Submit Your Article CMED MEACR meeting
Home Print this page Email this page Users Online: 1738
Home About us Editorial board Search Ahead of print Current issue Archives Submit article Instructions Subscribe Contacts Login 


 
 Table of Contents  
CASE REPORT
Year : 2013  |  Volume : 2  |  Issue : 1  |  Page : 53-56

Synovial sarcoma of the abdominal wall: An unusual presentation


1 Department of Pediatric Surgery, Lokmanya Tilak Municipal General Hospital and Lokmanya Tilak Municipal Medical College, Sion, Mumbai, Maharashtra, India
2 Department of Pediatric Surgery, Topiwala National Medical College and Bai Yamunabai Laxman Nair Charitable Hospital, Mumbai Central, Mumbai, Maharashtra, India

Date of Web Publication19-Apr-2013

Correspondence Address:
Parag J Karkera
B/18, Rachna Apartments, V. P. Road, Andheri (West), Mumbai - 400 058
India
Login to access the Email id

Source of Support: None, Conflict of Interest: None


DOI: 10.4103/2278-0513.110786

Rights and Permissions
  Abstract 

Synovial sarcoma (SS) is a malignant mesenchymal neoplasm which commonly occurs in the extremities in close association with tendon sheaths, bursae, joint capsules, and fascial structures. Rarely, SS may be present in unexpected location such as the abdominal wall. Surgical resection with wide margins is the initial standard treatment; however, a multimodal approach including radiotherapy and chemotherapy is often favored. Here, we present a case of SS of the anterior abdominal wall in a 14-year-old patient with a right upper abdominal lump. He underwent wide surgical excision and has received adjuvant chemotherapy. He is doing well on follow-up of six months.

Keywords: Anterior abdominal wall, soft tissue sarcoma, synovial sarcoma


How to cite this article:
Karkera PJ, Kothari PR, Sandlas G. Synovial sarcoma of the abdominal wall: An unusual presentation. Clin Cancer Investig J 2013;2:53-6

How to cite this URL:
Karkera PJ, Kothari PR, Sandlas G. Synovial sarcoma of the abdominal wall: An unusual presentation. Clin Cancer Investig J [serial online] 2013 [cited 2019 Oct 23];2:53-6. Available from: http://www.ccij-online.org/text.asp?2013/2/1/53/110786


  Introduction Top


The term "synovial sarcoma" (SS) was coined to denominate tumors arising near the tendon sheaths and the joint capsules. Despite its name, SS does not arise from the synovial membrane but rather from a yet unknown multipotent stem cells that are capable of differentiating into mesenchymal and/or epithelial structures and lack synovial differentiation. [1] SS is a rare tumor, accounting for 5% to 10% of soft tissue sarcomas. This tumor rarely arises from an intra-articular location, although it usually arises in an extremity or limb girdle. Rarely, SS may be present in unexpected location such as the abdominal wall. [2] Here, we report a case of SS of anterior abdominal wall in a 14-year-old patient who underwent complete surgical excision and is doing well on follow-up of six months.


  Case Report Top


A 14-year-old male had presented to our hospital with complaints of low-grade diffuse pain in the right upper abdomen since six months, with an increase in the pain intensity since 15 days. He had also noticed a lump in the right upper abdomen since one month which was gradually increasing in size. He did not have any associated complaints of fever, vomiting, jaundice, urinary, or bowel defects. On examination, a lump of approximately 12 cm in diameter, mildly tender, firm in consistency, having an irregular surface was palpable in the right upper quadrant of the abdomen. Clinical examination suggested that this mass was fixed to the muscles of the abdominal wall.

All hematological and biochemical investigations were within normal limits. On ultrasonography of the abdomen, a 12 cm × 11.5 cm sized homogenous solid mass was seen related to the medial surface of the right lobe of liver, posteriorly compressing the pancreatic head and the inferior vena cava, and hence, suggesting a possibility of a pancreatic mass. Computed tomography scans of the abdomen [Figure 1] revealed an 11.3 cm × 10 cm sized hypodense lesion having heterogeneous contrast enhancement, probably arising from the porta hepatis and involving the anterior abdominal wall. There was also a resultant intrahepatic biliary radicle dilatation with compression of the stomach and gall bladder without involving them. Origin of the mass from the liver or the abdominal wall was unclear.
Figure 1: CT scan of the abdomen showing a hypodense lesion anteriorly involving the anterior abdominal wall and posteriorly probably arising from the liver

Click here to view


In view of the above investigations, the patient was posted for exploratory laparotomy. On laparotomy, the mass was seen arising from the rectus abdominis involving the perichondrium of the 12 th rib and compressing the inferior surface of the liver without involving the liver parenchyma or the biliary ducts. The mass was excised with a wide margin (including partial excision of the right rectus abdominus, 12 th rib). During intraoperative dissection of the mass, a rent occurred in the right hemidiaphragm with breach of the right pleura. The diaphragmatic rent was repaired, and a right-sided intercostal drain was kept. The abdominal defect was closed with a prolene mesh. The postoperative recovery was uneventful.

