LGCmain
Indian Journal of Pathology and Microbiology
Home About us Instructions Submission Subscribe Advertise Contact e-Alerts Ahead Of Print Login 
Users Online: 2229
Print this page  Email this page Bookmark this page Small font sizeDefault font sizeIncrease font size
IJPM is coming out with a Special issue on "Genitourinary & Gynecological pathology including Breast". Please submit your articles for these issues


 
  Table of Contents    
LETTERS TO EDITOR  
Year : 2019  |  Volume : 62  |  Issue : 4  |  Page : 643-645
Recurrent monophasic synovial sarcoma in a child


Department of Pathology, IPGME and R, Kolkata, West Bengal, India

Click here for correspondence address and email

Date of Web Publication14-Oct-2019
 

How to cite this article:
Chaudhuri PM, Islam N, Das M, Chatterjee U, Datta C. Recurrent monophasic synovial sarcoma in a child. Indian J Pathol Microbiol 2019;62:643-5

How to cite this URL:
Chaudhuri PM, Islam N, Das M, Chatterjee U, Datta C. Recurrent monophasic synovial sarcoma in a child. Indian J Pathol Microbiol [serial online] 2019 [cited 2019 Dec 8];62:643-5. Available from: http://www.ijpmonline.org/text.asp?2019/62/4/643/269073




Editor,

Synovial sarcoma (SS) is the most common non -habdomyosarcomatous sarcoma in children.[1] Usually found in adolescents and young adults, it is rare below the age of 2 years, with only a handful of cases describing this entity in infants.[2],[3],[4] It commonly occurs around the knee joint. The histomorphology of monophasic synovial sarcoma (MSS) overlaps with that of fibrosarcoma and malignant peripheral nerve sheath tumor (MPNST). Infantile fibrosarcoma (IFS) is a distinct entity seen in infants which shares similar histological features with MSS.[5] MSS and IFS differ considerably in treatment and prognosis, necessitating differentiation from each other.

We describe a case of a recurrent mass in the popliteal region in a 3-year-old girl. X-ray showed a heterogeneous soft tissue mass in the popliteal region with focal calcifications, with no changes in the underlying long bone or periosteum. At 6 months of age, she underwent surgery for a tumor in the same location. The relevant clinical and histological details were lost by the parents. In view of recurrence, a clinical diagnosis of IFS was made and she underwent complete excision of the tumor.


   Pathological Findings Top


The excised tumor measured 5 × 4 × 4 cm with muscular attachments and was grey-white on sectioning. Histopathological sections showed short sweeping bundles of monomorphic spindle cells, with three mitotic figures per 10 high-power fields, and a few myxoid areas without inflammatory cell infiltration or extramedullary hematopoiesis. Differential diagnoses of recurrent IFS and MPNST were considered.

Immunohistochemistry (IHC) showed strong and diffuse positivity for CD99 and Bcl2, strong nuclear expression of TLE1, positive EMA, and focally positive CK expression. IHC was negative for CD34, S100, myogenin, and desmin [Figure 1]. Based on these, diagnosis of MSS was made.
Figure 1: (a) X-ray showing heterogeneous, soft tissue mass in popliteal region with focal calcification, no changes in bone or periosteum. (b) Low-power view showing sweeping bundles of monomorphic spindle cells (H and E stain). (c) High-power view showing oval to spindle cells with hyperchromatic, uniform nuclei (H and E stain). (d) Low-power view showing strong nuclear positivity for TLE1; inset showing high-power view. (e) Low-power view showing strong Bcl 2 positivity; inset showing high-power view. (f) Low-power view showing strong cytoplasmic and membrane staining for CD99; inset showing high-power view

Click here to view


The child received cyclophosphamide and actinomycin-D as part of postoperative chemotherapy. She is doing well on a follow-up of 2 years.

SS is the second most common sarcoma in children, after rhabdomyosarcoma.[1] Histologically, MSS shares similarities with other soft tissue sarcomas such as MPNST and IFS. MSS needs to be differentiated from IFS as they differ in their treatment protocols and prognosis. SS requires complete excision followed by radiotherapy and chemotherapy. Surgery alone suffices in case of IFS. SS can metastasize to the lungs and bones, and 5-year survival rate in children is 83%.[6] It often recurs around the knee joint and at the back. On the other hand, IFS rarely metastasizes, and 5-year survival rate is almost 100%. Recurrence rate for IFS is 5%–50%.

SS is a slow-growing painless mass most prevalent in adolescents and young adults. But cases have been reported in infants, neonates, and even preterm neonates.[2],[3],[4] It has a predilection for the lower limbs, usually occurring around the knee joint. Grossly, it is well-circumscribed, pseudo-encapsulated, and cut section is soft and grey-white. Fibrosarcoma, on the other hand, is a rapidly growing mass found in the superficial and deep soft tissues of distal extremities, less commonly in the trunk and head and neck. Fibrosarcoma is divided into two groups, the adult and the infantile types. The infantile type develops within the first 2 years of life and is often congenital. Osseous erosion may be present. Grossly, it is poorly circumscribed, lobulated, and infiltrates soft tissues. The cut surface is fleshy and tan.

