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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