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  Table of Contents    
CASE REPORT  
Year : 2015  |  Volume : 58  |  Issue : 3  |  Page : 335-337
Sclerosing rhabdomyosarcoma presenting on the knee-joint


1 Department of Pathology, Mindong Hospital of Fujian Medical University, Fudong Hospit Ningde, Fujian, China
2 Department of Medicine, Mindong Hospital of Fujian Medical University, Fudong Hospit Ningde, Fujian, China

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Date of Web Publication14-Aug-2015
 

   Abstract 

Sclerosing rhabdomyosarcoma is a rare pathological diagnosis that easily misdiagnosed. The majority of cases reported the tumor increased rapidly in size and the Ki-67 proliferation index ranged from 10% to 60%. Here, we report the first case of the tumor increased very slowly for 20 years and the Ki-67 proliferation index was lower than 2%, and discuss its histological features and immunohistochemical reactivity with Desmin and Ki-67 and so on.

Keywords: Ki-67, sarcoma, sclerosing rhabdomyosarcoma

How to cite this article:
Zheng Y, Liu X, Mao Y, Lin M, Li T. Sclerosing rhabdomyosarcoma presenting on the knee-joint. Indian J Pathol Microbiol 2015;58:335-7

How to cite this URL:
Zheng Y, Liu X, Mao Y, Lin M, Li T. Sclerosing rhabdomyosarcoma presenting on the knee-joint. Indian J Pathol Microbiol [serial online] 2015 [cited 2020 Jun 6];58:335-7. Available from: http://www.ijpmonline.org/text.asp?2015/58/3/335/162867



   Introduction Top


Sclerosing rhabdomyosarcoma (SRMS) is exceedingly rare in all soft tissue tumors. In the current WHO soft tissue tumor classification, rhabdomyosarcoma (RMS) is subdivided into three groups: Embryonal RMS, alveolar RMS and pleomorphic RMS. [1] To the best of our knowledge, 41 cases (the present case is not included) of SRMS have been reported. [2],[3],[4],[5],[6],[7],[8] The cases have reported the tumor increased rapidly in size and the Ki-67 proliferation index ranged from 10% to 60%. [2] Here, we report the first case of SRMS, which the tumor increased very slowly for 20 years. And the Ki-67 proliferation index was lower than 2%.


   Case Report Top


A 35-year-old male presented with a swelling on his right knee-joint area about 2 cm size with 20 years' duration, which didn't cause for concern. For the last 2 months, the swelling increased rapidly in size. Physical examination revealed a 4 cm mass in subcutaneous which felt firm and adhered to the adjacent tissue, the mass was relatively circumscribed. Then the tomor was excised and was sent for pathological examination.

Gross the resect tumor measured 4.0 cm 3.8 cm 3.5 cm, and was relatively circumscribed, the cut surface showed solidly and grey-white in colour [Figure 1]. Microscopic examination showed that the tumor included a lot of prominent hyalinzing matrix, often mimicking osteoid or chondroid [Figure 2]. In some dense areas, the tumor cells arranged into microalveolar or cords structures. In addition, the tumor cells often exhibited abundant eosinophilic cytoplasm and irregular nuclei deeply stained [Figure 3].
Figure 1: The cut surface showed solidly, circumscribed and grey-white in colour


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Figure 2: Prominent hyalinizing matrix, mimicking osteoid (×100)


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Figure 3: The tumor cells showed eosinophilic cytoplasm, irregular nuclei that deeply stained pattern in abundant hyalinizing matrix (×200)


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Immunostaining showed that the tumor was positive for Vimentin, Desmin [Figure 4] and MyoD1, and was negative for CK, p63, EMA, NSE, CD45, CD30, S-100, CD99, Myoglobin, CD68, CD34, CD163, CD31, α-SMA. Ki-67 was expressed <2% of all tumor cells [Figure 5]. According to the morphological and immunohistochemical findings, the tumor was diagnosed as a SRMS.
Figure 4: Immunohistochemical examination with Desmin confirmed the tumor cells deriving from striated muscle (×400)


