Table 1.

Summary of recent cases of Ewing sarcoma FT of the pancreas in the literature.

Author	Age	Gender	Symptom	Pathologic features	Cytogenetics	Primary/metastatic	Location	Size (cm)	Treatment	Progression/outcome	Ref.
Present case	29	M	None	CD99	EWSR1-FLI1 fusion (type 1)	Metastatic—primary at the right tricep with metastases to the right lung, right greater sphenoid, and most recently to the pancreas	Body and neck	1.8 × 1.6	Cyclophosphamide, topotecan, and RAD	12 mos; later; NED	Present case
Nishizawa et al. (2015)	22	M	Upper abdominal pain, nausea, and vomiting	CD99, NSE, VIM, synaptophysin, and neural cell adhesion molecule	22q11 rearrangement	Primary	Pancreatic head	8.5 × 5.0 × 6.2	Whipple, CHE, and RAD	12 mos; AWD	[5]
Rubin et al. (1985)	28	M	None	N/A	N/A	Metastatic—primary ES of the right pubic bone with recurrence in 3 years and metastasis to the pancreas		8 × 6	CHE and RAD	8 mos; died of disease	[10]
Obuz et al. (2000)	15	F	Jaundice	CD99		Metastatic—primary ES in the right hemithorax invading mediastinum	Head	N/A	Partial pancreatic resection	N/A	[12]
Mulligan et al. (1997)	26	M	None	PAS		Metastatic—primary tumor in the left femur and concurrent mass found in the pancreas	Body and tail	N/A	RAD and chemotherapy	15 mos; recurrence, DOD	[13]
Bose et al. (2012)	31	F	Gallstone pancreatitis	CD99 and VIM	EWSR1 gene at 22q12 (FISH)	Primary ES in the pancreas	Posterior junction of the body and tail	3	Distal pancreatectomy, splenectomy, and VAC with alternating IE	18 mos; NED	[14]
Kent et al. (2018)	72	F	Jaundice	CD99 and PAS granules	N/A	Primary	Papilla of Vater	3 × 2 × 1.5	Whipple	2 mos; DOD	[15]
Komforti et al. (2018)	39	M	Abdominal pain	CD99, chromogranin, and synaptophysin	EWSR1-FLI1	Primary	Pancreatic body	8 × 5.8	Pain control	1 mo; AD	[16]
Liu et al. (2018)	36	M	Upper abdominal pain and jaundice	CD99, Ki-67, VIM, β-catenin, alpha-1-antichymotrypsin, and S-100	N/A	Primary	Head	6.3 × 3.6 × 4.8	Roux-en-Y choledochojejunostomy, refused RAD and CHE	1 mo; liver metastasis, 2 mos; DOD	[17]
Golhar et al. (2017)	17	F	Jaundice and itching	CD99 and cytokeratin AE1/AE3		Primary	Pancreatic head and uncinate process	5.6 × 7.4	Pancreotoduodectomy, vincristine, cyclophosphamide, and doxorubicin	2 wks; AWD	[18]
Saif et al. (2017)	38	F	Abdominal pain	CD99	t(11;22), (q24; q12)	Primary	Body and tail of the pancreas	8 × 10	Distal pancreatectomy, IE, and VAC	6 mos; AWD	[19]
Teixeira et al. (2015)	28	F	Epigastric pain, jaundice, and pruritus	CD99, VIM, automated CKM, and CD56		Primary	Head and body	13 × 9 × 13	Gastroduodenopancreatectomy	NED postop	[20]
Changal et al. (2014)	60	M	Epigastric pain	CD99, NSE, FLI-1, synaptophysin, and VIM	t(11;22) (q24; q12) via FISH	Primary	Head	3.1 × 2.2	VIDE × 3	3 mos; AWD	[21]
Dias et al. (2013)	25	F	Abdominal pain	CD99 and cytokeratin 8	t(11;22) (q24; q12) translocation	Primary	Head	4.2 × 4.0	Pancreaticoduodenectomy, VAI, and VDC	8 mos; DOD	[22]
Jayant et al. (2013)	20	F	LUQ pain	CD99		Primary	Body and tail	8 × 10	Distal pancreatectomy and splenectomy, VAC, and RAD	24 mos; DOD	[23]
Mao et al. (2013)	13	F	Abdominal pain, anorexia, polyuria, and polydipsia	CD99 and NSE	EWSR1-FLI1 fusion	Primary	Head	9 × 11 × 17	Surgical resection of uncinate process, RAD, VAC, and MAID	9 mos; metastasis to the liver, 36 mos; metastasis to the right kidney; 41 mos; metastasis to the 3rd vertebra, DOD	[24]
Rao et al. (2012)	47	F	Abdominal pain	CD99		Primary	Body and tail	12 × 15	Distal pancreatectomy and splenectomy, IE, and VAC	NED after 2 cycles	[25]
Reilly et al. (2013)	23	M	Upper abdominal pain, nausea	CD99, cytokeratin AE1/AE3, Cam 5.2, CK19, VIM, BCL-2, NSE, EMA, cyclin D1, and PAS granules	EWSR1-FLT1	Primary	Distal body and proximal tail	5.8 × 5.4	Distal pancreatectomy and splenectomy	Unknown	[26]
Jing et al. (2011)	24	F	None	N/A	N/A	Primary	Head	10 × 10 × 8	Resection and CHE	N/A	[27]
Maxwell et al. (2011)	11	M	Fatigue	CD99, broad-spectrum cytokeratin, and VIM	EWSR1-ERG fusion (RT-PCR)	Primary	Head	9.8 × 7.8 × 6.4	VDC alternating with IE, Whipple	3 mos; AWD	[28]
