Symptoms from a retroperitoneal sarcoma are usually produced by (AIIMS Nov 2011)
|A||Bleeding into the tumor mass|
|B||Compression of adjacent tissues|
|C||Invasion of retroperitoneal organs|
|D||Metastases to retroperitoneal lymph nodes|
a. About 15% of all soft tissue sarcomas occur in the retroperitoneum.
b. Patients with von Recklinghausen's disease and Li-Fraumeni syndrome have an increased incidence of sarcoma.
c. Patients with mutations of the p53 and RB-1 genes also appear to have a predilection for the development of sarcoma.
d. Most patients with retroperitoneal sarcomas present with an asymptomatic abdominal mass, often after the primary tumor has reached a considerable size.
e. Abdominal pain is present in half of patients.
f. Symptoms related to nerve compression by the tumor, such as lower extremity paresthesia and paresis, have also been associated with retroperitoneal sarcoma.
g. CT and MRI provide important information regarding size and precise location of the primary tumor and its relationship to major vascular structures as well as the presence or absence of metastatic disease.
h. Preoperative imaging studies provide important clues to the diagnosis; hence, CT-guided core biopsy is usually reserved for lesions significantly likely to be lymphoma or germ cell tumor.
i. Lymph node metastases by sarcoma are rare (<5%);
j. The prognostic factors in retroperitoneal sarcoma include the size of the tumor and the histologic grade.
k. Rates of resectability of the primary retroperitoneal sarcoma vary widely, based on the extent of disease at presentation.
l. Local recurrence after complete resection of retroperitoneal sarcoma is common, occurring in 40% to 80% of cases.
m. Additionally, patients with high-grade sarcoma also have a higher risk for systemic disease and death than patients with low-grade sarcoma