjustment is required
In case of hepatic impairment
Mild impairment (bilirubin <1.5 x upper normal limit and any serum alanine aminotransferase): No dosage adjustment necessary
Moderate impairment (bilirubin ≥1.5-3 x upper normal limit and any serum alanine aminotransferase): Reduce the dose to 200 mg orally each day
Severe impairment (bilirubin >3 x upper normal limit and any serum alanine aminotransferase): The drug is not recommended in this case
Administer dose of 1.5 mg/m² intravenously every three weeks until the disease advances
Administer through a central venous line over a period of 24 hours Hepatic impairment
Moderate hepatic impairment: 0.9 mg/ m² Renal impairment
Mild-to-moderate: dose modification not required
Severe: No study performed
Permanently discontinue
Continued adverse reactions demanding a postponement of dosing for more than 3 weeks
Adverse reactions demanding reduction in dosage after administering a 1 mg/m² dose
Future Trends
Media Gallary
References
ncbi.nlm.nih.gov/books/NBK519533/
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Soft tissue sarcoma are tumors that affect individuals of any age and originate from any body part. These tumors range in clinical presentation from benign lipomas to aggressive metastatic angiosarcomas.
They can affect skeletal muscle, blood, lymphatic system, adipose tissue, peripheral nerves, and connective tissue.
Soft tissue sarcomas are rare, with 4740 fatalities and 12,020 new cases reported in the United States. They estimate about 1% of all cancer incidence and 2% of cancer-related fatalities in the United States.
In terms of the extremities, tumors occur more commonly in the proximal limb, particularly the thigh accounting for 44% of occurrences. Trunk sarcomas occur more frequently than retroperitoneal and intraperitoneal sarcomas.
Sarcomas are connective tissue tumors; thus, they develop in bone, cartilage, fat, muscle, or vascular or hematopoietic tissues. Sarcomas are substantially less common than carcinomas.
Most develop locally and infect neighboring tissues. Most patients report a hard mass or discomfort caused by pressure on adjacent nerves and soft tissues.
There are several factors causative factors.
Radiation
The effects of radiation are dose-dependent and often occur near the radiation field’s periphery. Disease-specific survival is shorter as compared to spontaneous alternatives. Children acquire soft tissue sarcoma after radiation at 11.8 years later on average and in a dose-dependent manner.
Carcinogens
Patients exposed to Thorotrast, a thorium-IV-based contrast in the period 1930 to 1955, developed hepatic angiosarcoma after 20 to 30 years.
Von Recklinghausen Disease
Mutations in the NF1 gene, which codes for a protein known as neurofibromin, cause the autosomal dominant disorder.
Ras oncogene signaling pathway tumor suppressor
Multiple cutaneous neurofibromas are caused by mutations in the NF1 gene.
Curative excision for circumscribed and early-stage lesions is possible with acceptable long-term endurance; however, recurrences are prevalent. The risk of recurrence exists even after 10 to 15 years, and patients should be monitored for a prolonged time.
Most recurrences occur within the first five years of treatment. A cure is not achievable for patients with a severe condition, and the median survival time ranges from 12 to 18 months, based on the subtype.
Chemotherapy has a minimal response rate of about 10% to 50%. The response is also affected by the grade and histological subtype.
pazopanib is indicated in patients who have received chemotherapy in the past for soft tissue sarcoma
A dose of 800 mg orally, each day, is administered on an empty stomach (1 hour before meals or 2 hours after meals)
Dose Adjustments
In case of renal impairment
All grades: No dosage adjustment is required
In case of hepatic impairment
Mild impairment (bilirubin <1.5 x upper normal limit and any serum alanine aminotransferase): No dosage adjustment necessary
Moderate impairment (bilirubin ≥1.5-3 x upper normal limit and any serum alanine aminotransferase): Reduce the dose to 200 mg orally each day
Severe impairment (bilirubin >3 x upper normal limit and any serum alanine aminotransferase): The drug is not recommended in this case
Administer dose of 1.5 mg/m² intravenously every three weeks until the disease advances
Administer through a central venous line over a period of 24 hours Hepatic impairment
Moderate hepatic impairment: 0.9 mg/ m² Renal impairment
Mild-to-moderate: dose modification not required
Severe: No study performed
Permanently discontinue
Continued adverse reactions demanding a postponement of dosing for more than 3 weeks
Adverse reactions demanding reduction in dosage after administering a 1 mg/m² dose
ncbi.nlm.nih.gov/books/NBK519533/
medtigo
Soft tissue sarcoma
Updated :
August 9, 2023
Soft tissue sarcoma are tumors that affect individuals of any age and originate from any body part. These tumors range in clinical presentation from benign lipomas to aggressive metastatic angiosarcomas.
They can affect skeletal muscle, blood, lymphatic system, adipose tissue, peripheral nerves, and connective tissue.
Soft tissue sarcomas are rare, with 4740 fatalities and 12,020 new cases reported in the United States. They estimate about 1% of all cancer incidence and 2% of cancer-related fatalities in the United States.
In terms of the extremities, tumors occur more commonly in the proximal limb, particularly the thigh accounting for 44% of occurrences. Trunk sarcomas occur more frequently than retroperitoneal and intraperitoneal sarcomas.
Sarcomas are connective tissue tumors; thus, they develop in bone, cartilage, fat, muscle, or vascular or hematopoietic tissues. Sarcomas are substantially less common than carcinomas.
Most develop locally and infect neighboring tissues. Most patients report a hard mass or discomfort caused by pressure on adjacent nerves and soft tissues.
There are several factors causative factors.
Radiation
The effects of radiation are dose-dependent and often occur near the radiation field’s periphery. Disease-specific survival is shorter as compared to spontaneous alternatives. Children acquire soft tissue sarcoma after radiation at 11.8 years later on average and in a dose-dependent manner.
Carcinogens
Patients exposed to Thorotrast, a thorium-IV-based contrast in the period 1930 to 1955, developed hepatic angiosarcoma after 20 to 30 years.
Von Recklinghausen Disease
Mutations in the NF1 gene, which codes for a protein known as neurofibromin, cause the autosomal dominant disorder.
Ras oncogene signaling pathway tumor suppressor
Multiple cutaneous neurofibromas are caused by mutations in the NF1 gene.
Curative excision for circumscribed and early-stage lesions is possible with acceptable long-term endurance; however, recurrences are prevalent. The risk of recurrence exists even after 10 to 15 years, and patients should be monitored for a prolonged time.
Most recurrences occur within the first five years of treatment. A cure is not achievable for patients with a severe condition, and the median survival time ranges from 12 to 18 months, based on the subtype.
Chemotherapy has a minimal response rate of about 10% to 50%. The response is also affected by the grade and histological subtype.
ncbi.nlm.nih.gov/books/NBK519533/
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