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CASE REPORT Table of Contents   
Year : 2010  |  Volume : 27  |  Issue : 4  |  Page : 146-148
Fine needle aspiration cytology diagnosis of paravertebral extraosseus Ewing's sarcoma


1 Department of Pathology, Padmashree Dr. D.Y. Patil Medical College, Pimpri, Pune - 411 020, India
2 Department of Radiology, Padmashree Dr. D.Y. Patil Medical College, Pimpri, Pune - 411 020, India

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Date of Web Publication27-Nov-2010
 

   Abstract 

Extraskeletal Ewing's sarcoma (EES) is a rare tumor. Paravertebral Ewing's sarcoma requires more extensive therapy as compared to Ewing's sarcoma of bone. Fine needle aspiration cytology (FNAC) plays an important role in the early diagnosis of these cases. We present a case where paravertebral extraosseous Ewing's sarcoma was diagnosed on FNAC in a 19-year-old girl.

Keywords: Extraskeletal Ewing′s sarcoma; fine needle aspiration cytology; paravertebral region

How to cite this article:
Buch AC, Panicker N K, Sarawagi S, Anwekar S, Kharat AT. Fine needle aspiration cytology diagnosis of paravertebral extraosseus Ewing's sarcoma. J Cytol 2010;27:146-8

How to cite this URL:
Buch AC, Panicker N K, Sarawagi S, Anwekar S, Kharat AT. Fine needle aspiration cytology diagnosis of paravertebral extraosseus Ewing's sarcoma. J Cytol [serial online] 2010 [cited 2017 Sep 22];27:146-8. Available from: http://www.jcytol.org/text.asp?2010/27/4/146/73304



   Introduction Top


Ewing's sarcoma of bone (ES), extraskeletal Ewing's sarcoma (EES) and primitive peripheral neuroectodermal tumor (pPNET) are now considered to be the same tumor with variable differentiation and no significant biological or therapeutic distinction. [1] With a change in treatment modality of Ewing's-pPNET family of tumors by preoperative chemotherapy, fine needle aspiration cytology (FNAC) plays a useful role in the diagnosis of these lesions with obvious advantage over the open/needle core biopsy.

EES has been described in different locations such as larynx, scalp, nasal fossa, neck, chest wall, lung, perineum, finger, arm, lip and toe. EES requires more extensive therapy as compared to ES. It is essential to diagnose these cases early to change clinician's traditional method of handling such a case. We report one such case of paravertebral EES diagnosed by ultrasound guided FNAC.


   Case Report Top


A 19-year-old girl was admitted with complaints of progressively increasing dull aching pain in the lower back and right lower limb since 2 months. There was a history of loss of appetite, mild fever and malaise. The patient was thin built. On general examination, there was a mild pallor. Systemic examination did not reveal any abnormalities, except weakness (power 3/5) in the right lower limb.

Investigations revealed hemoglobin of 10 g%, total leucocyte count of 9200 cells/mm 3 with 75% polymorphs and 23% lymphocytes. Erythrocyte sedimentation rate (ESR) was 65 mm at the end of 1 hour. The urine examination for albumin, sugar, vinyl mandelic acid and the bone marrow examination did not reveal any abnormalities. Tuberculin test was negative. Chest radiograph was normal. Radiograph of the spine revealed a radiopaque soft tissue mass in right paravertebral region, measuring 8Χ7 cm, in L5-S1 region . Ultrasound revealed a large ill-defined echo-poor heterogeneous lesion in the right paraspinal region.

