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 D.
AU - Wilson, David J.
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.
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U2 - 10.1097/IOP.0b013e3181f9df17
DO - 10.1097/IOP.0b013e3181f9df17
M3 - Article
C2 - 21088632
AN - SCOPUS:79957811055
SN - 0740-9303
VL - 27
SP - 173
EP - 179
JO - Ophthalmic plastic and reconstructive surgery
JF - Ophthalmic plastic and reconstructive surgery
IS - 3
ER -