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Therapy and Outcome of Orbital Primitive Neuroectodermal Tumor
Sameer Bakhshi, MD,1,2,3* Rachna Meel, MD,3,4,5 Syed Ghaffar Hasan Naqvi, MBBS,1,2,3 B.K. Mohanti, MD,2,3,6
Seema Kashyap, MD,3,5,7 Neelam Pushker, MS,3,4,5 and Seema Sen, MD3,5,7
Peripheral primitive neuroectodermal tumor (PNET) along with
Ewing sarcoma is clubbed under the Ewing sarcoma family of
tumors . Peripheral PNET is a group of soft tissue malignancies
that usually affects children and adolescents and represents 4% of
all childhood and adolescent soft tissue tumors . It is most
commonly seen in the thoracopulmonary region followed by
head and neck region . Primary PNET in the orbit are rare with
only 10 cases reported in English literature to the best of our
Retrospective data analysis of primary orbital PNET treated at a
tertiary care cancer center ofAll India Institute ofMedical Sciences,
between June 2003 and June 2008, was undertaken. Patients
Primary orbital primitive neuroectodermal tumor (PNET) is rarewith no reported series. We report six cases of orbital PNET treated ata tertiary care oncology center in northern India from 2003 to2008. None of them had distant metastases. All were treated withneoadjuvant chemotherapy followed by exenteration in two, radio-therapy and adjuvant chemotherapy in ve cases. Three out of six
achieved complete remission at end of therapy with globe salvage inthree and vision in two cases. Chemoradiotherapy may help usto avoid mutilating surgery in large or locally advanced tumors,allowing preservation of vision or the globe. Pediatr Blood Cancer2009;52:544547. 2008 Wiley-Liss, Inc.
Key words: Ewing sarcoma; orbit; primitive neuroectodermal tumor; therapy
2008 Wiley-Liss, Inc.DOI 10.1002/pbc.21902Published online 17 December 2008 in Wiley InterScience(www.interscience.wiley.com)
Additional Supporting Information may be found in the online version
of this article.
1Department of Medical Oncology, New Delhi, India; 2Dr B.R.A.
Institute Rotary Cancer Hospital, New Delhi, India; 3All India Institute
of Medical Sciences, New Delhi, India; 4Oculoplastics & Ocular
Oncology Service, New Delhi, India; 5Dr. Rajendra Prasad Centre for
Ophthalmic Sciences, New Delhi, India; 6Department of Radiotherapy,
New Delhi, India; 7Ocular Pathology Services, New Delhi, India
*Correspondence to: Sameer Bakhshi, Associate Professor of Pediatric
Oncology, Department of Medical Oncology, Dr B.R.A. Institute
Rotary Cancer Hospital, All India Institute of Medical Sciences, New
Delhi, India. E-mail: firstname.lastname@example.org
Received 9 September 2008; Accepted 12 November 2008
544 Brief Reports
demographic prole, clinical data including ophthalmic ndings,
imaging, metastatic workup, treatment, globe salvage, and survival
were studied. Complete remission, partial remission, and progres-
sive disease were dened as per RECIST criteria .
Six cases of primary orbital PNETwere treated during the study
period. Three out of these had a large tumor involvingorbit aswell as
neighboring structures at presentation thatmade it difcult to decide
the primary site of tumor origin. However, these patients primarily
presented with ophthalmic signs and symptoms, imaging showed
predominantly orbital involvement and thus considered as primary
There were three males and three females with a median age of
10.5 years (range: 1.5 months to 20 years; Table I). The youngest
patient who had symptoms from birth was a case of congenital
primary orbital PNET (case 1; Fig. 1a). Vision on the affected side
ranged from no perception of light to 6/12. Four out of six cases had
deterioration of vision secondary to refractive error induced by the
orbital tumor. One case had severe exposure keratitis (case 1).
Fundus examination revealed optic atrophy in one (case 3) and disc
edema with choroidal folds in two patients (cases 2 and 4).
Diagnostic work up including bone marrow biopsy, bone scan, and
CT scan chest did not reveal metastasis or primaries elsewhere in
Computed Tomography (CT) scan of brain and orbit showed a
variably enhancing mass lesion with heterogenous attenuation in
all cases, and average size being 4.25 cm (range: 2.56 cm).
Hypodense areas suggestive of necrosis and hemorrhage were
present in one case (case 1); small calcic densities in two cases
(Fig. 1b) and osseous-cartilaginous calcied matrix with admixed
radiating spicules in another (Fig. 1c). There was bone erosion in
ve out of six cases with tumor extension beyond the orbit in four
cases (Table I).
Histopathology revealed a small round malignant tumor with
cells arranged in nests and sheets; rosettes were not seen. The cells
had a high nucleocytoplasmic ratio with frequent mitotic gures.
Periodic acid Schiff stain was negative in all cases. The tumor cells
were positive forMIC 2 antigen and S100 protein in all cases; either
neuron specic enolase and/or synaptophysin were also positive.
They were negative for desmin, actin, cytokeratin, and leukocyte
common antigen. One case was initially misdiagnosed as brosar-
coma on incisional biopsy, probably because of a small specimen
that was not representative of thewhole tumor. However, we did not
have the facility to study the reciprocal chromosomal translocation
t(11;22)(q24;q12), which is considered pathognomic for this
Pediatr Blood Cancer DOI 10.1002/pbc
TABLE I. Details of Present Case Series
Case#1 Case#2 Case#3 Case#4 Case#5 Case#6
Age/sex 1.5 months/M 4 years/M 9 years/F 12 years/M 13 years/F 20 years/F
Chief complaint Proptosis Proptosis Proptosis Proptosis Lower eyelid and
Duration of symptoms 1.5 months 2 months 6 months 1.5 months 2 months 10 months
Size (maximum dimension) 3 cm 4.5 cm 2.5 cm 3 cm 6 cm 6 cm
Bone erosion Yes Yes Yes Yes Yes No
Calcication Yes (Calcic
No Yes (Calcic
No Yes (Osseous
Extraorbital extension Yes (Anterior