Magnetic resonance imaging (MRI) scan revealed relatively well-defined, lobulated, heterogeneously enhancing soft tissue lesion with large paraspinal component encroaching spinal canal through the right L5-S1 foramina, causing a mass effect [Figure 1]. Associated altered signals and the pressure erosions over the adjacent bones due to infiltration and inflammatory reaction were noted. The lesion was suspected to be of neurogenic origin. FNAC was advised for definite diagnosis . USG guided FNAC was done using long 22 gauge disposable needle and 10 ml syringe. Slides were prepared and stained with May-Grünwald-Geimsa (MGG). Two slides were reserved for special stains and studies.
Figure 1 :T1-weighted MRI showing a tumor mass at L5-S1 region

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FNAC revealed cellular smears composed of densely dispersed, small, monomorphic round cells with fine nuclear chromatin and round nuclei and scanty clear cytoplasm [Figure 2]a. Occasional mitoses were noted at places. Many cells showed irregularly vacuolated cytoplasm which on periodic acid Schiff (PAS) stain revealed intense PAS positivity [Figure 2]b. Focal areas showed striking clusters, occasional rosette-like formations and moulding of nuclei. The background was clean.
Figure 2 :(a) EES shows clusters of monomorphic tumor cells with vacuolated cytoplasm (MGG, ×400), (b) Tumor cells showing cytoplasm positivity with PAS (PAS, ×400)

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FNAC diagnosis of malignant small round cell tumor, most likely ES, was offered. Later, a core biopsy was performed for confirmation. Histopathological sections revealed tumor with solidly packed round cell pattern of striking uniformity. The individual cells possessed a round or ovoid nucleus measuring 10 ΅m in diameter with a distinct nuclear membrane, finely divided powdery chromatin, and a single minute nucleolus. The cytoplasm was scanty and pale staining. PAS stain revealed positive intracellular deposits of glycogen. Psuedorosettes were also noted. Vascularization was prominent [Figure 3]. The patient was referred to tertiary care centre for further management. Immunohistochemistry was performed there, in which the tumor cells exhibited nonspecific enolase (NSE) and vimentin along with diffuse membranous staining for CD-99. The patient was given multiagent chemotherapy combined with en bloc resection and radiotherapy.
Figure 3 :Photomicrograph showing sheets of monomorphic small round blue cells with scant cytoplasm (H and E, ×400)

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   Discussion Top


EES is a poorly differentiated, highly malignant, round cell tumor without cellular or structural differentiation, and aggressive clinical behavior with high rate of local recurrence and distant metastasis. [2],[3] The reported common sites of origin are trunk (paravertebral region and chest) (32%), extremity (26%), head and neck (18%), retroperitoneum, pelvis (16%), and others (10%). [2]

The origin of EES and ES has been a matter of research since the time James Ewing described the first case of ES in 1921. [3] EES was first described by Tefft et al, [4] in 1969. In 1975, Angerwall and Enzinger [5] reviewed 39 cases of EES resembling ES. In general, the tumor presents as a rapidly growing, deeply located mass measuring 5-10 cm in greatest diameter. The tumor is painful in about one-third of cases. If peripheral nerve or spinal cord is involved, there may be progressive sensory or motor disturbances. As with other round cell sarcomas, the preoperative duration of symptoms is usually less than 1 year. Unlike neuroblastoma, catecholamine determinations are within normal limits. Radiographs, computed tomography (CT) scan or MRI is essential for establishing the extraskeletal site of the tumor. Cytology and histopathology are essential for definite diagnosis.

Ewing's sarcoma is richly vascular and shows an abundance of thin-walled vessels within the fibrovascular septae. The vessels tend to get compressed and obscured by closely packed tumor cell population. This cytomorphological observation may prove to be a useful clue to cytological diagnosis. [6]

ES represents the most undifferentiated, and the pPNET, the most differentiated ends of the spectrum. The two ends differ in morphology as well as in neuronal differentiation. This family of tumors shares common cytogenetic and molecular changes which involve the translocation of Ewing's sarcoma gene on chromosome 22 (22q12) on to a number of other genes like fli-1 on chromosome 11 (11q24) in 90% of cases and erg on chromosome 21 (21q22). [7] The tumors also share expression of glycoprotein surface antigen p30/32 (mic2)/CD99, a cell membrane protein of unknown function.

The differential diagnosis on FNAC and histopathology includes other round cell tumors like metastatic neuroblastoma, rhabdomyosarcoma and non Hodgkin's Lymphoma (NHL) in children, and metastatic small cell anaplastic carcinoma and NHL need exclusion in adults.

