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Table 1 EB patients treated with cetuximab in the literature

From: EGFR inhibition for metastasized cutaneous squamous cell carcinoma in dystrophic epidermolysis bullosa

 Age at tumor’s presentation (years)Tumor characteristicsLymph node metastases presentOrgan metastases presentTreatment options before cetuximabaPatient outcome under cetuximabReference
129primary tumor on right forearm; axillary lymph node metastases as well as in-transit cutaneous metastases on the right upper limbaxillarytreatment initially well-tolerated, but wound healing deficits after 6 months; progression free survival for 9 months; treatment discontinuation after 2 years after grade 2 allergic reaction; death 1 year later after use of other treatment optionsMedek et al., J Dtsch Dermatol Ges. 2019
224primary well-differentiated SCC on right elbow; subcutaneous metastasis next to SCC; tumor regrowth within weeks after 3x excisions (amputation declined by patient)2 axillaryradiotherapy; chemotherapy with cisplatin and 5-fluorouracil (2 cycles)> > chemotherapy with cisplatin and paclitaxel for 1 month> > cetuximab for 12 cyclesbesides acneiform folliculitis on the face treatment well-tolerated; progression free survival for 3 monthsArnold et al., Dermatology, 2009
326moderately differentiated SCC on the dorsal right hand; quick tumor regrowth after excision, resulting in amputation2/22 axillarylungaxillary dis-section followed by local radiotherapy> > cetuximab for 10 cycles> > due to progression of lung metastasis combination with gemcitabinetreatment well tolerated; death around 3 weeks after initiation of cetuximab+ gemcitabine treatment on pneumoniaKim et al., Br J Dermatol. 2013
443well-differentiated SCC in axilla with extensive extranodal local spread; recurrence in axilla within 3 months after axillary node dissection6/16 axillarylungaxillary dis-section followed by local radiotherapy; cetuximab for 7 cycles> > due to development of multiple lung metastases: methotrexate 40 mg orally and then i.v. for 9 weeks> > treatment cessation due to progressive diseaseinitially development of a vesicular eruption, no other side effects; death 2 months after treatment discontinuation on pneumoniaKim et al., Br J Dermatol. 2013
5nananananaworsening of skin lesionsMaubec et al., J Clin Oncol. 2011
  1. All patients published so far had severe generalized recessive DEB
  2. Legend: +, present; −, absent; a, treatment options are presented in chronological order; na not available