Case Report

Cutaneous Epithelioid Clear Cells Angiosarcoma in a Young Woman with Congenital Lymphedema

Table 1

Clinical features of patients with cutaneous angiosarcoma associated with congenital lymphedema described in the literature (modified from Offori et al., 1993 [12] and Bernardi et al., 2009 [18] ).

AuthorSexAge at diagnosisTumor siteClinical appearanceInterval in diagnosisTreatmentSurvival

Kettle [19]F44 yRLBlue-red dislocationNot statedAmputationNot stated
Liszauer et al. [20] M28 yRLLUlcerated lesion4 mLocal excisionDied at 11 m
Scott et al. [21]F50 yLULPapillomatous nodules>7 mLocal excision/amputationDied at 37 m
Taswell et al. [22] M17 yLULUlcerated lesionNot statedDisarticulationDied at 24 m
Bunch [23] F13 yULNot statedNot statedInterscapular amputationDied at 1 y
Finlay-Jones [24] M34 yLLLUlcerated blue tumorNot statedExcision and combined therapyDied at 31 m
Merrick et al. [25] M52 yLULSwelling/blue nodules6 mWide local excision/amputation/radiotherapyDied at 36 m
Mackenzie [26] M64 yRLLNodules/ulceration2 mHindquarter amputation/radiotherapyAlive at 24 m
Dubin et al. [27] F29 yLULUlcerated lesion3 yDisarticulationDied at 45 m
Laskas et al. [28]F85 yRULPurple papules and blisters/ulceration10 mPalliative midhumeral amputationDied at 14 m
Banathy et al. [29] F50 yLULPurple nodulesNot statedMidhumeral amputationDied at 26 m
Sordillo et al. [30] F23 yNot statedNot statedHemipelvectomyAlive at 19 y
Bostrom et al. [31] F19 yRULInfected lesion4 mAmputation/disarticulationDied at 12 m
F10 yLLLBlue/red nodules8 mAmputation/disarticulationAlive at 10 m
Offori et al., 1993 [12]F43 yLLLBlue/purple nodules2 mAmputationAlive at 9 m
Andersson et al. [32] M35 yRLBlue nodulesNot statedHemipelvectomy, chemotherapyNot stated
Cerri et al. [33] F42 yPubisEcchymotic plaqueNot statedHemipelvectomy, radiotherapyNot stated
Bernardi et al. [18]F4 yLLLUlcerated lesion/blue nodules1 yLocal excisionAlive at 14 m
Deyrup et al., 2011 [14]F2 yFootNot statedNot statedLocal excisionDied at 1 y
Present caseF21 yGroinPurple/reddish infiltrated and ulcerated mass10 mLocal excisionDied at 3 m

LLL: left lower limb; RLL: right lower limb; LUL: left upper limb; RUL: right upper limb; m: months; and y: years.