Table 2 Clinicopathological and genetic features of current case and previously reported cases of primary pulmonary myxoid sarcoma

1(3)

27

F

EBC+

4

Ill-defined nodules of myxoid stroma, interweaving cords of small uniform, round or slightly elongated cells with eosinophilic cytoplasm, occasional mitoses.

Vimentin+, CK-, S100-, desmin-, SMA-, CD34-.

ND

Surgery

NEOD after 36

2(3)

43

F

EBC+

13

Ill-defined nodules of myxoid stroma, interweaving cords of small uniform, round or slightly elongated cells with eosinophilic cytoplasm, occasional mitoses.

Vimentin+, CK-, S100-, desmin-, SMA-, CD34-.

ND

Surgery

NEOD after 6

3(2)

27

F

EBC+

4

Well circumscribed, lobulated, reticular network with delicate lacelike strands and cords of cells within prominent myxoid stroma, tumor cells showed no or minimal atypia.

CK-, S100-, desmin-.

EWSR1-CREB1 fusion

Surgery

NEOD after 180

4(2)

33

F

EBC+

3.5

Lobulated, reticular network with delicate lacelike strands and cords of cells within prominent myxoid stroma, tumor cells showed mild to moderate atypia.

CK-, S100-, desmin-.

EWSR1-CREB1 fusion

Surgery

NEOD after 144

5(2)

45

F

EBC+

1.5

Circumscribed, lobulated, cellular sheets or patternless, tumor cells showed mild to moderate atypia.

S100 focal+, CK-, desmin-, p63-.

Negative

Surgery

NEOD after 12

6(2)

36

F

NR

NR

Circumscribed with fibrous pseudocapsule. Reticular network with delicate lacelike strands and cords of cells within prominent myxoid stroma, tumor cells showed minimal atypia.

CK-, EMA-, TTF1-, S100-, desmin-.

Negative

Surgery

DOD with brain metastases a few months after diagnosis

7(2)

32

F

EBC+

NR

Lobulated. Reticular network with delicate lacelike strands and cords of cells within prominent myxoid stroma, tumor cells showed moderate atypia.

CK-, EMA-,S100-, desmin-.

EWSR1-CREB1 fusion

Surgery

NR

8(2)

28

M

EBC+

2.8

Infiltrative and lobulated, cellular sheets or patternless, tumor cells showed mild to moderate atypia,

EMA weak+, CK-, TTF1-, S100-, HMB45-, melan A-, desmin-.

EWSR1-CREB1 fusion

Surgery

Left renal metastasis, alive and well after 3 years.

9(2)

67

M

EBC+

2.8

Well circumscribed and lobulated, reticular network with delicate lacelike strands and cords of cells within a prominent myxoid stroma., tumor cells showed minimal atypia.

EMA weak+, CK-, TTF-1-, S100-, desmin-.

EWSR1-CREB1 fusion

Surgery

NR

10(2)

68

F

EBC+

2.0

Well circumscribed and lobulated, reticular network with delicate lacelike strands and cords of cells within a prominent myxoid stroma, tumor cells showed moderate to marked atypia.

EMA weak+, CK-, p63-, TTF-1-, S100-, desmin-.

Negative

Surgery

NR

11(2)

63

F

EBC+

NR

Lobulated, cellular sheets or patternless, tunor cells showed mild to minimal atypia.

EMA weak +, CK-, TTF-1-, S100-, HMB45-, melan A-, desmin-.

EWSR1-CREB1 fusion

Surgery

NEOD after 48

12(2)

51

M

NR

2.0

Well circumscribed and lobulated, reticular network with delicate lacelike strands and cords of cells within a prominent myxoid stroma, tumor cells showed mild to moderate atypia.

NR

EWSR1-CREB1 fusion

Surgery

NR

13(5)

31

M

EBC+

2.7

Well circumscribed, reticular cords of oval, short spindle or polygonal cells with mild atypia, rare mitotic figures, an abundant myxoid stroma, scattered lymphoplasmacytic infiltrates.

Vimentin+, EMA focal+, CK-,

TTF-1-, napsin A-, S-100-, CD34-, desmin-, SMA-, CD10-, p63-, calponin- caldesmon-, c-kit-,

HMB-45-, synaptophysin-, GFAP-

EWSR1-CREB1 fusion

Surgery

NEOD after 68

14(6)

66

F

EBC+

4

Polygonal to spindled cells, reticular network with delicate lacelike strands and cords of cells within a prominent myxoid stroma, tumor cells showed mild atypia.

EMA focal+, CK-, p63-, S100-, desmin-.

EWSR1-CREB1 fusion

Surgery

NR

15(6)

28

M

NR

8.5

Lobulated, biphasic, ~ 40% composed of myxoid pools, exuberant fibroinflammatory reaction with confluent plasma cells, tumor cells showed moderate atypia.

