Keratin-positive Ewing's sarcoma: An ultrastructural study of 12 cases

Amitabh Srivastava, Andrew Rosenberg, Martin Selig, Brian P. Rubin, G. Petur Nielsen

Research output: Contribution to journalArticle

25 Citations (Scopus)

Abstract

Ewing's sarcoma/primitive neuroectodermal tumor (EWS/PNET) is an aggressive neoplasm of bone and soft tissue. Histologically, it is characterized by the presence of small round blue cells, which usually express MIC-2 and FLI-1 immunohistochemically. The most specific feature for diagnosis, however, is cytogenetic or molecular evidence of a consistent abnormality, the t(11;22)(q24;q12), or variants thereof. The immunohistochemical expression of keratins in a significant proportion of these cases has been highlighted in several recent studies. The ultrastructural features of these keratin-positive tumors have not, however, been characterized in detail. In this study we analyzed the ultrastructural features of 12 well-documented EWS/PNETs that stained strongly for pankeratin by immunohistochemistry. Ultrastructurally, the tumor cells contained a few organelles, which included a small number of mitochondria, poorly developed Golgi complexes, free ribosomes, and inconspicuous rough-endoplasmic reticulum. Rudimentary cell junctions were seen in 2 tumors while prominent junctions were observed in the remaining 10. Five tumors contained intracytoplasmic filaments, and definite tonofibrils were identified in 2. Well-developed basal lamina around tumor cells were also demonstrated in 2 tumors. Follow-up information was available for all cases. Seven patients died of disease, 2 are alive with disease, and 3 have no current evidence of disease. The cohort includes 5 patients with a type-1 translocation, which has been associated with a better prognosis in some studies; 4 of these patients have died of their disease, and 1 is alive with recurrent disease. This study shows that keratin-positive EWS/PNETs have evidence of epithelial differentiation ultrastructurally, and may possibly represent a more aggressive subset of the EWS/PNET group of tumors.

Original languageEnglish
Pages (from-to)43-50
Number of pages8
JournalInternational Journal of Surgical Pathology
Volume13
Issue number1
StatePublished - Jan 1 2005
Externally publishedYes

Fingerprint

Ewing's Sarcoma
Keratins
Primitive Neuroectodermal Tumors
Neoplasms
Bone Tissue Neoplasms
Soft Tissue Neoplasms
Intercellular Junctions
Rough Endoplasmic Reticulum
Golgi Apparatus
Ribosomes
Basement Membrane
Cytogenetics
Organelles
Mitochondria
Immunohistochemistry

Keywords

  • Electron microscopy
  • Ewing's sarcoma
  • Keratin
  • Peripheral primitive neuroectodermal tumor
  • PNET
  • Ultrastructure

ASJC Scopus subject areas

  • Anatomy
  • Pathology and Forensic Medicine
  • Surgery

Cite this

Keratin-positive Ewing's sarcoma : An ultrastructural study of 12 cases. / Srivastava, Amitabh; Rosenberg, Andrew; Selig, Martin; Rubin, Brian P.; Nielsen, G. Petur.

In: International Journal of Surgical Pathology, Vol. 13, No. 1, 01.01.2005, p. 43-50.

Research output: Contribution to journalArticle

Srivastava, A, Rosenberg, A, Selig, M, Rubin, BP & Nielsen, GP 2005, 'Keratin-positive Ewing's sarcoma: An ultrastructural study of 12 cases', International Journal of Surgical Pathology, vol. 13, no. 1, pp. 43-50.
Srivastava, Amitabh ; Rosenberg, Andrew ; Selig, Martin ; Rubin, Brian P. ; Nielsen, G. Petur. / Keratin-positive Ewing's sarcoma : An ultrastructural study of 12 cases. In: International Journal of Surgical Pathology. 2005 ; Vol. 13, No. 1. pp. 43-50.
@article{2fadc522e2984d30abf4eeda5accb644,
title = "Keratin-positive Ewing's sarcoma: An ultrastructural study of 12 cases",
abstract = "Ewing's sarcoma/primitive neuroectodermal tumor (EWS/PNET) is an aggressive neoplasm of bone and soft tissue. Histologically, it is characterized by the presence of small round blue cells, which usually express MIC-2 and FLI-1 immunohistochemically. The most specific feature for diagnosis, however, is cytogenetic or molecular evidence of a consistent abnormality, the t(11;22)(q24;q12), or variants thereof. The immunohistochemical expression of keratins in a significant proportion of these cases has been highlighted in several recent studies. The ultrastructural features of these keratin-positive tumors have not, however, been characterized in detail. In this study we analyzed the ultrastructural features of 12 well-documented EWS/PNETs that stained strongly for pankeratin by immunohistochemistry. Ultrastructurally, the tumor cells contained a few organelles, which included a small number of mitochondria, poorly developed Golgi complexes, free ribosomes, and inconspicuous rough-endoplasmic reticulum. Rudimentary cell junctions were seen in 2 tumors while prominent junctions were observed in the remaining 10. Five tumors contained intracytoplasmic filaments, and definite tonofibrils were identified in 2. Well-developed basal lamina around tumor cells were also demonstrated in 2 tumors. Follow-up information was available for all cases. Seven patients died of disease, 2 are alive with disease, and 3 have no current evidence of disease. The cohort includes 5 patients with a type-1 translocation, which has been associated with a better prognosis in some studies; 4 of these patients have died of their disease, and 1 is alive with recurrent disease. This study shows that keratin-positive EWS/PNETs have evidence of epithelial differentiation ultrastructurally, and may possibly represent a more aggressive subset of the EWS/PNET group of tumors.",
keywords = "Electron microscopy, Ewing's sarcoma, Keratin, Peripheral primitive neuroectodermal tumor, PNET, Ultrastructure",
author = "Amitabh Srivastava and Andrew Rosenberg and Martin Selig and Rubin, {Brian P.} and Nielsen, {G. Petur}",
year = "2005",
month = "1",
day = "1",
language = "English",
volume = "13",
pages = "43--50",
journal = "International Journal of Surgical Pathology",
issn = "1066-8969",
publisher = "SAGE Publications Inc.",
number = "1",

