Table 1.
Clinicopathologic features of case of ALES
| Site | Age/Sex | Immunoprofile | Genetics | Treatment for primary | Additional treatment | Outcome | ||||||||
|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
| Cytokeratins | p63/p40 | S100 | CD99 | FEV::ETS | EWSR1/FUS rearrangement | Recurrence (mo) | Metastasis (site, mo) | Status | Length of follow-up (mo) | |||||
| 1* | Nasal cavity | 51/F | AE1/3+ CAM5.2+ CK5/6+ |
+/ | + | + | EWSR1::FLI1 | chemotherapy (VDC/IE) and radiation at diagnosis | Recurrence treated with surgery and chemotherapy with temozolamide/iriniotecan |
Y (138) | N | AWOD | 154 | |
| 2 | Neck | 59/M | pankeratin+ | /+ | EWSR1+ | N | N | AWOD | 61 | |||||
| 3 | Lung metastasis from submandibular primary | 26/M | AE1/3+ CAM5.2+ |
/ | - | + | EWSR1::FLI1 | Resection (regional lymph nodes) | VATS resection for lung metastasis and chemotherapy (VDC/IE x 17 cycles) | N | Y (lung, 4) | AWD | 40 | |
| 4* | Parotid | 72/M | AE1/3+ | /+ | - | + | EWSR1+ | Surgery, additional treatment pending | N | N | AWOD | 1 | ||
| 5 | Thyroid gland | 23/M | AE1/3+ | /+ | - | + | EWSR1+ | Surgery, radiation, and chemotherapy (VDC/IE) | Chemo after recurrence (irenotecan, temozolamide and vincristine) | N | Y (spine, 0; femur, 12) | Dead of complications of chemotherapy (Klebsiella pneumoniae pneumonia, sepsis, and ARDS) (15) | 15 | |
| 6* | Sinonasal | 37/F | pankeratin+ | /+ | + | + |
EWSR1+
FLI1+ |
Surgery | Recurrence with surgery, radiation, chemotherapy (docetaxel, carboplatin, capecitabine, methotrexate) | Y (24) | Y (dura, 46) | DWD (52) | 52 | |
| 7 | Gingiva | 20/M | CK5/6+ | +/ | - | weak | FUS::FLI1 | Mandibulectomy with neck dissection followed by concurrent radiation and chemotherapy (VDC/IE) | Metastasis treated with radiation | N | Y (scapula, 10) | AWOD | 16 | |
| 8* | Parotid | 32/M | AE1/3+ | /+ | - | + | EWSR1::FLI1 | EWSR1+ | surgery, radiation and chemotherapy (VDC/IE) | N | N | AWOD | 72 | |
| 9* | Parotid | 46/M | AE1/3+ | /+ | - | + | EWSR1+ | surgery, radiation, VDC/IE | N | N | AWOD | 58 | ||
| 10 | Submandibular gland | 69/M | AE1/3+ CAM5.2+ CK5/6+ |
/ | - | weak | EWSR1::FLI1 | Submandibular gland excision with neck dissection | Recurrence treated with chemotherapy (doxorubicin, ifosfamide and vincristine with mesna and growth factor support) and radiation | Y (3) | Y (lung, 17) | AWD | 17 | |
| 11 | Neck recurrence from mandible primary | 24/M | CK5/6+ 34BE12+ CK7- CK20- |
+/ | - | + | EWSR1::FLI1 | Radiation for mandibular primary | Resection and radiation for neck recurrence; Adjuvant cisplatin and proton for 2nd neck recurrence; After lung metastasis VAC ifosfamide/etoposide and Nivolumab | Y (22) | Y (regional nodes, 36; lung 61) | DOD (63) | 63 | |
| 12 | Hypopharynx | 61/M | AE1/3+ | /+ | - | + | EWSR1:FLI1 | Recent diagnosis | Recent diagnosis | Recent diagnosis | Recent diagnosis | 0 | ||
*
, previously reported; NP, not performed; +, positive; -, negative; chemo, chemotherapy; AWOD, alive without disease; NED, no evidence of disease; DOC, died of complications from chemotherapy; DWD, died with disease; DOD, died of disease; N/A, not available