Table 1.
Previously reported cases of malignant gastrointestinal neuroectodermal tumor
| Report | Age (years)/ Sex | Location | Morphology | S100 | HMB-45 and Melan A | EM | Genetic finding | Outcome | Additional findings | |
|---|---|---|---|---|---|---|---|---|---|---|
| 1 | Zambrano et al. [2] | 15/F | Small intestine | Oval, spindled | + | - | No melanosomes | t(12;22) (q13;q12) | DOD at 16 months | OLGC |
| 2 | 21/F | Small intestine | Oval, spindled | + | - | ND | ND | DOD at 12 months | OLGC | |
| 3 | 35/F | Small intestine | Oval, spindled | + | - | No melanosomes | ND | LN and liver metastasis at 12 months | OLGC | |
| 4 | 37/F | Small intestine | Oval, spindled | + | - | ND | NA | OLGC | ||
| 5 | 13/M | Stomach | Oval, spindled | + | - | No melanosomes | ND | recurrences at 12 months | OLGC | |
| 6 | 32/M | Small intestine | Oval, spindled | + | - | ND | ND | LN mets | OLGC | |
| 7 | Stockman et al. [1]a | 30/F | Jejunum | Epithelioid | + | - | Dense-core granules | EWSR1-ATF1 | AWD at 21 months | |
| 8 | 35/M | Jejunum | Epithelioid | + | - | Dense-core granules | EWSR1-ATF1 | DOD at 18 months | ||
| 9 | 33/M | Ileum | Epithelioid | + | - | Dense-core granules | EWSR1-CREB1 | AWD at 1.5 months | ||
| 10 | 50/F | Stomach | Epithelioid | + | - | Dense-core granules | EWSR1-ATF1 | AWD at 24 months | ||
| 11 | 20/F | Small intestine | Spindled | + | - | Dense-core granules | EWSR1 | NED at 20 months | ||
| 12 | 52/M | Ileum | Epithelioid | + | - | ND | - | DOD at 22 months | ||
| 13 | 46/M | Stomach | Epithelioid | + | - | ND | EWSR1 | NA | ||
| 14 | 34/F | Stomach | Epithelioid, Spindled | + | - | ND | EWSR1-ATF1 | DOD at 19 months | ||
| 15 | 37/F | Ileum | Epithelioid | + | ND | ND | ND | NA | ||
| 16 | 77/F | Colon | Epithelioid, Spindled | + | - | ND | EWSR1-ATF1 | DOD at 106 months | ||
| 17 | 31/M | Colon | Epithelioid | + | - | ND | - | DOD at 3 months | ||
| 18 | 17/M | Small intestine | Epithelioid | + | - | ND | EWSR1 | NA | ||
| 19 | 60/M | Iluem | Epithelioid | + | - | ND | EWSR1-ATF1 | AWD at 36 months | ||
| 20 | 60/F | Jejunum | Epithelioid, Spindled | + | - | ND | EWSR1-CREB1 | NED at 41 months | ||
| 21 | 56/M | Stomach | Epithelioid | + | - | ND | EWSR1-CREB1 | NA | ||
| 22 | 28/F | Small intestine | Epithelioid | + | - | ND | EWSR1 | DOD at 23 months | ||
| 23 | Insabato et al. [6] | 29/M | Stomach | Epithelioid, spindled | + | - | No melanosomes | EWSR1 | AWD at 74 months | Hx of Ewing sarcoma, FLI-1+ |
| 24 | Zhao et al. [7] | 33/F | Ileum | Epithelioid | + | - | EWSR1 | NA | ||
| 25 | Kong et al. [5] | 17/M | Stomach | Spindled, round | + | - | ND | EWSR1 | NED at 10 months | OLGC |
| 26 | Huang et al. [3] | 40/M | Stomach | Spindled, oval | + | - | ND | ND | LN mets at diagnosis | OLGC |
| 27 | Thway et al. [8] | 33/M | Small intestine | Epithelioid, spindled | + | - | ND | EWSR1-CREB1 | DOD at 7 months | Hx of hepato-blastoma |
| 28 | Friedrichs et al. [4] | 41/M | Small intestine | Epithelioid | + | - | ND | t(12;22) (q13;q12) | Liver mets at 6 months | OLGC |
| 29 | Boland et al. [9] | 46/F | Stomach | Epithelioid | + | - | ND | EWSR1-ATF1 | NA | Oncocytic change |
AWD alive with disease, DOD dead of disease, EM electron microscopy, Hx history, mets, metastasis, NA not available, NED no evidence of disease, ND not done, OLGC osteoclast-like giant cells
aEight cases described by Stockman et al. showed osteoclast-like giant cells