Although there is histological overlap between SS and IFS, the former has certain distinct features. SS can be monophasic or biphasic. Biphasic variant has both epithelial and spindle cell components. MSS has more ovoid-appearing cells with bland but hyperchromatic nuclei arranged in sheets or fascicles. There may be foci of cells with eosinophilic cytoplasm, ropy collagen, and mast cells. There may be evidence of calcification and ossification. Mitotic activity is variable. On the other hand, IFS is composed of oval to spindle-shaped cells arranged in sheets or herringbone fascicles. Cells are monomorphic with bland nuclei. Mitotic activity is brisk. Myxoid foci can be present. Chronic inflammatory cell infiltration consisting of lymphocytes and plasma cells as well as extramedullary hematopoiesis may exist. Other histological variations include hyalinization, collagenization, fibrin thrombi, hemorrhage, necrosis, calcification, or hemangiopericytoma-like vascular pattern.

IHC and genetic studies are necessary to definitively differentiate MSS from IFS. In case of SS, TLE1 is a highly sensitive marker.[7] In a study by Rekhi et al., the overall sensitivity of TLE1 in diagnosis of SS was 95.2%.[8] SS also shows strong and diffuse positivity for Bcl 2. Cytokeratin and EMA are also positive, sometimes with patchy expression. The tumor shows focal positivity for CD99, S100, CD56, and calponin. CD34 is negative. IFS shows positivity for SMA and desmin. There is occasional expression of CD34, CK, and S100. It is negative for TLE1.

[Table 1] lists the important clinicopathological differences between SS and IFS. The detection of t(X: 18)(p11;q11) and SSX and SS18 (SYT) gene fusion is highly sensitive and specific for identifying SS.[9],[10] TLE1 has emerged as a good substitute for t(X: 18) and can be used as a surrogate marker to differentiate SS from other histological mimics in the setting of limited molecular facilities. IFS shows t(12:15)(p13;q25) and ETV6-NTRK3 gene fusion (also shared by cellular congenital mesoblastic nephroma).
Table 1: Comparison between synovial sarcoma and infantile fibrosarcoma

Click here to view


In conclusion, SS is an important differential diagnosis of sarcomas in infants and young children, overlapping morphologically with IFS, which is more common in infants and has better prognosis and different treatment protocol. Histopathology combined with IHC and genetic studies are important to distinguish between these two entities.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
   References Top

1.
Qureshi SS, Bhagat M. Non-rhabdomyosarcoma soft-tissue sarcomas in children: Contemporary appraisal and experience from a single centre. J Indian Assoc Pediatr Surg 2015;20:165-9.  Back to cited text no. 1
[PUBMED]  [Full text]  
2.
Radhakrishnan V, Joel A, Sundersingh S, Raja A. Synovial sarcoma in a neonate. Indian Pediatr 2016;53:345-6.  Back to cited text no. 2
    
3.
Yokoyama H, Yamamoto T, Satsuma S, Kobayashi D, Kurosaka M, Yoshiya S. Biphasic synovial sarcoma in a 13-month-old girl. Kobe J Med Sci 2002;48:55-8.  Back to cited text no. 3
    
4.
Kose D, Annagur A, Erol C, Uoraş S, Koksal Y. Synovial sarcoma in a premature newborn. Pediatr Int 2014;56:17-20.  Back to cited text no. 4
    
5.
Willeke F, Mechtersheimer G, Schwarzbach M, Weitz J, Zimmer D, Lehnert T, et al. Detection of SYT-SSX1/2 fusion transcripts by reverse transcriptase-polymerase chain reaction (RT-PCR) is a valuable diagnostic tool in synovial sarcoma. Eur J Cancer 1998;34:2087-93.  Back to cited text no. 5
    
6.
Sultan I, Rodriguez-Galindo C, Saab R, Yasir S, Casanova M, Ferrari A. Comparing children and adults with synovial sarcoma in the Surveillance, Epidemiology, and End Results program, 1983 to 2005: An analysis of 1268 patients. Cancer 2009;115:3537-47.  Back to cited text no. 6
    
7.
Foo WC, Cruise MW, Wick MR, Hornick JL. Immunohistochemical staining for TLE1 distinguishes synovial sarcoma from histologic mimics. Am J Clin Pathol 2011;135:839-44.  Back to cited text no. 7
    
8.
Rekhi B, Basak R, Desai SB, Jambhekar NA. Immunohistochemical validation of TLE1, a novel marker, for synovial sarcomas. Indian J Med Res 2012;136:766-75.  Back to cited text no. 8
[PUBMED]  [Full text]  
9.
Norlelawati AT, Mohd Danial G, Nora H, Nadia O, Zatur Rawihah K, Nor Zamzila A, et al. Detection of SYT-SSX mutant transcripts in formalin-fixed paraffin-embedded sarcoma tissues using one-step reverse transcriptase real-time PCR. Malays J Pathol 2016;38:11-8.  Back to cited text no. 9
    
10.
Ladanyi M, Antonescu CR, Leung DH, Woodruff JM, Kawai A, Healey JH, et al. Impact of SYT-SSX fusion type on the clinical behavior of synovial sarcoma: A multi-institutional retrospective study of 243 patients. Cancer Res 2002;62:135-40.  Back to cited text no. 10
    

Top
Correspondence Address:
Uttara Chatterjee
Department of Pathology, IPGME and R, Kolkata, West Bengal
India
Login to access the Email id

Source of Support: None, Conflict of Interest: None


DOI: 10.4103/IJPM.IJPM_510_18

Rights and Permissions


    Figures

  [Figure 1]
 
 
    Tables

  [Table 1]



 

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


    Pathological Fin...
    References
    Article Figures
    Article Tables

 Article Access Statistics
    Viewed181    
    Printed3    
    Emailed0    
    PDF Downloaded12    
    Comments [Add]    

Recommend this journal