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LETTER TO EDITOR
Year : 2012  |  Volume : 60  |  Issue : 3  |  Page : 361-362

Multifocal Ewing's sarcoma, presenting radiologically as extradural hematoma


Department of Neurosurgery, SCB Medical College and Hospital, Cuttack, Odisha, India

Date of Submission01-May-2012
Date of Decision02-May-2012
Date of Acceptance14-May-2012
Date of Web Publication14-Jul-2012

Correspondence Address:
Deepak K Parida
Department of Neurosurgery, SCB Medical College and Hospital, Cuttack, Odisha
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0028-3886.98546

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How to cite this article:
Mishra SS, Parida DK, Senapati SB, Patra SK. Multifocal Ewing's sarcoma, presenting radiologically as extradural hematoma. Neurol India 2012;60:361-2

How to cite this URL:
Mishra SS, Parida DK, Senapati SB, Patra SK. Multifocal Ewing's sarcoma, presenting radiologically as extradural hematoma. Neurol India [serial online] 2012 [cited 2019 Nov 15];60:361-2. Available from: http://www.neurologyindia.com/text.asp?2012/60/3/361/98546


Sir,

An 11-year-old girl presented with painful progressing swelling over right parietal scalp region and left knee, following trivial trauma of 1 month duration and disorientation with poor feeding for 10 days. On examination, she responded to verbal commands but was not fully oriented to time, place, and person. Otherwise neurological examination was normal. Right parietal mass was firm and immobile with regular contours. There was no regional lymphadenopathy or hepatosplenomegaly. Non-contrast computed tomography of head revealed right parietal biconvex hyperdense lesion with midline shift [Figure 1]. There was also an overlying extracalvarial soft tissue swelling. Under general anesthesia, right parietal skin flap was done. An extracalvarial firm, well-defined, moderately vascular mass containing thick reddish-white fibrous material with jelly-like component adherent to bone surface was found. The rough and eroded bone was removed along with the extracalvarial mass. Similar extradural material was found underneath bone which was removed. Wound closed in layers and surgery was uneventful. The histopathological study revealed fibro-collagenous stroma and irregular boney specules enclosing clusters and lobules of uniform looking small dark round cells with scanty cytoplasm, areas of hemorrhage and necrosis suggestive of skeletal Ewing's sarcoma (ES) extending into soft tissue compartment [Figure 2]. Immunohistochemically, the lesion showed positive for CD-99. Plain X-ray of left knee joint [Figure 3] showed sclerotic periosteal reaction with cortical destruction. Fine needle aspiration cytology of the swelling at the upper end of tibia came out to be ES. On day 3 postoperative, the patient presented with enuresis, encopresis, and paraplegia with bilateral sensory loss below T10 level. Contrast-enhanced sagittal spine T2-weighted magnetic resonance imaging showed multiple lesions in T7, T10, and l1 vertebral bodies with a lumbar epidural mass. Ultrasound abdomen and pelvis showed multiple irregular mixed echogenic mass in the right lobe. X-ray spine and X-ray chest revealed no abnormality. The patient was referred for craniospinal chemo-radiation. Two months after treatment the patient died with recurrence of multiple scalp swelling and tumor cachexia.
Figure 1: Non-contrast computed tomography scan of head showing RT. Parietal extradural biconvex hyperdense lesion with midline shift and an overlying extracalvarial soft tissue swelling

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Figure 2: Tissue section from extracalvarial and extradural mass showing fibrocollagenous stroma and irregular bone spicules enclosing clusters and lobules of uniform looking small dark round cells with scanty cytoplasm

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Figure 3: Plain X-ray of LT. Knee joint (AP and lateral view) showed sclerotic periosteal reaction with cortical destruction

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ES occurs between the age of 10 and 20 years with an incidence of 9-10 per 1,000,000 populations. [1] Over 95% cases are characterized by a rearrangement of chromosome 22q12. [2] Spine and skull as primary sites of ES are rare and accounts for 6% and 2%, respectively. [3] Metastatic disease can be present in 25% of patients at the time of presentation. Multiple bone involvement has been shown to occur in the advanced stage of the disease. On rare occasions, ES involves multiple bones at the time of diagnosis. Multifocal ES and its radiological simulation as extradural hematoma (EDH) make our case a unique one.

