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Diagnostic work up
• Computed tomography (CT):
– Large lesions with fatty components causing mass effect
frequently represent liposarcomas.
– Teratomas often have fat, fluid, and calcified components.
– Paraspinal locations point to nerve sheath tumors or
neurogenic derivation .
• Magnetic resonance imaging (MRI) may demonstrate
additional imaging details to further classify the
lesion.
• CT-guided needle biopsy may be performed as the
initial means of obtaining tissue for histologic
diagnosis
RETROPERITONEAL SARCOMA
• Second most common malignant RPT.
• Arise from: nonepithelial, extraskeletal tissues:
(fat, muscle, nerve and nerve sheath, blood
vessels, other connective tissues).
• Incidence: 9,220 cases per year in the United
States.
• Common types:
– Leiomyosarcoma
– Liposarcoma
– Fibrosarcoma
• Primary treatment of retroperitoneal soft
tissue sarcoma (STS) is to attempt a gross total
resection.
• Criteria for unresectable tumors commonly
include major vessel invasion and spinal cord
or vertebral body involvement.
• Resectability has been reported in recent
series to range from 65% to 85%.
• Macroscopically positive margin is one the
most important prognostic features.
• Even after a complete excision of RPS, local
recurrence rates (33% to 77%) and overall
survival rates (35% to 63% 5-ys OS) are poor.
• retrospective data demonstrate a decrease in
local failure with adjuvant radiation therapy.
• Patients with unresectable disease:
– preoperative radiotherapy or chemotherapy in
attempt to shrink the tumor.
– Palliative debulking surgery may be offered.
• Patients with metastatic disease:
– Solitary or limited metastases:
• preoperative radiation therapy and/or chemotherapy,
metastasectomy, radiofrequency ablation. Chemotherapy
may be considered (most commonly, Doxorubicin-based
regimens).
– Disseminated metastatic disease:
• treated with palliative intent (chemotherapy, radiation
therapy, palliative surgery or best supportive care.)
Desmoid tumour
• Rare, benign neoplasms.
• Arise from muscle fascia, aponeuroses, tendons, and
scar tissue.
• More commonly in females .
• Associated with familial adenosis polyposis .
• locally aggressive and high chances to recur locally
after resection.
• Treatment is complete resection with a 2-3 cm margin.
• Postoperative radiation therapy is used in cases with an
unresectable primary, after multiple local recurrences.
Schwannomas
• Most common benign tumors in the retroperitoneum.
• Typically form large, well-circumscribed masses (may
display cystic degeneration, calcification, hemorrhage).
• MRI is the recommended imaging study.
• Not suggest CT-guided biopsy or fine-needle aspiration
for the diagnosis because of the risk of hemorrhage as
it is usually a highly vascular tumor.
• Complete surgical resection with negative margins is
the treatment of choice

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Diagnostic Workup & Treatment of Retroperitoneal Sarcomas

  • 1.
  • 2. Diagnostic work up • Computed tomography (CT): – Large lesions with fatty components causing mass effect frequently represent liposarcomas. – Teratomas often have fat, fluid, and calcified components. – Paraspinal locations point to nerve sheath tumors or neurogenic derivation . • Magnetic resonance imaging (MRI) may demonstrate additional imaging details to further classify the lesion. • CT-guided needle biopsy may be performed as the initial means of obtaining tissue for histologic diagnosis
  • 3. RETROPERITONEAL SARCOMA • Second most common malignant RPT. • Arise from: nonepithelial, extraskeletal tissues: (fat, muscle, nerve and nerve sheath, blood vessels, other connective tissues). • Incidence: 9,220 cases per year in the United States. • Common types: – Leiomyosarcoma – Liposarcoma – Fibrosarcoma
  • 4.
  • 5. • Primary treatment of retroperitoneal soft tissue sarcoma (STS) is to attempt a gross total resection. • Criteria for unresectable tumors commonly include major vessel invasion and spinal cord or vertebral body involvement. • Resectability has been reported in recent series to range from 65% to 85%.
  • 6. • Macroscopically positive margin is one the most important prognostic features. • Even after a complete excision of RPS, local recurrence rates (33% to 77%) and overall survival rates (35% to 63% 5-ys OS) are poor. • retrospective data demonstrate a decrease in local failure with adjuvant radiation therapy.
  • 7. • Patients with unresectable disease: – preoperative radiotherapy or chemotherapy in attempt to shrink the tumor. – Palliative debulking surgery may be offered. • Patients with metastatic disease: – Solitary or limited metastases: • preoperative radiation therapy and/or chemotherapy, metastasectomy, radiofrequency ablation. Chemotherapy may be considered (most commonly, Doxorubicin-based regimens). – Disseminated metastatic disease: • treated with palliative intent (chemotherapy, radiation therapy, palliative surgery or best supportive care.)
  • 8. Desmoid tumour • Rare, benign neoplasms. • Arise from muscle fascia, aponeuroses, tendons, and scar tissue. • More commonly in females . • Associated with familial adenosis polyposis . • locally aggressive and high chances to recur locally after resection. • Treatment is complete resection with a 2-3 cm margin. • Postoperative radiation therapy is used in cases with an unresectable primary, after multiple local recurrences.
  • 9. Schwannomas • Most common benign tumors in the retroperitoneum. • Typically form large, well-circumscribed masses (may display cystic degeneration, calcification, hemorrhage). • MRI is the recommended imaging study. • Not suggest CT-guided biopsy or fine-needle aspiration for the diagnosis because of the risk of hemorrhage as it is usually a highly vascular tumor. • Complete surgical resection with negative margins is the treatment of choice