Primitive neuroectodermal tumor of the orbit in adults: A case series

Weerawan Chokthaweesak, Christine C. Annunziata, Oday Alsheikh, John Ng, David Wilson, Atiya Mansoor, Vassil Kaimatchiev, Robert Weatherhead, Jack Rootman, Geva Mannor, Kanjana Leelapatranurak, Bobby S. Korn, Don O. Kikkawa

Research output: Contribution to journalArticle

12 Citations (Scopus)

Abstract

Purpose: To present the clinical, radiologic, and histopathologic features of orbital primitive neuroectodermal tumor (PNET) in 5 adult patients. Methods: Retrospective case series of 5 adult patients with orbital PNET. Orbitotomy was performed in all cases. The authors report clinical findings, radiologic features, histopathology, immunohistochemical analysis, management, and outcomes for 5 patients with orbital PNET. Results: Five adult patients presented with progressive unilateral proptosis and visual impairment. Common radiographic findings included a heterogeneous mass without associated destructive features, located in the superior and/or lateral orbit. Four cases demonstrated strong immunohistochemical staining for CD99 in a membranous pattern. One case required chromosomal analysis with fluorescence in situ hybridization to confirm the diagnosis. All patients received chemotherapy and/or orbital radiation with resolution of proptosis but no improvement of vision. One patient died of disease. Conclusions: To the authors' knowledge, this is the largest series of orbital PNET in adults. This tumor has an age demographic wider than previously believed and should be considered in the differential diagnosis of a hypercellular small round cell orbital tumor in both children and adults. Current treatment regimens are not standardized but typically use a similar approach to the treatment of Ewing sarcoma. Orbital PNET appears to have less propensity for metastasis compared with PNET in other locations. However, long-term aggressiveness remains to be proven.

Original languageEnglish (US)
Pages (from-to)173-179
Number of pages7
JournalOphthalmic Plastic and Reconstructive Surgery
Volume27
Issue number3
DOIs
StatePublished - May 2011

Fingerprint

Primitive Neuroectodermal Tumors
Orbit
Exophthalmos
Ewing's Sarcoma
Vision Disorders
Fluorescence In Situ Hybridization
Neoplasms
Differential Diagnosis
Demography
Radiation
Staining and Labeling
Neoplasm Metastasis
Drug Therapy
Therapeutics

ASJC Scopus subject areas

  • Ophthalmology
  • Surgery

Cite this

Primitive neuroectodermal tumor of the orbit in adults : A case series. / Chokthaweesak, Weerawan; Annunziata, Christine C.; Alsheikh, Oday; Ng, John; Wilson, David; Mansoor, Atiya; Kaimatchiev, Vassil; Weatherhead, Robert; Rootman, Jack; Mannor, Geva; Leelapatranurak, Kanjana; Korn, Bobby S.; Kikkawa, Don O.

In: Ophthalmic Plastic and Reconstructive Surgery, Vol. 27, No. 3, 05.2011, p. 173-179.

Research output: Contribution to journalArticle

Chokthaweesak, W, Annunziata, CC, Alsheikh, O, Ng, J, Wilson, D, Mansoor, A, Kaimatchiev, V, Weatherhead, R, Rootman, J, Mannor, G, Leelapatranurak, K, Korn, BS & Kikkawa, DO 2011, 'Primitive neuroectodermal tumor of the orbit in adults: A case series', Ophthalmic Plastic and Reconstructive Surgery, vol. 27, no. 3, pp. 173-179. https://doi.org/10.1097/IOP.0b013e3181f9df17
Chokthaweesak, Weerawan ; Annunziata, Christine C. ; Alsheikh, Oday ; Ng, John ; Wilson, David ; Mansoor, Atiya ; Kaimatchiev, Vassil ; Weatherhead, Robert ; Rootman, Jack ; Mannor, Geva ; Leelapatranurak, Kanjana ; Korn, Bobby S. ; Kikkawa, Don O. / Primitive neuroectodermal tumor of the orbit in adults : A case series. In: Ophthalmic Plastic and Reconstructive Surgery. 2011 ; Vol. 27, No. 3. pp. 173-179.
@article{568b39dd157a4d0d836a1be0ebb5b445,
title = "Primitive neuroectodermal tumor of the orbit in adults: A case series",
abstract = "Purpose: To present the clinical, radiologic, and histopathologic features of orbital primitive neuroectodermal tumor (PNET) in 5 adult patients. Methods: Retrospective case series of 5 adult patients with orbital PNET. Orbitotomy was performed in all cases. The authors report clinical findings, radiologic features, histopathology, immunohistochemical analysis, management, and outcomes for 5 patients with orbital PNET. Results: Five adult patients presented with progressive unilateral proptosis and visual impairment. Common radiographic findings included a heterogeneous mass without associated destructive features, located in the superior and/or lateral orbit. Four cases demonstrated strong immunohistochemical staining for CD99 in a membranous pattern. One case required chromosomal analysis with fluorescence in situ hybridization to confirm the diagnosis. All patients received chemotherapy and/or orbital radiation with resolution of proptosis but no improvement of vision. One patient died of disease. Conclusions: To the authors' knowledge, this is the largest series of orbital PNET in adults. This tumor has an age demographic wider than previously believed and should be considered in the differential diagnosis of a hypercellular small round cell orbital tumor in both children and adults. Current treatment regimens are not standardized but typically use a similar approach to the treatment of Ewing sarcoma. Orbital PNET appears to have less propensity for metastasis compared with PNET in other locations. However, long-term aggressiveness remains to be proven.",
author = "Weerawan Chokthaweesak and Annunziata, {Christine C.} and Oday Alsheikh and John Ng and David Wilson and Atiya Mansoor and Vassil Kaimatchiev and Robert Weatherhead and Jack Rootman and Geva Mannor and Kanjana Leelapatranurak and Korn, {Bobby S.} and Kikkawa, {Don O.}",
year = "2011",
month = "5",
doi = "10.1097/IOP.0b013e3181f9df17",
language = "English (US)",
volume = "27",
pages = "173--179",
journal = "Ophthalmic Plastic and Reconstructive Surgery",
issn = "0740-9303",
publisher = "Lippincott Williams and Wilkins",
number = "3",