Age and surgical treatment are important prognostic variables in the treatment of extraskeletal ES. The median survival for all patients is 4 months after the diagnosis of brain lesions. Six-month and 1-year survival rates are 27% and 11.5%, respectively. [4],[5] Our patient died 3 months after diagnosis. Treatment of cranial and spinal epidural lesions involves surgical excision followed by adjuvant radiotherapy and combination chemotherapy with vircristine sulfate, actinomycin-d, and cyclophosphamide (vac) alternated with ifosfamide, cisplatin, and etoposide (ice). [6],[7] New biologic modifiers, such as imatinib and zapamycin, are being investigated as adjunctive therapies to conventional therapy. [8] ES must be regarded as a systemic disease. Chemotherapy has increased survival rates to 65-70% in patients with localized disease and 25-30% in those with metastatic disease at the time of diagnosis. Micrometastasis and circulating tumor cells have recently been reported as major prognostic factors.

Multifocal ES involving calvarium, meninges, spinal cord, and long bones of lower extremity seem to be very uncommon. In the presence of trauma, the biconvex hyperdense extradural lesion is often confused with EDH. ES may mimic osteomyelitis in children. Therefore, in cases with multifocal progressive swellings following trivial trauma ES must always be kept in mind especially in childhood and should always be considered early in the work-up.

 
  References Top

1.Kurihashi A, Tamai K, Saotome K, Yamaguchi T. Multifocal Ewing's sarcoma and hypercalcemia. A case report. Clin Orthop Relat Res 1996;326:254-8.  Back to cited text no. 1
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2.Marec-Bérard P, Philip T. Ewing sarcoma: The pediatrician's point of view. Pediatr Blood Cancer 2004;42:477-80.  Back to cited text no. 2
    
3.Raney RB, Asmar L, Newton WA Jr, Bagwell C, Breneman JC, Crist W, et al. Ewing's sarcoma of soft tissues in childhood: A report from the intergroup Rhabdomyosarcoma Study, 1972 to 1991. J Clin Oncol 1997;15:574-82.  Back to cited text no. 3
    
4.Paulino AC, Nguyen TX, Barker JL Jr. Brain metastasis in childrenwith sarcoma, neuroblastoma, and wilms' tumor. Int J Radiat Oncol Biol Phys 2003;57:177-83.  Back to cited text no. 4
    
5.Somers GR, Shago M, Zielenska M, Chan HS, Ngan BY. Primary subcutaneous primitive neuroectodermal tumor with aggressive behavior and anunusual karyotype: Case report. Pediatr Dev Pathol 2004;7:538- 45.  Back to cited text no. 5
    
6.Sorensen PH, Lessnick SL, Lopez-Terrada D, Liu XF, Triche TJ, Denny CT. A second Ewing's sarcoma translocation, t(21;22), fuses the EWS gene to another ETS-family transcription factor, ERG. Nat Genet 1994;6:146-51.  Back to cited text no. 6
    
7.Jeon IS, Davis JN, Braun BS, Sublett JE, Roussel MF, Denny CT, et al. A variant Ewing's sarcoma translocation (7;22) fuses the EWS gene to the ETS gene ETV1. Oncogene 1995;10:1229-34.  Back to cited text no. 7
    
8.Rodriquez-Galindo C, Spunt SL, Pappo AS. Treatment of Ewing sarcoma family of tumors: Current status and outlook for the future. Med Pediatr Oncol 2003;40:276-87.  Back to cited text no. 8
    


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