Ewing's sarcoma family tumors mimicking primary central nervous system neoplasms

Diagnostic pitfall in the diagnosis of mesenchymal chondrosarcoma arising in the central nervous system

“…6 Second, most reported primary CNS Ewing sarcoma/ PNET are extra-axial, dural-based masses. 16 Histologically, the small round cell appearance in Ewing sarcoma/PNET shows a striking histological similarity to mesenchymal chondrosarcoma, both exhibiting sheets of small round [19] 44/F Parietal Dura GTR Died, 9 years (9 recurrences) 2 [20] 11/M Frontal Parenchyma GTR Alive, 5 months 3 [21] 48/F Frontal Parenchyma R Died, 3 days 4 [22] 18/F Frontal Dura STR Died, 16 months (4 recurrences) 5 [23] 51/F Parietal Dura STR, RT Died, 11 months (1 recurrence, metastasis) 6 [24] 13/M Frontoparietal Dura GTR Alive, 1.5 years 7 [25] 19/M Parietal Dura R, RT Alive, 1 year (1 recurrence) 8 [26] 19/M Occipital Dura GTR Unknown 9 [9] 7/M Temporal Dura R Alive, 7 years (1 recurrence) 10 [9] 17/F Frontal Dura R Died, 2 years 11 [9] 40/M Parietal Dura R Died, 5 days 12 [27] 11/M Parietooccipital Dura STR Alive, 8 years (1 recurrence, metastasis) 13 [28] 33/M Frontal Dura R Alive, 3 years (2 recurrences) 14 [28] 26/F Frontal Dura GTR Died, 3 days 15 [28] 23/F Parietal Dura R, RT Alive, 4.5 years 16 [28] 26/F Cerebellar Parenchyma R Alive, 2.5 years 17 [29] 11/F Biparietal Dura GTR, RT Died, 18 years 18 [30] 22/M Cerebellar Parenchyma R Died, 4 months (1 recurrence) 19 [31] 19/M Parietal Dura R, RT Alive, 12 months (1 recurrence) 20 [31] 21/F Frontal Dura R Died, 7 years (metastasis) 21 [32] 12/F Frontal Parenchyma GTR Died, 5 months (1 recurrence) 22 [33] 61/F Parietal Dura R, RT Alive, unknown 23 [34] 6/F Thalamus Parenchyma None Died, 8 years 24 [35] 11/F Parietal Parenchyma R, RT Died, 1.5 years (1 recurrence) 25 [36] 13/F Frontoparietal Dura GTR Alive, 3 years (1 recurrence) 26 [37] 11 months/M Frontal Dura R, RT Died, unknown 27 [37] 12/F Frontal Dura R, CT, RT Died, unknown (1 recurrence) 28 [38] 5/M Frontal Dura GTR, RT Alive, 14 months 29 [38] 7/F Sphenoid ridge Dura STR D...…”

“…However, medulloblastoma was thought to be unlikely in our patient due to the dural-based location, presence of glycogen-rich cytoplasm and CD99 positivity. 16,17 Hemangiopericytoma is similarly meningeal-based, and typically displays characteristic staghorn vessels, which may be seen in mesenchymal chondrosarcoma. 17 However, the absence of staghorn vessels and the presence of positive PAS staining in our case made this diagnosis unlikely.…”

Primary Ewing’s sarcoma affecting the central nervous system: a review and proposed prognostic considerations

“…63 Although patients with neurological deficit secondary to external spinal cord compression have shown improvement following administration of dexamethasone, palliative radiation and adjuvant chemotherapy, the role of urgent surgical decompression cannot be overemphasized. 64 Several series utilizing a strategy of radiation and chemotherapy without surgical resection for osseous ES showed inferior 5-year survival (28-62%) compared with multimodality approach including aggressive en bloc spondylectomy (74-92%). 61,65 Special considerations include the risk of spine destabilization and progressive deformity following extensive laminectomy and resection.…”

“…75 However, with radical resection, chemotherapy and radiation, long-term remission is common. 29,35,64 Due to the rarity of diagnosis, there is no recognized risk-stratification system for ES affecting the CNS. Predictors of poor outcome for all ES patients at diagnosis include the size of the lesion, presence of metastatic disease, pelvic location, high serum LDH, and age greater than 17 years of age, ongoing symptoms greater than 6 months duration.…”

Patterns of failure and optimal radiotherapy target volumes in primary intradural extramedullary Ewing sarcoma

“…ES tumors also have common translocations involving chromosome 22, most notably t(11;22)(q24;q12). This mutation has not been identified in cPNET [2]. …”