}

TY - JOUR

T1 - Keratin-positive Ewing's sarcoma

T2 - An ultrastructural study of 12 cases

AU - Srivastava, Amitabh

AU - Rosenberg, Andrew

AU - Selig, Martin

AU - Rubin, Brian P.

AU - Nielsen, G. Petur

PY - 2005/1/1

Y1 - 2005/1/1

N2 - Ewing's sarcoma/primitive neuroectodermal tumor (EWS/PNET) is an aggressive neoplasm of bone and soft tissue. Histologically, it is characterized by the presence of small round blue cells, which usually express MIC-2 and FLI-1 immunohistochemically. The most specific feature for diagnosis, however, is cytogenetic or molecular evidence of a consistent abnormality, the t(11;22)(q24;q12), or variants thereof. The immunohistochemical expression of keratins in a significant proportion of these cases has been highlighted in several recent studies. The ultrastructural features of these keratin-positive tumors have not, however, been characterized in detail. In this study we analyzed the ultrastructural features of 12 well-documented EWS/PNETs that stained strongly for pankeratin by immunohistochemistry. Ultrastructurally, the tumor cells contained a few organelles, which included a small number of mitochondria, poorly developed Golgi complexes, free ribosomes, and inconspicuous rough-endoplasmic reticulum. Rudimentary cell junctions were seen in 2 tumors while prominent junctions were observed in the remaining 10. Five tumors contained intracytoplasmic filaments, and definite tonofibrils were identified in 2. Well-developed basal lamina around tumor cells were also demonstrated in 2 tumors. Follow-up information was available for all cases. Seven patients died of disease, 2 are alive with disease, and 3 have no current evidence of disease. The cohort includes 5 patients with a type-1 translocation, which has been associated with a better prognosis in some studies; 4 of these patients have died of their disease, and 1 is alive with recurrent disease. This study shows that keratin-positive EWS/PNETs have evidence of epithelial differentiation ultrastructurally, and may possibly represent a more aggressive subset of the EWS/PNET group of tumors.

AB - Ewing's sarcoma/primitive neuroectodermal tumor (EWS/PNET) is an aggressive neoplasm of bone and soft tissue. Histologically, it is characterized by the presence of small round blue cells, which usually express MIC-2 and FLI-1 immunohistochemically. The most specific feature for diagnosis, however, is cytogenetic or molecular evidence of a consistent abnormality, the t(11;22)(q24;q12), or variants thereof. The immunohistochemical expression of keratins in a significant proportion of these cases has been highlighted in several recent studies. The ultrastructural features of these keratin-positive tumors have not, however, been characterized in detail. In this study we analyzed the ultrastructural features of 12 well-documented EWS/PNETs that stained strongly for pankeratin by immunohistochemistry. Ultrastructurally, the tumor cells contained a few organelles, which included a small number of mitochondria, poorly developed Golgi complexes, free ribosomes, and inconspicuous rough-endoplasmic reticulum. Rudimentary cell junctions were seen in 2 tumors while prominent junctions were observed in the remaining 10. Five tumors contained intracytoplasmic filaments, and definite tonofibrils were identified in 2. Well-developed basal lamina around tumor cells were also demonstrated in 2 tumors. Follow-up information was available for all cases. Seven patients died of disease, 2 are alive with disease, and 3 have no current evidence of disease. The cohort includes 5 patients with a type-1 translocation, which has been associated with a better prognosis in some studies; 4 of these patients have died of their disease, and 1 is alive with recurrent disease. This study shows that keratin-positive EWS/PNETs have evidence of epithelial differentiation ultrastructurally, and may possibly represent a more aggressive subset of the EWS/PNET group of tumors.

KW - Electron microscopy

KW - Ewing's sarcoma

KW - Keratin

KW - Peripheral primitive neuroectodermal tumor

KW - PNET

KW - Ultrastructure

UR - http://www.scopus.com/inward/record.url?scp=13544273527&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=13544273527&partnerID=8YFLogxK

M3 - Article

C2 - 15735854

AN - SCOPUS:13544273527

VL - 13

SP - 43

EP - 50

JO - International Journal of Surgical Pathology

JF - International Journal of Surgical Pathology

SN - 1066-8969

IS - 1

ER -