Current Issue Search Articles Instructions for authors
 

 HOME | Login   

 

 Small font sizeDefault font sizeIncrease font size Print this article Email this article Bookmark this page
 Users online: 130


  In this article
    Abstract
    Introduction
    Case Report
    Discussion
    Conclusion
    Acknowledgements
    References
    Article Figures

 Article Access Statistics
    Viewed18949    
    Printed283    
    Emailed0    
    PDF Downloaded74    
    Comments [Add]    

Recommend this journal

 
 
Year : 2010  |  Volume : 4  |  Issue : 1  |  Page : 18-21    Table of Contents


 
CASE REPORT

Metastasis from scapular Ewing's sarcoma presenting as sutural diastasis: An unusual presentation

1 Department of Orthopaedic Surgery, J. N. Medical College, A.M.U., Aligarh, India
2 Senior Resident, Department of Radiotherapy, J. N. Medical College, A.M.U., Aligarh, India

Correspondence Address:
Yasir Salam Siddiqui
Senior Resident, Department of Orthopaedic Surgery, J. N. Medical College, A.M.U., Aligarh
India
Login to access the Email id

Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0973-6042.68415

Rights and Permissions

 

 
Similar in PUBMED
   Search Pubmed for
   Search in Google Scholar for
 Related articles
Article in PDF (3,033 KB)
Reader Comments
Access Statistics
Citation Manager
Add to My List *
* Registration required (free)

Date of Web Publication12-Aug-2010

   Abstract 

Ewing's sarcoma is a malignant non-osteogenic primary tumor of the bone. It is one of the most common primary malignant tumors of bone. Peak incidence is noticed in second decade of life with male preponderance of 1.6:1. It occurs most frequently in long bones and flat bones of pelvic girdles. In 30% cases, Ewing's sarcoma is multicentric in origin. In 14-50%, multiple metastases are present at the time of diagnosis. CNS spread is rare and isolated CNS involvement is not seen. Skull metastasis of Ewing's sarcoma is not rare compared to primary Ewing's sarcoma of the skull, but the actual frequency is unknown. We wish to report a case of "Primary Ewing's sarcoma of scapula with metastasis to Skull Vault in a Child resulting in sutural diastasis" diagnosed by clinicoradiological examination and confirmed by histopathology.


Keywords: Ewing′s sarcoma, non-osteogenic, skull vault, sutural diastasis


How to cite this article:
Asif N, Khan AQ, Siddiqui YS, Mustafa H. Metastasis from scapular Ewing's sarcoma presenting as sutural diastasis: An unusual presentation. Int J Shoulder Surg 2010;4:18-21

How to cite this URL:
Asif N, Khan AQ, Siddiqui YS, Mustafa H. Metastasis from scapular Ewing's sarcoma presenting as sutural diastasis: An unusual presentation. Int J Shoulder Surg [serial online] 2010 [cited 2018 Dec 14];4:18-21. Available from: http://www.internationalshoulderjournal.org/text.asp?2010/4/1/18/68415



   Introduction Top


Ewing's sarcoma is a malignant non-osteogenic primary tumor of the bone. It is one of the most common primary malignant tumors of bone. Peak incidence is noticed in second decade of life with male preponderance of 1.6:1. It occurs most frequently in long bones and flat bones of pelvic girdles. [1] In 30% cases, Ewing's sarcoma is multicentric in origin. [2] In 14-50%, multiple metastases are present at the time of diagnosis. [3] CNS spread is rare and isolated CNS involvement is not seen. [4] Skull metastasis of Ewing's sarcoma is not rare compared to primary Ewing's sarcoma of the skull, but the actual frequency is unknown. [13],[14],[15] We wish to report a case of "Primary Ewing's sarcoma of scapula with metastasis to Skull Vault in a Child resulting in sutural diastasis" diagnosed by clinicoradiological examination and confirmed by histopathology.

