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Table 6 Reported cases of (c)SCC, in EB patients treated by radiotherapy and topical photodynamic therapy

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

Reference (s) Age yr/sex EB subtype Histological subtype of cSCC Histological differentiation Site(s) treated Radiation delivery Response Survival (time before death) Note (s)
Didolkar19 33 F RDEB Adenoacanthoma Unknown Sacrum Cumulated dose = 60Gy GR Death (?) Death due to hypercalcemia (and unknown metastasis)
Reed20 32 M RDEB Unknown Unknown Back Unknown GR Death (3 years) Death due to other cSCC
Keeff21 35 F RDEB Common Well Hand 3Gyx10 = 30Gy
3Gyx11 = 33Gy
PR then NR Death (?)  
McGrath22 48 F RDEB Angiosarcomatoid Unknown Wrist
5Gyx9 = 45Gy
4Gyx3 = 12Gy
Death (6months)  
Schreiber23 33 M RDEB Unknown Unknown Cervical lymphadenopathy Unknown Unknown Unknown  
Bastin24 41 F
28 M
RDEB Common
1.8Gyx32 = 57.6Gy
1.5x25Gy = 37.5Gy
PR then NR
Death (4months)
Death (1month)
Lotem25 34 F KS Common Well Hard palate 1.8x40Gy = 72Gy CR Alive at 2 years  
Weber12 26 F RDEB Common Moderately Leg and groin Cumulated dose = 60Gy PR Death (7months)  
Mseddi26 18 F RDEB Common Well Groin Cumulated dose = 45Gy NR Death (2months)  
Mallipedi27 45 M JEB Unknown Unknown Bladder Unknown Unknown Unknown Lost to follow-up
Souza28 51 F RDEB Bowen disease Not applicable Hand Not applicable CR Alive at 2 years PDT+ 5ALA
Emmanuel29 57 F KS Unknown Moderately-poorly Hand Unknown NR Unknown Neoadjuvant RTH
Arnold7 24 F RDEB Unknown Well Arm
Cumulated dose = 61.2 and 50Gy PR Alive at 3months Remission probably due in part to systemic therapya
Mituzani30 43 M KS Common Well Knee and epiglottis Unknown CR Alive at 2.5 years  
Yuen5 55 M JEB Common Unknown Nasal cavity Unknown CR Alive at 7months  
Onsun31 45 F RDEB-I Unknown Well Oesophagus Unknown NR Death (8months)  
Kim18 26 F
43 M
RDEB Common Moderately
Unknown NR Death (6months)
Death (2months)
Despite systemic treatmentb, c
  1. CR complete response, cSCC squamous cell carcinoma, F female, GR good response, EB inherited epidermolysis bullosa, JEB junctional epidermolysis bullosa, KS Kindler syndrome, M male, mo month, NR non-response, PDT photodynamic therapy, PR partial response, RDEB recessive dystrophic epidermolysis bullosa, RTH radiotherapy, Yr year, 5ALA 5 aminolevulinic-acid
  2. a5FU-cisplatin then carboplatin-taxol then cetuximab
  3. bcetuximab then cetuximab-gemcitabine
  4. ccetuximab then methotrexate orally then methotrexate intravenously