Histopathological examination of the specimen showed oval to spindle pleomorphic cells in a loose myxoid stroma suggesting a high-grade spindle cell sarcoma. On immunohistochemistry analysis, the tumor was positive for immunohistochemistry markers such as EMA, pancytokeratin [Figure 2]a, Bcl-2 [Figure 2]b, MIC-2 [Figure 2]c, and Ki-67 and focally positive for CD34 marker and negative for smooth muscle actin, which suggested of SS.
Figure 2:

Click here to view


The patient has received six cycles of adjuvant chemotherapy (ifosfamide and adriamycin) and is asymptomatic on follow-up of six months.


  Discussion Top


SS is the fourth most common soft tissue sarcoma after malignant fibrous histiocytoma, liposarcoma and rhabdomyosarcoma. [3] About 85% to 90% of the tumors arise from the extremities. [2],[3] The most common location of origin is near the knee in the popliteal fossa. The tumors occur most frequently in adolescents and young adults, with 30% of SS presenting in the first two decades of life with the median age of presentation being 13 years. [4],[5] SS of the abdominal wall is more common in females, while SS in the extremities and the neck are more common in males. [2] No race or ethnic predilection have been reported. [4]

Although the etiology of SS is unknown, almost all SS cells are characterized by a translocation fusing of two genes (SYT) located on the chromosome 19q11, and SSX1, SSX2, or SXX4 located on the chromosome Xp11 breakpoint. Tumors expressing the SYT-SSX1 fusion proteins more often exhibit biphasic histology, a higher proliferation rate, and are associated with a poor outcome. Monophasic tumors express either SYT-SSX1 or SYT-SSX2 genetic rearrangement. [2],[4],[6]

SS usually presents as a slow growing, palpable soft tissue swelling. Occasionally, the swelling may be associated with pain or tenderness and possible minor limitation to range of motion. Constitutional symptoms such as weight loss are rare. Abdominal SSs may present with vague intestinal symptoms along with abdominal pain or lump. These tumors usually occur equally on both sides of the abdomen and are more common in the lower half of the abdomen. 16% to 25% of patients at their initial presentation have metastasis to the lungs (the most frequent site), while others being the bone, the bone marrow, and the lymph nodes. [4],[7]

There are three main histologic subtypes of SS: (1) biphasic, (2) monophasic, and (3) poorly differentiated. The biphasic type represents 20% to 30% of lesions and has both mesenchymal spindle cell components and an obvious epithelial component usually forming glands. The monophasic type is the most common (50-60%) in which the spindle cell component predominates. The poorly differentiated SS are epithelioid in morphology and have high mitotic activity. This type has the poorest prognosis. [2],[4],[7]

In addition to the microscopic evaluation of these tumors, a recent immunohistochemical profile suggests that EMA, cytokeratin AE1/AE3 and E-cadherin, in combination with CD34 negativity, are the most useful and sensitive markers for diagnosing SS. [4],[8] CD34 may rarely be positive and Bcl-2 and MIC-2(CD 99) usually positive in SS as was seen in our case. [9]

SS, on X-ray films appear as round or oval lobulated swellings or masses of moderate density. Although, half of the X-rays are normal, calcification is seen in up to 30% of the cases. Calcification is usually eccentric or peripheral within the soft tissue mass, represented by multiple small spotty radiopacities. Extensive calcification is associated with an improved prognosis. [2],[4],[10] SS appear as a heterogeneous soft tissue mass with attenuation, similar or slightly less than that of the muscle, as seen on computed tomography scans. Area of necrosis or hemorrhage presenting as lower density areas are common. [2] Magnetic resonance imaging is the optimal radiological modality for assessing the characteristics and the extent of SS. Intermixed areas of low, intermediate, and high signal intensities on long repetition time images have been marked as the triple sign apparently resulting in a mixture of solid cellular elements, hemorrhage or necrosis, and calcified or fibrotic regions with hemorrhage, fluid levels, and septa creating the bowl of grapes sign. This triple sign has been described to be occurring in 35% to 57% tumors. However, the triple sign is also seen in other soft tissue tumors; therefore, this finding alone lacks a high degree of specificity. [4],[11] Angiographic studies of the SS frequently reveal a prominent vascularity of primary lesions and metastatic disease. [4]

