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Table 5 Systemic treatment of cSCC in EB patients

From: Inherited epidermolysis bullosa and squamous cell carcinoma: a systematic review of 117 cases

Reference (s) Age (yr)/sex EB subtype Site of cSCC Histological differentiation/size of tumor (cm) Site(s) of metastases Treatment Outcome
Schwartz16 55 F DDEB Lower extremity (knee) Well/>5 Not applicable Intra arterial Doxorubicin + MTX Surgical excision
Survival unknown
Lentz17 22 F
26 F
RDEB Upper extremity (forearm)
Trunk
Well/unknown
Unknown/>2 < 5
Axillary lymph nodes, pulmonary nodules
Axillary lymph nodes
Cisplatin
5FU-cisplatin
PR
Death from cSCC
PR, surgical resection, alive at 12mo
Arnold7 24 F RDEB Upper (elbow) and lower (feet) extremities Well/>5 Axillary lymph nodes 1.5FU-cisplatin
2. carboplatin-taxol
3. cetuximab
1. PR
2. PD
3. GR, alive at 3mo
Kim18 26 F
43 M
RDEB Upper (hand) extremity
Unknown
Moderately/>5
Well/>5
Axillary lymph nodes, pulmonary nodules
Axillary lymph nodes, pulmonary nodules
1. cetuximab
2. cetuximab-gemcitabine
1. cetuximab
2. MTX orally
3. MTX IV
1. PD
2. Death from pneumonia
1. PD
2. Death from pneumonia
  1. cSCC squamous cell carcinoma, cm centimeter, DDEB dominant dystrophic epidermolysis bullosa, F female, GR good response, EB inherited epidermolysis bullosa, IV intravenously, M male, mo month, MTX methotrexate, PD progressive disease, PR partial response, RDEB recessive dystrophic epidermolysis bullosa, Yr year, 5FU 5 fluorouracil