Ewing's sarcoma is a malignant non-osteogenic primary tumor of the bone. Originally, James Ewing described it in 1921 as a tumor arising from undifferentiated osseous mesenchymal cells; however, recent studies suggest that Ewing's tumor may be neuroectodermally derived from the primitive neural tissue. [8] Ewing's sarcoma is a malignant tumor of the bone that usually occurs during the first two decades of life. It has predilection for the trunk and long bones. 30% of these tumors are multicentric and metastasis is often present at the time of diagnosis. [2],[3] Primary Ewing's sarcoma of skull vault is very rare. Skull metastasis of Ewing's sarcoma is not rare compared to primary Ewing's sarcoma of the skull, but the actual frequency is unknown. The location of skull metastasis of Ewing's sarcoma is also not clear. [13],[14],[15] The earliest symptom is pain. Other common symptoms include fever, anemia, and non-specific signs of inflammation, such as an increase in sedimentation rate, moderate leukocytosis, and an increase in serum LDH. Classical radiological presentation of Ewing's sarcoma is a destructive lesion in the diaphysis of long bones with an onionskin periosteal reaction. Skip metastases are rare in Ewing's sarcoma. They are a well-recognized feature of osteosarcoma and are associated with a poorer prognosis than cases clear of such lesions. Skip lesions appear to be rare in Ewing's sarcoma, [16],[17],[18],[19] and the prognostic implications of skip lesions in Ewing's sarcoma remains unclear. In flat bones, Ewing's sarcoma appears as a nonspecific destructive lesion. From remote primary sites like pelvis and long bones, Ewing's sarcoma may metastasize to skull. Secondry skull lesions presents with osteolysis with erosion of inner and outer table associated with soft tissue swelling on plain radiographs.

The management of Ewing's sarcoma includes multiagent neoadjuvent chemotherapy followed by enblock excision of the tumor mass. Postoperative radiotherapy is given if there is doubt of tumor residua. The most unfavorable prognostic factor in Ewing's sarcoma is the presence of distant metastasis at diagnosis. Other unfavorable prognostic factors include an age older than 10 years, a size larger than 200 ml, more central lesions (as in the pelvis or spine), and poor response to chemotherapy. [5],[6],[7]


   Case Report Top


A 5-year-old boy presented with 5 months history of pain followed by swelling around the right shoulder girdle. 2 months prior to consultation at J N Medical College, AMU, Aligarh, patient's mother noticed gradually increasing swelling over scalp. The patient had no history of headache, weakness of upper and lower limbs. There were no signs of raised intracranial tension. There was no evidence of weakness of upper and lower limbs and other localizing signs of intracranial mass. On clinical examination, the shoulder girdle swelling was diffuse with ill-defined margins, firm to hard in consistency with tense and shiny over lying skin [Figure 1]. Scalp swelling was present in frontoparietal region, which was hard in consistency and tender [Figure 2]. Rest of the skeletal survey was normal. At the time of presentation, plain radiograph of the affected shoulder showed a sclerotic lesion of scapula with loss of definition of margins of scapula with soft tissue mass [Figure 3]. Lateral radiograph of skull showed osteolysis with erosion of inner and outer table associated with sutural diastasis and periosteal reaction [Figure 4]. All the blood parameters were normal except the alkaline phosphatase level, which was raised. FNAC from the scapular and scalp swelling was consistent with malignant small round cell tumor - Ewing's sarcoma [Figure 5]. The patient was subjected to local radiotherapy followed by multiagent chemotherapy after histological confirmation of Ewing's sarcoma. The patient showed dramatic response to chemoradiation therapy both clinically and radiologically [Figure 6] and [Figure 7]. The post-treatment lateral radiograph of skull showed development of sclerosis in and around the lesion [Figure 8]. The sclerosis seems to be bridging the diastasis.
Figure 1 : Clinical photograph showing massive scapular swelling

Click here to view
Figure 2 : Clinical photograph showing skull vault swelling in the frontoparietal region

Click here to view
Figure 3 : Pre-treatment radiograph of right shoulder showing a sclerotic lesion of scapula with loss of definition of margins of scapula and soft tissue mass

Click here to view
Figure 4 : Lateral radiograph of skull vault showing an osteolytic lesion with erosion of inner and outer tables associated with sutural diastasis and periosteal reaction

Click here to view
Figure 5 : FNAC from scapular mass was consistent with small round cell tumor of childhood - Ewing's sarcoma. (a) Photomicrograph showing loose clusters and the discrete cell population of monomorphic, round cells with scant cytoplasm, slightly irregular nuclei, and occasional pseudo-rosette and focal necrosis. Note that a mixture of two types of cell population - small and large - can be appreciated, one of the criteria for diagnosis of Ewing's sarcoma (H and E stain). (b) Photomicrograph focused to show rosette like structure formation, as occasionally seen in cases of Ewing's sarcoma (H and E stain). (c) Photomicrograph showing PAS positivity in few viable cells (PAS stain)