Wakao et al. (2011)	3	M	Upper abdominal pain and swelling	N/A	N/A	N/A	Head	N/A	Pancreatoduodenectomy, VDC/IE, AST, and RAD	8 mos; AWD	[29]
Doi et al. (2009)	37	M	Jaundice	CD99, VIM, CD56, and NSE	EWSR1 rearrangement at 22q12	Primary	Head	6	Pancreatoduodecotomy, hepatic resection, VDC × 7 alternating with IE, RAD	12 mos; NED	[30]
Menon et al. (2006)	8	F	Abdominal pain, menstrual bleeding, breast development, and pubic hair	CD99	N/A	Primary	Body	10 × 6 × 10	Cholecystectomy, doxorubicin, and RAD	CR postop, 19; death 2/2 to heart failure	[31]
Schutte et al. (2006)	2	F	Vaginal bleeding, breast development, and pubic hair	Synaptophysin, CD99, chromogranin A, S-100, VIM, estrogen receptor, progesterone receptor, inhibin, and epithelial membrane antigen	N/A	Primary	Body	6 × 4	Distal pancreatectomy, vincristine, adriamycin, and cyclophosphamide (VDC) alternating with cisplatin and etoposide	12 mos; NED	[32]
Welsch et al. (2006)	33	M	Abdominal pain and vomiting	CD99, VIM, NSE, cytokeratin, EMA, synaptophysin, CD56, and CD117	Translocation involving EWS gene at 22q12	Primary	Body and tail	18 × 18 × 16	Partial gastric resection, left pancreatic resection, splenectomy, VIDE × 6, VAI × 1, and AST	1 mo; metastases to the liver, 12 mos, NED	[33]
Perek et al. (2003)	31	M	RUQ pain	CD99, VIM, Leu 7, and synaptophysin		Primary	Head and body	10 × 12	Whipple, AI × 6, ifosphamide × 6, doxorubicin, and docetaxel	4 mos; local recurrence, 24/36 mos; metastasis to the right lung, 50 mos; DOD	[34]
Takeuchi et al. (2003)	10	F	Upper abdominal pain	N/A	N/A	N/A	Body	N/A	CHE and AST	3 mos; DOD	[35]
Gemechu et al. (2002)	17	M	Abdominal swelling	Synaptophysin and chromogranin		Primary	Body	N/A	N/A	36 mos; AWD	[36]
Movahedi-Lankarani et al. (2002)	17	M	Abdominal pain and jaundice	CD99, cytokeratin AE1/AE3, and NSE	t(11;22) (q24; q12)	Primary	Head	9.0	Whipple and CHE	33 mos; NED	[37]
	20	M	Abdominal pain and jaundice	CD99, cytokeratin AE1/AE3, NSE, and epithelial membrane antigen	t(11;22) (q24; q12)	Primary	Head	3.5	Whipple	27 mos; AWD	[37]
	25	F	Abdominal pain	CD99 and cytokeratin AE1/AE3	N/A	Primary	Head	N/A		N/A	[37]
	21	F	Abdominal pain	CD99, cytokeratin AE1/AE3, NSE, chromogranin, synaptophysin, and epithelial membrane antigen	t(11;22) (q24; q12)	Primary	Head	N/A	Whipple	Death due to OR complication	[37]
	25	F	Abdominal pain and jaundice	CD99 and NSE		Primary	Head	8.0		N/A	[37]
	13	M	Abdominal pain	CD99 and NSE	N/A	Primary	Head	6.0	VDC	43 mos; NED	[37]
	6	M	Abdominal pain and jaundice	CD99, cytokeratin AE1/AE3, NSE, and synaptophysin	t(11;22) (q24; q12)	Primary	Head	3.5	Whipple and VDC	48 mos; recurrence, DOD	[37]
O'Sullivan et al. (2001)	20	M	None	CD99, VIM cytokeratin, and membrane antigen	EWS-7 to Fli1-5	Primary	Head	3.5	Pancreatoduodenectomy, CHE, and RAD	Metastasis to the lungs at 30 mos, AWD	[38]
Bulchmann et al. (2000)	6	F	Paleness, dizziness, and fatigue	CD99, pancytokeratin, NSE, gamma-enolase and squamoid corpuscles, and S-100	Loss of cosmids F7 and E4 distal EWSR1 breakpoint	Primary	Head	4.0 × 5.4 × 3.0	Whipple and colon segmentectomy (refused CHE)	6 mos; recurrence, DOD	[39]
Luttges et al. (1997)	13	F	Dyspepsia and exophthalmos	CD99 and NSE		N/A	Body and tail	22 × 8 × 10	Whipple and CHE	2 mos; AWD	[40]
	31	M	Upper abdominal pain	CD99, NSE, and vimentin		N/A	Body	N/A	CHE and resection	N/A	[40]
Danner et al. (1994)	17	M	Upper abdominal pain	Cytokeratin, NSE, and 12E7	t(11;12) (q24; q12)	Primary	Head	N/A	Pancreatoduodenectomy, VDC/cisplatin+etoposide, and RAD	33 mos; AWD	[41]

AI = adriamycin (doxorubicin)/ifosfamide; IE = ifosphamide and etoposide; VAC = vincristine, adriamycin, and cyclophosphamide; VDC = vincristine, doxorubicin and cyclophosphamide; RAD = radiation; VIDE = vincristine, ifosfamide, doxorubicin, and etoposide; VAI = vincristine, actinomycin D and ifosfamide; CR = complete remission; AST = autologous stem transplant; MAID = doxorubicin/dacarvazine/ifosfamide; AWD = alive without disease; DOD = died of disease; DOC = died of complications; AD = alive with disease; NED = no evidence of disease; CHE = chemotherapy (specific agents unknown); NSE = neuronspecific antigen; VIM = vimentin.