Updated: 6/22/2021

Epithelioid Sarcoma

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  • summary
    • Epitheliod Sarcomas are rare, slow-growing, malignant, nodular, soft tissue tumors that most commonly occur in the hand and wrist.
    • The condition is typically seen in patients between the ages of 10 and 35 who present with a small, firm, painless, mass in the upper extremity.
    • Diagnosis is made with a biopsy showing a characteristic nodular pattern with central necrosis within granulomatous areas.
    • Treatment is usually wide surgical excision with radiation. 
  • Epidemiology
    • Demographics
      • occurs in adolescents and young adults (ages 10-35 years)
      • 2:1 male to female ratio
    • Anatomic location
      • most common soft tissue sarcoma of the hand and wrist
        • also occurs in forearm, buttock/thigh, knee, and foot
      • mass is deep or superficial
        • may ulcerate and mimic skin carcinoma, rheumatoid nodule, or granuloma when superficial
        • may be attached to tendons, tendon sheaths, or fascia when deep
  • Etiology
    • Genetics
      • unknown
    • Malignancy
      • regional lymph node metastases common
      • systemic metastasis to lungs can occur
  • Symptoms
    • Symptoms
      • small, firm, painless, slow-growing mass commonly occurring in the upper extremity
    • Physical exam
      • 3-6 cm firm, painless mass
        • may have skin ulceration
  • Imaging
    • Radiographs
      • calcification occurs within the lesion in 10-20%
      • erosion of adjacent bone is sometimes found
    • MRI
      • indeterminate in appearance
        • dark on T1
        • bright on T2
      • tendon sheath nodule may be visualized
  • Histology
    • Characteristics
      • nodular pattern with central necrosis within granulomatous areas
      • epithelial appearance with ovoid or polygonal cells with eosinophilic cytoplasm
      • dense, hyalinized collagen deposits intercellularly
      • cellular pleomorphism is rare
      • keratin-positive staining
  • Treatment
    • Operative
      • wide excision with adjuvant radiotherapy
        • indication
          • all operable tumors
        • technique
          • perform sentinel node biopsy to evaluate for regional lymph node metastasis
        • outcomes
          • high rate of multiple recurrences if mistaken for a benign lesion and inadequately excised
          • re-excision of the tumor bed is recommended for inadequate initial resection
      • amputation
        • indications
          • may be necessary to prevent spread of disease in cases of multiple recurrences
  • Prognosis
    • Extremely poor

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(SBQ05PA.3) Figure A shows the clinical photo of a 28-year-old homeless man who presents with a left thumb mass which has been present for the last three years. He reports having a similar mass that was excised at an outside hospital 5 years earlier. A biopsy of the mass is taken, and the histopathology is shown in Figures B and C. What is the most likely diagnosis?

QID: 1988
FIGURES:
1

Rheumatoid nodule

2%

(22/1133)

2

Squamous cell carcinoma

39%

(438/1133)

3

Epithelioid sarcoma

51%

(580/1133)

4

Angiosarcoma

5%

(54/1133)

5

Epidermal inclusion cyst

3%

(31/1133)

L 4 D

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