Retroperitoneal Sarcoma

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Retroperitoneal Sarcoma

• Edward Chin, M.D.
• February 20, 2003

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Case

• 68F with asymptomatic abdominal mass
• s/p lumpectomy/axillary dissection for breast
  cancer 00
• CT scan reveals large R retroperitoneal mass, R
  hydronephrosis
• Further studies (MRI/A, bone scan, chest CT)

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Hospital Course

• CT-guided biopsy --gt spindle cell tumor

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OR

• 12/13/03
• Retroperitoneal mass adherent to duodenum, R
  kidney, vena cava
• En bloc resection of mass with kidney, sleeve of
  duodenum, cava
• Biopsy tract excised

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Pathology

• High-grade spindle cell tumor
• Duodenal, kidney margins negative
• Caval margin?

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Spindle Cell Tumors

• GIST
• Sarcomas
• Liposarcoma
• Leiomyosarcoma
• Malignant peripheral nerve sheath tumor
• Melanoma
• Sarcomatoid carcinoma
• Fibrous tumor
• Fibromatosis
• Histopathology 2001 38 387-402

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Retroperitoneal Sarcoma

• Soft tissue sarcomas 1 of solid tumors
• 15 retroperitoneal
• Presentation
• New abdominal mass (80-90)
• Neurologic deficit (lumbosacral plexus, femoral,
  obturator nerves)
• Bowel or ureteral obstruction
• Anemia, GI bleeding
• Fever

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Size

• Late presentation
• 500 patients at MSKCC

Ann Surg 1998 228 355-65
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Imaging Appearance

• Heterogenous mass
• Areas of liquefaction
• Adjacent abdominal structures ? displaced

Pathognomonic anterior rotational displacement
of kidney
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Differential Diagnosis

• Lymphoma
• GU tumor (renal cell, ureteral)
• GIST
• Ovarian tumor
• Adrenal tumor
• Metastasis
• e.g. germ cell examine testes!

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Preoperative biopsy

• Only indicated if
• Diagnostic uncertainty
• Potential for different management
• Open vs. percutaneous (extraperitoneal)
• Lymphoma (Type B symptoms weight loss, night
  sweats, fever)

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Surgical Approach

• Goal is en bloc resection, negative margins
• Frequently requires resection of adjacent
  structures
• Kidney (46)
• Colon (24)
• Pancreas (10)
• Large vessel (10)
• Spleen (10)
• Ann Surg 1990 212(1) 51-58

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Results

• MSKCC 500 pts with RTS (1982-1997)

Ann Surg 1998 228(3) 355-365
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Median Survival
Ann Surg 1998 228(3) 355-365
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Incomplete Resection

• 55 patients with retroperitoneal liposarcomas

grossly positive margins JACS 2001 193(4)
373-79
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Adjuvant Therapy

• Randomized, prospective trial of intraop
  XRT/low-dose post-op (group 1) vs. high-dose
  post-op (group 2)

Arch Surg 1993 128 402-412