Updated: 12/15/2019

Epithelioid Sarcoma

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Introduction
  • A rare malignant slow-growing, nodular, soft tissue tumor
    • often mistaken for a benign granulomatous process.
  • Age & location
    • occurs in adolescents and young adults (ages 10-35 years)
    • 2:1 male to female ratio
    • 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
  • Genetics
    • unknown
  • Malignancy
    • regional lymph node metastases common  
    • systemic metastasis to lungs can occur
  • Prognosis
    • extremely poor
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 post 
    • 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
 

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(SBQ05PA.2) A 25-year-old right hand dominant male presents for a second opinion with the painless mass seen in Figure A. He denies recent fevers, chills, or any other systemic symptoms. An incisional biopsy is performed and a histologic specimen of the lesion is seen in Figure B. Which of the following is the most likely diagnosis? Review Topic | Tested Concept

QID: 1987
FIGURES:
1

Rheumatoid nodule

5%

(181/3754)

2

Ganglion cyst

4%

(152/3754)

3

Epithelioid sarcoma

79%

(2953/3754)

4

Squamous cell carcinoma

10%

(376/3754)

5

Chondrosarcoma

2%

(69/3754)

L 2 C

Select Answer to see Preferred Response

(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? Review Topic | Tested Concept

QID: 1988
FIGURES:
1

Rheumatoid nodule

2%

(15/927)

2

Squamous cell carcinoma

39%

(362/927)

3

Epithelioid sarcoma

52%

(479/927)

4

Angiosarcoma

4%

(41/927)

5

Epidermal inclusion cyst

3%

(25/927)

L 4 C

Select Answer to see Preferred Response

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