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Sarcoma (Epitheloid and Synovial)

  • Epithelioid sarcoma:
    • A high-grade soft-tissue sarcoma that is prone to local recurrence, lymph node invasion, and pulmonary metastases.
    • Most common soft-tissue sarcoma of the upper extremity
    • Often confused with granulomatous processes
    • Occurs often in young patients (15-40 years old)
  • Synovial sarcoma:
    • A high-grade malignancy on the soft tissue
    • Occurs in para-articular regions
    • Rarely occurs inside a joint
    • Locations:
      • Lower extremity: 60%
      • Upper extremity: 25%
      • Trunk: 10%
      • Head/neck: 10%
General Prevention
No preventive means are known.
No causes are known, but a genetic connection for synovial sarcoma exists.
  • No data available for epithelioid sarcoma:
    • Young patients
    • Upper extremity
  • Synovial sarcoma:
    • Occurs in young patients: 15-40 years old
    • Male:Female ratio is 1.2:1.
Risk Factors
No risk factors are known.
  • Epithelioid: No genetic factors are known.
  • Synovial:
    • A characteristic chromosomal abnormality is found in all cases.
    • Balanced reciprocal translocation: t(X;18) (p11.2;q11.2):
      • SYT gene on chromosome 18
      • SSX1 or SSX2 on the X chromosome
      • Gene fusion products: SYT-SSX1, SYT-SSX2
  • Epithelioid and synovial sarcoma:
    • Unregulated growth of the soft-tissue mass
    • Hematologic spread to the lungs
    • Lymphatic spread to the lymph nodes
  • Epithelioid sarcoma can arise in the superficial or deep tissues.
    • When superficial, it grows in the subcutaneous tissues as a nodule and may ulcerate through the skin.
    • In the deep tissues, it often is firmly attached to muscles, tendons, or fascial structures.
No etiologic factors are known.
Associated Conditions
No associated conditions are known.
Signs and Symptoms
  • Patients present with a soft-tissue mass.
    • Pain is present in ~50%.
    • Some patients note a long-term presence of the mass.
    • May be slow or rapid growth
Patients may note that the mass has been present for a short time and is growing or that it has existed for a long time with little or no growth.
Physical Exam
  • Carefully examine the extremity and note the following features of the mass:
    • Size
    • Depth: Above or below the fascia (attached to skin or deep tissues?)
    • Mobility: Fixed or movable?
  • Also note any overlying skin changes, such as erythema.
  • Check for lymphadenopathy.
Imaging studies are the main modality for defining the anatomic parameters of the mass.
No specific laboratory studies
  • Radiography:
    • Plain films are used to evaluate the primary lesion.
    • Look for:
      • Cortical bone destruction
      • Periosteal reaction
      • In synovial sarcoma only: Mineralization within the mass (occurs in ~20% of patients)
  • MRI:
    • The most useful imaging study
    • Used to define lesion’s size, depth, and relationship to important structures such as nerves and blood vessels
  • CT:
    • Used to look for pulmonary metastases and lymphadenopathy
    • Chest in all patients
    • Pelvis/abdomen, axilla to evaluate lymph node chains from the primary site
Diagnostic Procedures/Surgery
  • Needle biopsy is necessary to differentiate synovial sarcoma from other sarcomas and the many benign causes of soft-tissue tumors.
  • Because of the propensity for lymph node involvement, sentinel-node biopsy sometimes is necessary.
Pathological Findings
  • Characteristic morphologic findings for epithelioid sarcoma:
    • Nodular growth pattern
    • Central necrosis
    • Epithelial appearance of the cells
  • For synovial sarcoma, several types (listed in order of occurrence):
    • Biphasic (epithelial and fibrous cells)
    • Monophasic fibrous
    • Poorly differentiated
    • Monophasic epithelial
Differential Diagnosis
  • Soft-tissue masses have a long list of benign and malignant differential diagnoses.
  • Epithelioid sarcoma can be confused with a number of entities, both clinically and pathologically:
    • Granuloma annulare
    • Rheumatoid nodule
    • Squamous cell carcinoma
    • Necrotizing infectious granuloma
    • Necrobiosis lipoidica
Special Therapy
  • Virtually all patients are treated with a combination of wide surgical resection and radiation therapy.
  • Radiation therapy can be delivered in a number of different ways:
    • Preoperative external beam
    • Postoperative external beam
    • Brachytherapy tubes
Physical Therapy
Physical therapy often is used after surgery to regain ROM and strength.
  • Chemotherapy often is used to reduce the risk of pulmonary metastases.
    • Multiagent intensive therapy
  • Wide surgical resection with as large a margin as possible is the cornerstone of treatment and necessary to reduce the risk of local recurrence.
    • Lymph node dissection is necessary if abnormal nodes are found on physical examination or after imaging.
    • Limb preservation in >90% of patients
    • Amputation occasionally is necessary.
      • Involvement of major nerves and blood vessels
      • Inability to remove the entire tumor with negative margins
  • Patients are followed at close intervals to monitor for pulmonary metastases and local recurrence.
    • CT of the chest every 3-4 months for 2-3 years, then every 6 months until 5 years posttreatment, and then annually thereafter
    • MRI with contrast of the limb every 6 months for 2-3 years to monitor for local recurrence
  • Patients with epithelioid and synovial sarcoma should be referred to a multidisciplinary team:
    • Orthopedic oncologist
    • Radiation oncologist
    • Medical oncologist
    • Plastic surgeon
  • The prognosis for epithelioid sarcoma is fair with adequate local control.
    • ~40-50% disease-free survival
    • Metastases to lymph nodes, lungs, and other soft-tissue sites
  • Poor prognostic findings:
    • Male gender
    • Nondistal extremity tumors
    • Size >5 cm
    • Increased tumor depth
    • High mitotic index
    • Necrosis
    • Vascular invasion
    • Inadequate initial excision
  • The prognosis for synovial sarcoma is excellent if local control can be achieved and pulmonary metastases do not develop.
    • 5-year survival rates have been reported to be 50-80%.
    • Favorable variables:
      • Age <25 years
      • Size <5 cm
      • Absence of poorly differentiated areas
    • Unfavorable variables:
      • Age >40 years
      • Size >5 cm
      • Poorly differentiated areas
  • The major complications of surgery and irradiation are:
    • Delayed wound healing
    • Arthrofibrosis (stiffness in joints)
    • Infection
Patient Monitoring
Patients are followed closely after surgery to monitor wound healing, ROM, local recurrence, and pulmonary metastases.
171.___Neoplasm, malignant, connective tissue
Patient Teaching
  • Patients must be taught the essential features of this tumor and the principles of treatment.
    • Wide surgical resection is necessary.
    • Irradiation often is necessary to reduce the risk of local recurrence.
    • Careful monitoring with imaging studies and physical examination is necessary after surgery.
Unrestricted activity after full wound healing
No preventive methods
Q: Is surgery necessary for all patients?
A. Surgery with as wide a margin as possible is necessary to reduce the risk of local recurrence.
Q: What is the risk of pulmonary metastases?
A: Metastasis to the lungs occurs in ~50% of patients.
Q: How often can the limb be saved?
A: Limb preservation can be achieved in ~90% of patients.
Q: Is chemotherapy necessary for all patients?
A: Patients at high risk for pulmonary metastases, especially those with large tumors (>10 cm), should consider chemotherapy.

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