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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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