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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 characteristics

Lymph node metastases present

Organ metastases present

Treatment options before cetuximaba

Patient outcome under cetuximab

Reference

1

29

primary tumor on right forearm; axillary lymph node metastases as well as in-transit cutaneous metastases on the right upper limb

axillary

treatment 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 options

Medek et al., J Dtsch Dermatol Ges. 2019

2

24

primary well-differentiated SCC on right elbow; subcutaneous metastasis next to SCC; tumor regrowth within weeks after 3x excisions (amputation declined by patient)

2 axillary

radiotherapy; chemotherapy with cisplatin and 5-fluorouracil (2 cycles)> > chemotherapy with cisplatin and paclitaxel for 1 month> > cetuximab for 12 cycles

besides acneiform folliculitis on the face treatment well-tolerated; progression free survival for 3 months

Arnold et al., Dermatology, 2009

3

26

moderately differentiated SCC on the dorsal right hand; quick tumor regrowth after excision, resulting in amputation

2/22 axillary

lung

axillary dis-section followed by local radiotherapy> > cetuximab for 10 cycles> > due to progression of lung metastasis combination with gemcitabine

treatment well tolerated; death around 3 weeks after initiation of cetuximab+ gemcitabine treatment on pneumonia

Kim et al., Br J Dermatol. 2013

4

43

well-differentiated SCC in axilla with extensive extranodal local spread; recurrence in axilla within 3 months after axillary node dissection

6/16 axillary

lung

axillary 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 disease

initially development of a vesicular eruption, no other side effects; death 2 months after treatment discontinuation on pneumonia

Kim et al., Br J Dermatol. 2013

5

na

na

na

na

na

worsening of skin lesions

Maubec 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