Epithelioid Hemangioendothelioma with WWTR1-CAMTA1 Fusion in the Parotid Gland Presenting as Bell’s Palsy

Table 1

Characteristics of published cases of epithelioid hemangioendothelioma in the parotid gland.

	Current case	Suarez-Zamora et al. [9]	Koide et al. [8]	Falvo et al. [6]	Amin et al. [7]	Pigadas et al. [10]
Age, gender	37, female	62, female	70, female	28, male	81, male	48, female
Presenting signs/symptoms	24-month history otalgia, recent waxing-waning facial nerve palsy	12-month history slow-growing painless mass	18-month history swelling and pain	2-month history of rapidly enlarging lump with intermittent oppressive pain	6-month history initially asymptomatic mass; deep boring pain developed during workup	12-month history swelling with recent enlargement and intermittent sharp pain
Physical exam findings	CN VII paralysis and numbness; area of tenderness and fullness without definite mass	Mass partially fixed to neighboring tissues	Left CN XII paralysis; mass with overlying skin erythema & induration	Mass fixed to underlying planes	NR	Firm, semifixed, nontender mass
Size of tumor (cm)	1.2	1.8	4.2	3.8	2.0	1.5
Perineural invasion	+	NR	NR (presumed positive)	-	+	NR
Mitotic rate	<1 per 10 HPF	<3 per 50 HPF	5 per 50 HPF	5 per 10 HPF	>1 per HPF	“Inconspicuous”
Ki-67 (MIB-1) index	NR	5%	NR	6%	NR	“Negative staining”
Cytologic atypia	Marked	Absent	NR	Modest	Marked	Minimal
Necrosis	-	-	NR	-	+	NR
WWTR1-CAMTA1 fusion	+	NR	+	NR	NR	NR
Margins	Negative	NR (presumed negative)	Positive	NR (presumed negative)	Negative	NR (presumed negative)
Treatment	Surgery (TP)	Surgery (SP)	Surgery (TP, node dissection), radiation	Surgery (TP, node dissection), radiation	Surgery (TP)	Surgery (SP)
Metastasis	No	No	None at presentation, died from distant mets seen at nearly 5 months in lung liver and lumbar spine	No	No	No
Outcome	NED 12 months	NED 12 months	DOD 13 months	NED 18 months	NED 7 months	NED 18 months

NR: not reported; HPF: high power field; TP: total parotidectomy; SP: superficial parotidectomy; NED: no evidence of disease; DOD: died of disease.