The treatment of choice for SS is a wide local excision with negative surgical margins to minimize the chances of recurrence. [2],[4],[5] Radiotherapy is useful in local control of diseases, especially in gross residual tumor. Adjuvant chemotherapy mostly consists of regimens with combination of alkylating agents (cyclophosphamide or ifosphamide) and anthracyclines. Although chemotherapy is useful in disease control, their overall benefit in survival rate is controversial. [5] The 5-year survival rate for intermediate to high-grade sarcoma is 36% to 76%. [4] The clinical course of SS is characterized by a high rate of local recurrence (30-50%) and metastatic disease. The majority of metastases occur within the first 2 years to 5 years after treatment. [2],[4]

Various adverse prognostic features in SS include metastasis, invasiveness, positive surgical margins, high histological grade, and poor differentiation. While, SYT-SSX2 gene fusion, monophasic SS, size less than 5 cm, less than 15 years of age, and distal extremity location have better prognostic outcomes; the significance of these findings is still debated. [2],[4],[5]

Our case presentation shows an atypical SS location within the abdominal wall. It should be considered as one of the differential diagnosis in anterior abdominal wall tumors.

 
  References Top

1.Fisher C. Synovial sarcoma. Curr Diagn Pathol 1994;1:13-8.  Back to cited text no. 1
    
2.Saif AH. Primary synovial sarcoma of the abdominal wall: A case report and review of literature. J Family Community Med 2008;15:123-5.  Back to cited text no. 2
    
3.Armstrong AV Jr, Aedo A, Phelps S. Synovial sarcoma: A case report. Clin Podiatr Med Surg 2008;25:167-81.  Back to cited text no. 3
    
4.McNeill J, Nguyen YV. Synovial sarcoma of the abdominal wall. J Radiol Case Rep 2007;2:108.  Back to cited text no. 4
    
5.Okcu MF, Munsell M, Treuner J, Mattke A, Pappo A, Cain A, et al. Synovial Sarcoma of childhood and adolescence: A multicenter, multivariate analysis of outcome. J Clin Oncol 2003;21:1602-11.  Back to cited text no. 5
    
6.Murphey MD, Gibson MS, Jennings BT, Crespo-Rodriguez AM, Fanburg-Smith J, Gajeswski DA. From the archives of the AFIP: Imaging of synovial sarcoma with radiologic-pathologic correlation. Radiographics 2006;26:1543-65.  Back to cited text no. 6
    
7.Enzinger FM, Weiss SW. Soft tissue tumors. 3 rd ed. St. Louis, MO: Mosby-Year Book, Inc; 1995. p. 757-86.  Back to cited text no. 7
    
8.Pelmus M, Guillou L, Hostein I, Sierankowski G, Lussan C, Coindre JM. Monophasic fibrous and poorly differentiated synovial sarcoma: Immunohistochemical reassessment of 60 t (X; 18) (SYT-SSX)-positive cases. Am J Surg Pathol 2002;26:1434-40.  Back to cited text no. 8
    
9.Heim-Hall J, Yohe SL. Application of immunohistochemistry to soft tissue neoplasms. Arch Pathol Lab Med 2008;132:476-89.  Back to cited text no. 9
    
10.Horowitz AL, Resnick D, Watson RC. The roentgen features of synovial sarcomas. Clin Radiol 1973;24:481-4.  Back to cited text no. 10
    
11.Jones BC, Sundaram M, Kransdorf MJ. Synovial sarcoma: MR imaging findings in 34 patients. AJR Am J Roentgenol 1993;161:827-30.  Back to cited text no. 11
    


    Figures

  [Figure 1], [Figure 2]



 

Top
 
 
  Search
 
Similar in PUBMED
   Search Pubmed for
   Search in Google Scholar for
 Related articles
Access Statistics
Email Alert *
Add to My List *
* Registration required (free)

 
  In this article
Abstract
Introduction
Case Report
Discussion
References
Article Figures

 Article Access Statistics
    Viewed1882    
    Printed57    
    Emailed0    
    PDF Downloaded76    
    Comments [Add]    

Recommend this journal