Table 2.
Clinicopathological and genetic features of current case and previously reported cases of primary pulmonary myxoid sarcoma
| Case No. (Ref. No.) | Age | sex | EBC (+/−) | Size (cm) | Pathological features | IHC | Molecular genetics | Treatment | Follow-up (months) |
|---|---|---|---|---|---|---|---|---|---|
| 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 |
F female, M male, EBC+/− endobronchial component involved (+) or not involved (−). IHC immunohistochemical stains, ND not done, NR not reported, PA pulmonary artery, DOD died of disease, NEOD no evidence of disease, CK cytokeratin(s), EMA epithelial membrane antigen, SMA smooth muscle actin, SMMHC smooth muscle myosin heavy chain, TTF-1 thyroid transcription factor-1