}

TY - JOUR

T1 - Primitive neuroectodermal tumor of the orbit in adults

T2 - A case series

AU - Chokthaweesak, Weerawan

AU - Annunziata, Christine C.

AU - Alsheikh, Oday

AU - Ng, John

AU - Wilson, David

AU - Mansoor, Atiya

AU - Kaimatchiev, Vassil

AU - Weatherhead, Robert

AU - Rootman, Jack

AU - Mannor, Geva

AU - Leelapatranurak, Kanjana

AU - Korn, Bobby S.

AU - Kikkawa, Don O.

PY - 2011/5

Y1 - 2011/5

N2 - Purpose: To present the clinical, radiologic, and histopathologic features of orbital primitive neuroectodermal tumor (PNET) in 5 adult patients. Methods: Retrospective case series of 5 adult patients with orbital PNET. Orbitotomy was performed in all cases. The authors report clinical findings, radiologic features, histopathology, immunohistochemical analysis, management, and outcomes for 5 patients with orbital PNET. Results: Five adult patients presented with progressive unilateral proptosis and visual impairment. Common radiographic findings included a heterogeneous mass without associated destructive features, located in the superior and/or lateral orbit. Four cases demonstrated strong immunohistochemical staining for CD99 in a membranous pattern. One case required chromosomal analysis with fluorescence in situ hybridization to confirm the diagnosis. All patients received chemotherapy and/or orbital radiation with resolution of proptosis but no improvement of vision. One patient died of disease. Conclusions: To the authors' knowledge, this is the largest series of orbital PNET in adults. This tumor has an age demographic wider than previously believed and should be considered in the differential diagnosis of a hypercellular small round cell orbital tumor in both children and adults. Current treatment regimens are not standardized but typically use a similar approach to the treatment of Ewing sarcoma. Orbital PNET appears to have less propensity for metastasis compared with PNET in other locations. However, long-term aggressiveness remains to be proven.

AB - Purpose: To present the clinical, radiologic, and histopathologic features of orbital primitive neuroectodermal tumor (PNET) in 5 adult patients. Methods: Retrospective case series of 5 adult patients with orbital PNET. Orbitotomy was performed in all cases. The authors report clinical findings, radiologic features, histopathology, immunohistochemical analysis, management, and outcomes for 5 patients with orbital PNET. Results: Five adult patients presented with progressive unilateral proptosis and visual impairment. Common radiographic findings included a heterogeneous mass without associated destructive features, located in the superior and/or lateral orbit. Four cases demonstrated strong immunohistochemical staining for CD99 in a membranous pattern. One case required chromosomal analysis with fluorescence in situ hybridization to confirm the diagnosis. All patients received chemotherapy and/or orbital radiation with resolution of proptosis but no improvement of vision. One patient died of disease. Conclusions: To the authors' knowledge, this is the largest series of orbital PNET in adults. This tumor has an age demographic wider than previously believed and should be considered in the differential diagnosis of a hypercellular small round cell orbital tumor in both children and adults. Current treatment regimens are not standardized but typically use a similar approach to the treatment of Ewing sarcoma. Orbital PNET appears to have less propensity for metastasis compared with PNET in other locations. However, long-term aggressiveness remains to be proven.

UR - http://www.scopus.com/inward/record.url?scp=79957811055&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=79957811055&partnerID=8YFLogxK

U2 - 10.1097/IOP.0b013e3181f9df17

DO - 10.1097/IOP.0b013e3181f9df17

M3 - Article

C2 - 21088632

AN - SCOPUS:79957811055

VL - 27

SP - 173

EP - 179

JO - Ophthalmic Plastic and Reconstructive Surgery

JF - Ophthalmic Plastic and Reconstructive Surgery

SN - 0740-9303

IS - 3

ER -