Therapy and outcome of orbital primitive neuroectodermal tumor

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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 [1]. 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 [24]. It is most

    commonly seen in the thoracopulmonary region followed by

    head and neck region [5]. Primary PNET in the orbit are rare with

    only 10 cases reported in English literature to the best of our

    knowledge [614].


    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(

    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:

    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 [15].


    Clinical Prole

    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

    orbital PNET.

    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

    any case.

    Local Imaging

    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

    cheek swelling

    Upper eyelid


    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


    matrix with




    Extraorbital extension Yes (Anterior

    cranial fossa)