Bone Marrow Findings in Patients With Ewing Sarcoma/Primitive Neuroectodermal Tumor

Abstract Ewing sarcoma/primitive neuroectodermal tumor is classically a tumor of the soft tissue or bone in children and young adults, but several cases have been described in patients of all ages. Within the last decade, the clinicopathologic spectrum of Ewing sarcoma/primitive neuroectodermal tumor has been markedly expanded by recognition that the tumor may also have a visceral origin. We describe a case of primitive neuroectodermal tumor arising in the stomach of a 66-year-old woman. The neoplasm was excised using a radical surgical procedure. Microscopically, the tumor was made up of solid nests and sheets of round cells. Immunohistochemically, the tumor cells showed immunoreactivity for CD99, S100, neuron-specific enolase, and vimentin. A multiplex real-time polymerase chain reaction assay detected an EWS-ERG fusion. To our knowledge, this is the first description of a primitive neuroectodermal tumor arising in the stomach.

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Primary Ewing sarcoma/primitive neuroectodermal tumor of the lung

Autopsy and Case Reports ◽

10.4322/acr.2020.199 ◽

2020 ◽

Vol 10 (3) ◽

Author(s):

Devika Gupta ◽

Tathagata Chatterjee ◽

Rohit Tewari ◽

Arti Trehan ◽

Anuradha Ahuja

Keyword(s):

Ewing Sarcoma ◽

Primitive Neuroectodermal Tumor ◽

Neuroectodermal Tumor

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Ewing Sarcoma/Primitive Neuroectodermal Tumor of the Chest Wall

Transactions of the Meeting of the American Surgical Association ◽

10.1097/01.sla.0000089857.45191.52 ◽

2003 ◽

Vol 121 ◽

pp. 256-261 ◽

Cited By ~ 5

Author(s):

Robert C. Shamberger ◽

Michael P. LaQuaglia ◽

Mark C. Gebhardt ◽

James R. Neff ◽

Nancy J. Tarbell ◽

...

Keyword(s):

Chest Wall ◽

Ewing Sarcoma ◽

Primitive Neuroectodermal Tumor ◽

Neuroectodermal Tumor

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PRIMITIVE NEUROECTODERMAL TUMOR (PNET)/EWING SARCOMA (ES)

Atlas of Head and Neck Pathology ◽

10.1016/b978-0-7216-9788-8.50049-6 ◽

2008 ◽

pp. 1125-1133

Keyword(s):

Ewing Sarcoma ◽

Primitive Neuroectodermal Tumor ◽

Neuroectodermal Tumor

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Superficial Ewing Sarcoma/Primitive Neuroectodermal Tumor

Diagnostic Pathology: Neoplastic Dermatopathology ◽

10.1016/b978-0-323-44310-4.50160-3 ◽

2017 ◽

pp. 524-527

Keyword(s):

Ewing Sarcoma ◽

Primitive Neuroectodermal Tumor ◽

Neuroectodermal Tumor

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Detection of circulating tumor cells in patients with Ewing's sarcoma and peripheral primitive neuroectodermal tumor.

Journal of Clinical Oncology ◽

10.1200/jco.1997.15.2.583 ◽

1997 ◽

Vol 15 (2) ◽

pp. 583-588 ◽

Cited By ~ 86

Author(s):

D C West ◽

H E Grier ◽

M M Swallow ◽

G D Demetri ◽

L Granowetter ◽

...

Keyword(s):

Bone Marrow ◽

Tumor Cells ◽

Primitive Neuroectodermal Tumor ◽

Ewing’S Sarcoma ◽

Ewing's Sarcoma ◽

Residual Disease ◽

Neuroectodermal Tumor ◽

Peripheral Primitive Neuroectodermal Tumor ◽

Rt Pcr ◽

Relapsed Disease

PURPOSE To determine the feasibility of detecting Ewing's sarcoma (ES) or peripheral primitive neuroectodermal tumor (PNET) through a reverse-transcriptase polymerase chain reaction (RT-PCR) of the t(11;22)(q24;q12) fusion transcript in blood and bone marrow samples from patients with these neoplasms. PATIENTS AND METHODS Peripheral-blood (PB) and/or bone marrow aspirate (BM) samples were obtained from 28 patients with ES or PNET at initial presentation or at relapse. Patients were divided into two groups: newly diagnosed patients with nonmetastatic disease and those with metastatic/relapsed disease. RNA was extracted from fractionated BM and PB samples, and RT-PCR was performed for the EWS/HumFLI1 fusion mRNA was transcribed across the t(11;22) breakpoint. RESULTS Among the 16 patients with nonmetastatic disease, three of 16 were RT-PCR positive for EWS/HumFLI1 RNA in BM and three of 10 were positive in PB. The total number of nonmetastatic patients who were positive in either PB or BM was four of 16 (25%). Among patients with metastatic/relapsed disease, two of six were positive in BM and five of 10 were positive in PB. The total fraction of patients with metastatic/relapsed disease that was positive in either BM or PB was six of 12 (50%). CONCLUSION In this study, we show that it is possible to amplify the EWS/HumFLI1 RNA by RT-PCR from the BM and PB of a subset of patients with both nonmetastatic and metastatic ES or PNET, which implies that occult tumor cells are present at these sites. The true biologic and clinical meaning of this information is unknown. However, it does suggest a possible application of RT-PCR for the monitoring of residual disease in patients who are undergoing therapy for ES or PNET. This approach may permit early identification of patients who may benefit from alternative therapy or who may be spared possible overtreatment.

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