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Figure 5: Less than 2% of tumor cells were immunoreactive for Ki-67 antibody (×400)


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   Discussion Top


The SRMS is first described by Mentzel and Katenkamp [9] and is still controversial. According to the related case reports, SRMS can occur over a broad age (from 0.3 to 70 years). The most commonly involved sites are the extremities and the head and neck. Because of the rarity, SRMS is misdiagnosed easily. The present reported case, clinician misdiagnosed the tumor as a lipoma and excised it without the database of diagnostic imaging. To our knowledge, this is the first reportance that the tumor have increased for 20 years and Ki-67 proliferation index was lower than 2%. The patient who we followed-up about half a year didn't make any medical treatment, and the disease didn't recur. Some authors considered that SRMS was a malignant mesenchymoma, but we think there may be an exception. Gene detection may help us resolve the confusion.

 
   References Top

1.
Fletcher CD, Unni KK, Mertens F. Skeletal muscle tumors, WHO classification of tumours: Pathology and genetics of tumours of soft tissue and bone. 3 rd ed. Lyon: IARC Press; 2006. p. 141-54.  Back to cited text no. 1
    
2.
Robinson JC, Richardson MS, Neville BW, Day TA, Chi AC. Sclerosing rhabdomyosarcoma: Report of a case arising in the head and neck of an adult and review of the literature. Head Neck Pathol 2013;7:193-202.  Back to cited text no. 2
    
3.
Kuhnen C, Herter P, Leuschner I, Mentzel T, Druecke D, Jaworska M, et al. Sclerosing pseudovascular rhabdomyosarcoma-immunohistochemical, ultrastructural, and genetic findings indicating a distinct subtype of rhabdomyosarcoma. Virchows Arch 2006;449:572-8.  Back to cited text no. 3
    
4.
Bouron-Dal Soglio D, Rougemont AL, Absi R, Barrette S, Montpetit A, Fetni R, et al. SNP genotyping of a sclerosing rhabdomyosarcoma: Reveals highly aneuploid profile and a specific MDM2/HMGA2 amplification. Hum Pathol 2009;40:1347-52.  Back to cited text no. 4
    
5.
Cantley RL, Cimbaluk D, Reddy V, Iacusso C, Gattuso P. Fine-needle aspiration diagnosis of a metastatic adult sclerosing rhabdomyosarcoma in a lymph node. Diagn Cytopathol 2010;38:761-4.  Back to cited text no. 5
    
6.
Gavino AC, Spears MD, Peng Y. Sclerosing spindle cell rhabdomyosarcoma in an adult: Report of a new case and review of the literature. Int J Surg Pathol 2010;18:394-7.  Back to cited text no. 6
    
7.
Martorell M, Ortiz CM, Garcia JA. Testicular fusocellular rhabdomyosarcoma as ametastasis of elbow sclerosing rhabdomyosarcoma: A clinicopathologic, immunohistochemical and molecular study of one case. Diagn Pathol 2010;5:52.  Back to cited text no. 7
    
8.
Folpe AL, McKenney JK, Bridge JA, Weiss SW. Sclerosing rhabdomyosarcoma in adults: Report of four cases of a hyalinizing, matrix-rich variant of rhabdomyosarcoma that may be confused with osteosarcoma, chondrosarcoma, or angiosarcoma. Am J Surg Pathol 2002;26:1175-83.  Back to cited text no. 8
    
9.
Mentzel T, Katenkamp D. Sclerosing, pseudovascular rhabdomyosarcoma in adults. Clinicopathological and immunohistochemical analysis of three cases. Virchows Arch 2000;436:305-11.  Back to cited text no. 9
    

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Correspondence Address:
Dr. Yuanyin Zheng
Department of Pathology, Mindong Hospital of Fujian Medical University, Fu'an - 355 000, Ningde, Fujian
China
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0377-4929.162867

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    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]



 

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