The divisions of these tumors into skeletal and extraskeletal tumors appears more relevant than subtyping into ES, EES and pPNET. The skeletal Ewing sarcoma is known to have a 5-year survival of 75%. [8] The EES and pPNET, on the other hand, have 38% survival. [9] Paravertebral EES patients may require more extensive field radiation and more intensive chemotherapy to achieve better local and systemic tumor control. [10]


   Conclusions Top


FNAC is a very useful, economic and quick procedure in the diagnosis of Ewing sarcoma family of tumors. Accurate diagnosis can be made even in deep seated tumors as in our case, if one gets adequate material under radiological guidance. One needs biopsy for confirmation if scanty material is obtained. FNAC is also useful in long-term follow-up of these cases for diagnosis of recurrence or rarely intercurrent second malignancy. [ 11]

 
   References Top

1.Kilpatrick SE, Giesinger KR. Soft tissue sarcomas: the usefulness and limitations of fine-needle aspiration biopsy. Am J Clin Pathol 1998;110:50-68.  Back to cited text no. 1
    
2.Kasper GJ, Kamphorst W, van de Graff M, van Alphen HA, Veerman AJ. Primary spinal epidural extra osseous Ewing's sarcoma. Cancer 1991;68:648-54.  Back to cited text no. 2
    
3.Yadav TP, Singh RP, Gupta VK, Chaturvedi NK, Prasad CV. Paravertebral extraosseous Ewing's sarcoma. Indian Pediatr 1998;35:557-60.  Back to cited text no. 3
[PUBMED]    
4.Tefft M, Vawter GF, Mitus A. Paravertebral "round cell tumors" in children. Radiology 1969;92:1501-9.   Back to cited text no. 4
[PUBMED]    
5.Angervall L, Enzinger FM. Extraskeletal neoplasm resembling Ewing's sarcoma. Cancer 1975;36:240-51.  Back to cited text no. 5
[PUBMED]    
6.Malhotra P, Hora H, Agrawal U. Capillary vessels in Ewing's sarcoma: Overlooked? J Cytol 2009;26:126-7.  Back to cited text no. 6
  Medknow Journal  
7.de Alava E, Kawai A, Healey JH, Fligman I, Meyers PA, Huvos AG, et al. EWS-FLI1 fusion transcript structure is an independent determinant of prognosis in Ewing's sarcoma. J Clin Oncol 1998;16:1248-55.  Back to cited text no. 7
[PUBMED]  [FULLTEXT]  
8.Rosai J. Bone and joints. In: Rosai A, editor. Surgical pathology. 9 th ed. St Louis: Mosby, Elsevier; 2009. p. 2176.  Back to cited text no. 8
    
9.Harimaya K, Oda Y, Mastuda S, Tanaka K, Chuman H, Iwamoto Y. Primitive neuroectodermal tumor and extraskeletal Ewing sarcoma arising primarily around the spinal column: report of four cases and a review of the literature. Spine 2003;28:408-12.  Back to cited text no. 9
    
10.Ortega JA, Wharam M, Gehan EA, Ragab AH, Crist W, Webber B, et al. Clinical features and results of therapy for children with paraspinal soft tissue sarcoma: a report of the Intergroup Rhabdomyosarcoma Study. J Clin Oncol 1991;9:796-801.  Back to cited text no. 10
[PUBMED]  [FULLTEXT]  
11.Kuttesch JF Jr, Wexler LH, Marcus RB, Fairclough D, Weaver-McClure L, White M, et al. Second malignancy in Ewing's sarcoma: radiation dose-dependency of secondary sarcomas. J Clin Oncol 1996;14:2818-25.  Back to cited text no. 11
[PUBMED]  [FULLTEXT]  

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Correspondence Address:
Archana C Buch
B-603, Gold Coast, Ivory Estates, Someshwarwadi, Pune - 411 008
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0970-9371.73304

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