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 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 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 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 Whipple 2 mos; DOD [15 ] Komforti et al. (2018) 39 M Abdominal pain CD99, chromogranin, and synaptophysin EWSR1-FLI1 Primary Pancreatic body 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 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 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 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 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 mos; AWD [21 ] Dias et al. (2013) 25 F Abdominal pain CD99 and cytokeratin 8 t(11;22) (q24; q12) translocation Primary Head Pancreaticoduodenectomy, VAI, and VDC 8 mos; DOD [22 ] Jayant et al. (2013) 20 F LUQ pain CD99 Primary Body and tail 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 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 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 Distal pancreatectomy and splenectomy Unknown [26 ] Jing et al. (2011) 24 F None N/A N/A Primary Head 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 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, 12 mos; NED [30 ] Menon et al. (2006) 8 F Abdominal pain, menstrual bleeding, breast development, and pubic hair CD99 N/A Primary Body 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 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 Partial gastric resection, left pancreatic resection, splenectomy, 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 Whipple, 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 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 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 ]
