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Radiation Oncology/Benign/Dupuytrens

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Dupuytren's Contracture


Overview

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  • Proliferative disorder of connective tissue involving palmar fascia of the hand
  • Highest prevalence in regions of Scotland, Ireland, and France
  • Usually starts in 4th decade, and peaks in 5th - 6th decade
  • Male: female = 3:1, with 2/3 developing bilateral disease
  • Etiology and pathogenesis still poorly understood
  • Clinical progression:
    • Early stage: subcutaneous nodules, may be fixed to skin
    • Intermediate stage: tough cords
    • Late stage: cords attach to periosteum of hand bones, leading to characteristic contracture of the palm, medial phalangeal (MP) and proximal interphalangeal joints (PIP)
  • Typically no invasion of muscles (unlike desmoid)
  • Clinical progression in early stage disease ~50%
  • Role of RT based on retrospective studies. Based on mechanism of disease, RT appears best when used early in the disease process. Randomized evidence suggests 3 Gy x 7 fractions sufficient


Staging

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Tubiana's Classification (1966)

Stage Description Contracture degree
N Symptoms only (nodules, skin retraction, fixation) None
N/I Symptoms + deformity 1-10
I Symptoms + deformity 11-45
II Symptoms + deformity 46-90
III Symptoms + deformity 91-135
IV Symptoms + deformity >135

Evidence

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  • Essen, Germany (1997-1998) -- RT 30/10 (15/5 + 15/5) vs RT 21/7
    • Randomized. 129 patients, 198 involved hands. Early stage progressive disease, no surgery. Stage N (37%), Stage N/I (31%), Stage I (30%), Stage II (3%). Prophylactic RT orthovoltage, margin 1-2 cm proximal/distal and 0.5-1 cm radial. Arm 1) RT 15/5, 8 week break, 15/5 vs. Arm 2) RT 21/7. Minimum F/U 1 year
    • 2001 PMID 11172962 -- "Radiotherapy optimization in early-stage Dupuytren's contracture: first results of a randomized clinical study." (Seegenschmiedt MH, Int J Radiat Oncol Biol Phys. 2001 Mar 1;49(3):785-98.)
      • Outcome: 1-year regression 56% vs 53% (NS), stable 37% vs. 38% (NS), progression 7% vs 9% (NS). Treatment "failure" 8%, but only 2% required hand surgery for progression
      • Toxicity: Acute minimal, chronic Grade 1 in 5% sites, no difference between groups
      • Conclusion: Both dose schedules equally effective to prevent disease progression. Long term F/U needed
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