Epithelioid Sarcoma

Topic updated on 12/10/12 5:19pm
  • 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
    • small, firm, painless, slow growing mass commonly occurring in the upper extremity
  • Physical exam
    • 3-6 cm firm, painless mass
      • may have skin ulceration
  • 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
  • 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
  • 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
    • amputation
      • indications
        • may be necessary to prevent spread of disease in cases of multiple recurrences


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Qbank (3 Questions)

(SBQ05.1) A 25-year-old man presents with a nodular mass on the palm of his hand that has been slowly enlarging over the past six months. The patient denies a history of injury, arthralgias, fevers, and weight loss. On examination, the mass is firm with minimal tenderness to palpation and no erythema. A clinical photograph and biopsy specimen are shown in Figures A and B. The lesion depicted here has a propensity to metastasize to what location? Topic Review Topic
FIGURES: A   B        

1. Brain
2. Bone
3. Lung
4. Lymph nodes
5. Thyroid

(SBQ05.2) 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? Topic Review Topic
FIGURES: A   B        

1. Rheumatoid nodule
2. Ganglion cyst
3. Epithelioid sarcoma
4. Squamous cell carcinoma
5. Chondrosarcoma

(SBQ05.3) 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? Topic Review Topic
FIGURES: A   B   C      

1. Rheumatoid nodule
2. Squamous cell carcinoma
3. Epithelioid sarcoma
4. Angiosarcoma
5. Epidermal inclusion cyst



This video demonstrates the histologic characteristics of a Epithelioid Sarcoma....
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