Desmin+, EMA focal+, CK-, p63-. S100-.

Negative

Surgery

NR

16(6)

28

M

EBC+

6

Infiltrative, focal necrosis and inflammation, tumor cells showed severe atypia.

EMA focal+, CK-, p63-, S100-.

EWSR1 rearrangement,. but not CREB1

Surgery

NR

17(7)

26

M

EBC+

9

Multinodular, reticular network with delicate lacelike strands and cords of cells within prominent myxoid stroma, tumor cells showed mild to moderate atypia.

Vimentin+, EMA focal+, CD99 focal weak+, SMA-, desmin-, caldesmon-H-, calponin-, S100-, CK-, CD31-, CD34-, p63-, CD56-, synaptophysin-.

EWSR1-CREB1 fusion

Surgery

NEOD after 19

18(7)

49

F

EBC-

4

Multinodular, reticular network with delicate lacelike strands and cords of cells within prominent myxoid stroma, tumor cells showed mild to moderate atypia.

Vimentin+, EMA focal+, CD99 focal weak+, SMA focal+, desmin-, caldesmon-H, calponin-, S100-, CK-, CD31-, CD34-, p63-, CD56-, synaptophysin-.

EWSR1-CREB1 fusion

Surgery

NEOD after 117

19(7)

54

F

EBC+

4.5

Multinodular, reticular network with delicate lacelike strands and cords of cells within prominent myxoid stroma, tumor cells showed moderate atypia.

Vimentin+, EMA focal+,

CD99 focal+, SMA-, desmin-, caldesmon-H-, calponin-, S100-, CK-, CD31-, CD34-, p63-, CD56-, synaptophysin-.

EWSR1-CREB1 fusion

Surgery

NEOD after 152

20(7)

65

M

EBC+

13

Multinodular, reticular network with delicate lacelike strands and cords of cells within prominent myxoid stroma, tumor cells showed mild atypia.

Vimentin+, EMA focal+,

CD99 focal+, SMA-, desmin-, caldesmon-H-, calponin-, S100-, CK-, CD31-, CD34-, p63-, CD56-, synaptophysin-.

EWSR1-CREB1 fusion

Surgery

Metastasis to contralateral lung, NEOD 72 months after removal of metastasis.

21(8)

29

F

EBC-

3

Well circumscribed, short spindle, ovoid or stellate cells in reticular network, myxoid stroma, lymphoplasmacytic infiltration, tumor cells showed mild atypia.

Vimentin+, EMA+. SMA-, SMMHC-, calretinin-, TTF-1-, CK-, p63-, S-100-, CD34-, CD56-.

EWSR1-CREB1 fusion

Surgery

NEOD after 17

22(4)

80

F

EBC+

NR

Multinodular, spindle cells arranged in reticular pattern within prominent myxoid stroma, tumor cells showed moderately atypia

Vimentin+, EMA focal+. CK-, S100-, HMB45-, CD31-, CD34-, SMA-, caldesmon-H-, desmin-, GFAP-.

EWSR1-CREB1 fusion

Surgery

NEOD after 36.

23(9)

32

F

NR

3.5

Well-delineated lobulated, anastomosing cords and small nests of epithelioid cells admixed with stellate cells in chondromyxoid matrix.

Vimentin+, CD68 weak+, CD163 weak+, synaptophysin weak+. CK-, EMA-, calponin-, GFAP-, SMA-, desmin-, caldesmon-H-, S-100-, HMB-45-, CD34-, CD31-, chromogranin-.

EWSR1-CREB1 fusion

Surgery

NEOD after 96.

24(10)

21

F

PA/EBC-

NR

Polypoid tumor, trabecular networks, rare solid areas, tumor cells showed oval nuclei and eosinophilic cytoplasm,

CK weak+, SMA+, INI1-, EMA-, S100-, desmin-, ERG-, MDM2-, CDK4-.

EWSR1-CREB1 fusion

Surgery

NEOD after 38

25(11)

48

M

NR

14

Bland looking medium-sized oval to round epithelioid cells arranged in prominent reticular and microcystic lace-like chordoid pattern in highly myxoid stroma.

Vimentin+, CD10 focal+、EMA focal+, CK-, TTF-1-, ERG-, CD31-, p63-, desmin-, SMA-, S100-, CD34-, CD30- , MUC4-, TLE1-, STAT6-.

Negative

Surgery

NEOD after 23

26 (our case)

45

F

EBC-

2.1

Well circumscribed, multinodular, reticular network of delicate lace-like cellular strands and cords in abundant myxoid stroma, chondrocyte or physaliferous-like tumor cells with mild atypia.

Vimentin+, EMA+, CK-, TTF-1-, CAM5.2-, S-100-, calponin-, SMA-, desmin-, ALK-, CD31-, CD34-.

EWSR1-CREB1 fusion

Surgery

NEOD after 38