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Case Report
Copyright: ©Author(s) 2026.
World J Clin Oncol. Aug 24, 2026; 17(8): 124132
Published online Aug 24, 2026. doi: 10.5306/wjco.124132
Table 1 Summary of congenital dermatofibrosarcoma protuberans case series (≥ 3 cases)
Ref.
Cases
Presentation/location
Size
Diagnostic delay
Treatment (margin)
Outcome (followup)
Maire et al[6], 200798 plaques, 1 nodule; trunk (4), lower extremity (2), other (3); all initially suspected benign< 0.5-10 cm (most > 4 cm)5.5 months-15 yearsWide excision; reexcision for positive margins (3)2 cases NED at 7 months and 6 months; 1 untreated (family refused); others NR
Terrier-Lacombe et al[7], 20032Buttock and thigh2-3 cmBorn with lesionsWide excision1 NED at 36 months; 1 NA
Posso-De Los Rios et al[8], 20147Trunk: Back (4), abdomen (1); scalp (1); genital (1); 4 masses, 2 nodules, 1 atrophic plaqueMean 3.0 ± 1.4 cmMean 6.1 yearsWLE (majority); margins 1-4 cm; reexcision for positive margins (2), imatinib (1 case)No recurrence (mean 3.0 years; range 1 months - 8 years)
Weinstein et al[9], 20036Scalp, ankle, back (2), neck, thigh; 50% in atypical locations (scalp, neck, ankle); all initially misdiagnosed0.7-3 cm5 months-14 yearsWLE (5), MMS (1); 4/6 positive margins → reexcision3 NED (1-6 years); 1 recurrence; 1 LFU; 0 metastasis


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