Click here to view
Figure 6 : Clinical photograph showing significantly reduced scapular swelling (post treatment)

Click here to view
Figure 7 : Radiograph of right shoulder showing extensive sclerosis of the lesion after chemoradiation

Click here to view
Figure 8 : Post-treatment lateral radiograph of skull vault showing sclerosis in and around the lesion. The sclerosis seems to be bridging the diastasis

Click here to view



   Discussion Top


Ewing sarcoma is the most common primary bone tumor of the flat bones. Primary Ewing's sarcoma of skull vault is very rare. Skull metastasis of Ewing's sarcoma is not rare compared to primary Ewing's sarcoma of the skull, but the actual frequency is unknown. [13],[14],[15]

In our patient appearance of a scapular lesion was consistent with osteogenic sarcoma, as the lesion is primarily sclerotic and it does not look like Ewing's at all. Radiographic appearance of Ewing's sarcoma in the thorax is quite variable and the lesion may be primarily lytic or sclerotic or may represent the mixture of the two. [10],[11] A universal feature of the disease is the presence of a large soft tissue mass relatively larger than the extent of bone changes as was evident in our patient. Sometimes the only apparent change in a radiograph is the presence of a mass, cortical erosion or periosteal reaction being hardly visible. [12] Unlike osteogenic sarcoma, Ewing's sarcoma occurs almost as frequently in flat bone as in long bones, with a small bias toward long bones. As Ewing's sarcoma shows close resemblance to changes seen in osteomyelitis, osteogenic sarcoma, eosinophilic granuloma, metastatic deposits, Vohkam VG et al, concluded that diagnosis of Ewing's sarcoma by roentgenography alone is in some cases not warranted at all and in others it is, at best, a presumptive diagnosis which requires confirmation by histological examination in all. [12]

Secondry skull lesions presents with osteolysis with erosion of inner and outer table associated with soft tissue swelling on plain radiographs. In our patient metastasis from primary lesion resulted in sutural diastasis. Similar skull involvement may also be seen in lesions in Eosinophilic granuloma/Hand-Schuller-Christian syndrome, Burkitt's lymphoma, fibrous dysplasia, aneurysmal bone cysts, osteoclastoma, and giant cell reparative granuloma. Radiologically it is difficult to distinguish them; however, they can be distinguished by a different pattern of destruction of skull vault, presence of soft tissue swelling, calcification, cystic component, and septations. However, histopathology is more specific for their differentiation. MRI is the imaging modality of choice for evaluating the extent of the primary tumor, to monitor the response to neoadjuvent chemotherapy and to follow up unresected Ewing's sarcoma. Bone scintigraphy is necessary to detect skeletal metastasis at the earliest, and 201-thalium scanning has been shown to be sensitive in the monitoring of the response [9] to treatment. Our patient had evidence of distant (skull) metastasis at the time of diagnosis, an axial not an appendicular lesion and extensive lesion-all unfavorable prognostic factors for disease.


   Conclusion Top


Skull metastasis of Ewing's sarcoma is not rare compared to primary Ewing's sarcoma of the skull, but metastasis resulting in sutural diastasis is rare. Patient presents with scalp swelling with or without non-specific neurological signs. CT scan and MRI delineate skull vault lesion with associated epidural soft tissue mass. Histopathology differentiates it from other primary skull vault lesions.


   Acknowledgements Top


We gratefully acknowledge Dr. Rana Sherwani, Head of the Department of Pathology, and Dr. Maddipati Veera Venkata Rao, Junior Resident Department of Orthopaedic Surgery for their valuable contribution in managing this patient.

 
   References Top

1.Dahlin DC, Unni KK. Bone Tumors. In: Thomas CC, Sprigfield IL, editors. General aspects and data on 8542 cases, No. 4. Illinois: Charles C Thomas Pub Ltd.; 1986. p. 269-305.  Back to cited text no. 1      
2.Miller RW. Contrasting epidemiology of childhood osteosarcoma, Ewing's tumor, and rhabdomyosarcoma. Natl Cancer Inst Monogr 1981;56:9-15.  Back to cited text no. 2  [PUBMED]    
3.Ewing J. The Classic: Diffuse endothelioma of bone. Proceedings of the New York Pathological Society. 1921;12:17. Clin Orthop Relat Res 2006;450:25-7.   Back to cited text no. 3      
4.Trigg ME, Glaubiger D, Nesbit ME Jr. The frequency of isolated CNS involvement in Ewing's sarcoma. Cancer 1982;49:2404-9.   Back to cited text no. 4  [PUBMED]    
5.Evans R, Nesbit M, Askin F, Burgert O, Cangir A, Foulkes M, et al . Local recurrence, rate and sites of metastases, and time to relapse as a function of treatment regimen, size of primary and surgical history in 62 patients presenting with non-metastatic ewing's sarcoma of the pelvic bones. Int J Radiat Oncol Biol Phys 1985;11:129-36.  Back to cited text no. 5  [PUBMED]    
6.Fredrickson BE, Baker D, McHolick WJ, Yuan HA, Lubicky JP. The natural history of spondylolysis and spondylolisthesis. J Bone Joint Surg Am 1984;66:699-707.  Back to cited text no. 6      
7.Gehan EA, Nesbit ME Jr, Burgert EO Jr, Viettit J, Tefft M, Perez CA, et al. Prognostic factors in children with Ewing's sarcoma. Natl Cancer Inst Monogr 1981;56:273-8.  Back to cited text no. 7  [PUBMED]    
8.Yalcin S, Turoglu HT, Ozdamar S, Sadikoglu Y, Gurbuzer B, Yenici O. Ewing's tumor of the mandible. Oral Surg Oral Med Oral Pathol 1993;76:362-7.  Back to cited text no. 8  [PUBMED]    
9.Henk CB, Grampp S, Wiesbauer P, Zoubek A, Kainberger F, Breitenseher M, et al. Ewing sarcoma. Diagnostic imaging. Radiologe 1998;38:509-22.   Back to cited text no. 9  [PUBMED]  [FULLTEXT]  
10.Franken EA Jr, Smith JA, Smith WL. Tumors of the chest wall in infants and children. Peadiatr Radiol 1977;6:13-8.  Back to cited text no. 10      
11.Coombs RJ, Bayar EA, Matloub YH, Velasco ME. Pediatric case of the day. Ewing sarcoma of the rib with intraspinal extension. Radiographics 1999;19:241-4.   Back to cited text no. 11  [PUBMED]  [FULLTEXT]  
12.Vohkam V. G., BBS (BoM.), DMRE (CAMB.); Roentgen manifestations in ewing's sarcoma a Study of 156 Cases; From the Department of Radiology, Tata Memorial Hospital, Bombay, India. Presented at the ninth International Cancer Congress, Tokyo, Japan: October 1966.  Back to cited text no. 12      
13.Davidson MJ. Ewing's sarcoma of the temporal bone. A case report. Oral Surg Oral Med Oral Pathol 1991;72:534-6.  Back to cited text no. 13  [PUBMED]    
14.Watanabe H, Tsubokawa T, Katayama Y, Koyama S, Nakamura S. Primary Ewing's sarcoma of the temporal bone. Surg Neurol 1992;37:54-8.  Back to cited text no. 14  [PUBMED]    
15.Zenke K, Hatakeyama T, Hashimoto H, Sakaki S, Manabe K. Primary Ewing's sarcoma of the occipital bone - case report. Neurol Med Chir (Tokyo) 1994;34:246-50.  Back to cited text no. 15  [PUBMED]  [FULLTEXT]  
16.Sundaram M, Merenda G, McGuire MM. A skip lesion in association with Ewing sarcoma. Report of a case. J Bone Joint Surg Am 1989;71:764-8.  Back to cited text no. 16  [PUBMED]    
17.Richardson ML, Gillespy T. Magnetic resonance imaging. In: Kricun ME editor. Imaging of bone tumors. Philadelphia: Saunders; 1993. p. 365.  Back to cited text no. 17      
18.Davies AM, Makwana NK, Grimer RJ, Carter SR. Skip metastases in Ewing's sarcoma: A report of three cases. Skeletal Radiol 1997;26:379-84.  Back to cited text no. 18  [PUBMED]  [FULLTEXT]  
19.Jiya TU, Wuisman PI. Long-term follow-up of 15 patients with non-metastatic Ewing's sarcoma and a skip lesion. Acta Orthop 2005;76:899-903.  Back to cited text no. 19  [PUBMED]  [FULLTEXT]  


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7], [Figure 8]



 

Top
Print this article  Email this article