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Table of Contents 
CASE REPORT
Year : 2014  |  Volume : 59  |  Issue : 3  |  Page : 297-298
Myxoinflammatory fibroblastic sarcoma: An uncommon tumour at an unusual site


1 Department of Pathology, National Institute of Pathology, ICMR, Safdarjung Hospital Campus, New Delhi, India
2 Department of Dermatology, Safdarjung Hospital, New Delhi, India

Date of Web Publication28-Apr-2014

Correspondence Address:
Dr. Avninder Singh
213-Pocket B Sukhdev Vihar, New Delhi - 110 025
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0019-5154.131412

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   Abstract 

Myxoinflammatory fibroblastic sarcoma is a low grade sarcoma that is composed of a mixed inflammatory infiltrate along with spindled, epithelioid and bizarre appearing cells in a background of hyaline and myxoid zones. Seen affecting the distal extremities commonly, with an equal sex predilection, these tumors are rare and require an extensive immunohistochemical work up for proper diagnosis. They have a tendency to recur.


Keywords: Hodgkins lymphoma, Myxoinflammatory fibroblastic sarcoma, Myxoid Liposarcoma


How to cite this article:
Mallya V, Singh A, Siraj F, Ramesh V. Myxoinflammatory fibroblastic sarcoma: An uncommon tumour at an unusual site. Indian J Dermatol 2014;59:297-8

How to cite this URL:
Mallya V, Singh A, Siraj F, Ramesh V. Myxoinflammatory fibroblastic sarcoma: An uncommon tumour at an unusual site. Indian J Dermatol [serial online] 2014 [cited 2019 Jul 18];59:297-8. Available from: http://www.e-ijd.org/text.asp?2014/59/3/297/131412

What was known?
It is a low grade sarcoma that is composed of a mixed infl ammatory infi ltrate along with spindled, epithelioid and bizarre appearing cells in a background of hyaline and myxoid zones.


Myxoinflammatory fibroblastic sarcoma is a rare low grade sarcoma affecting the males and females equally in the fourth and fifth decades of life. Described for the first time in three independent reports in 1998 by Montgomery et al.[1] , Michal [2] and Meis Kindblom et al.[3] , this tumor mostly affects the hands and feet.

We report the case of a 60 year old male, who presented to the dermatology department with two papulonodular lesions over the popliteal fossa [Figure 1]. The lesions were asymptomatic and present since two years. There was no other swelling elsewhere on the body. The lesions were excised and received at our department for histopathology. On microscopy, an inflammatory infiltrate comprising of lymphocytes, plasma cells, few eosinophils and neutrophils was seen, associated with alternating hyaline and myxoid zones. Within the stroma, there were spindled cells, large polygonal cells, many of which had macronucleoli resembling the Reed-Sternberg-like cells. [Figure 2]. Multivacuolated lipoblast like cells were also seen. Mitotic count was low, though nuclear atypia and enlargement was noted. The tumour was present within the dermis. The myxoid areas stained blue with Alcian blue staining. On immunohistochemistry (IHC), the neoplastic cells were strongly positive for vimentin [Figure 3] and variably positive for cluster of differentiation CD68, CD34 and Epidermal growth factor receptor (EGFR). They were negative for S100, SMA, CD15 and CD30. Based on the hemotoxylin and eosin staining (H and E) and IHC, a diagnosis of Myxoinflammatory fibroblastic sarcoma was made. Myxoinflammatory fibroblastic sarcoma is a low grade sarcoma involving the distal extremities and presenting as a slow growing, poorly defined painless mass, with reduced mobility. [4] Characterized histologically as poorly circumscribed masses arising in the subcutaneous tissue with dermal involvement and composed of three types of cells, namely spindle cells, large bizarre ganglion like cells and cells resembling lipoblasts against a background of dense acute and chronic inflammatory infiltrate. [4],[5] The tumor cells are positive for vimentin, CD68 and CD34 and negative for SMA and S100. [4],[5] Microscopically, this entity needs to be distinguished from myxofibrosarcoma. Absence of curvilinear vessels and high grade.
Figure 1: Clinical photo showing papulonodular lesions over the popliteal fossa

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Figure 2: Photomicrograph showing spindled and polygonal cells with macronucleoli against a background of myxoid and hyaline zones, multivacuolated lipoblast like cells are also seen. (H and E, ×400)

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Figure 3: IHC showing the neoplastic cells to be strongly positive for vimentin. (IHC, ×400)

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Pleomorphism excluded a diagnosis of myxofibrosarcoma. [4],[5] Negative CD15 and CD30 staining of the Reed-Sternberg-like cells ruled out the diagnosis of Hodgkins lymphoma. [4],[5] True lipoblasts and prominent capillary vessels characteristic of myxoid liposarcoma were absent, hence the possibility of myxoid liposarcoma was also ruled out. Absence of osteoclast like giant cells and presence of areas of Myxoinflammatory fibroblastic sarcoma helped in excluding the possibility of Giant cell tumour of tendon sheath. [1],[2],[3] Thus, it is important that this entity be recognized and a full clinical, histological and immunohistochemical work up done for its proper diagnosis. This will aid proper management, avoid unnecessary invasive procedures and a wrong diagnosis of it being a benign or an infectious entity. [4]


   Acknowledgement Top


The authors wish to thank Dr Eduarado Calonje, Director of Diagnostic Dermatopathology, St John's Institute of Dermatology, London for reviewing the slides.

 
   References Top

1.Montgomery EA, Devaney KO, Giordano TJ, Weiss SW. Inflammatory myxohyaline tumour of distal extremities with virocyte or Reed-Sternberg-like cells: A distinctive lesion with features simulating inflammatory conditions, Hodgkins disease, and various sarcomas. Mod Pathol 1998;11:384-91.  Back to cited text no. 1
    
2.Michal M. Inflammatory myxoid tumour of the soft parts with bizarre giant cells. Pathol Res Pract 1998;194:529-33.  Back to cited text no. 2
[PUBMED]    
3.Meis-Kindblom J, Kindblom LG. Acral myxoinflammatory fibroblastic sarcoma: A low-grade tumour of the hands and feet. Am J Surg Pathol 1998;22:911-24.  Back to cited text no. 3
    
4.Hassanein AM, Atkinson SP, Al-Quran SZ, Jain SM, Reith JD. Acral myxoinflammatory fibroblastic sarcomas: Are they all low-grade neoplasms? J Cutan Pathol 2008;35:186-91.  Back to cited text no. 4
    
5.Jurcic V, Zidar A, Montiel MD, Frkovic-Grazio S, Nayler SJ, Cooper K, et al. Myxoinflammatory fibroblastic sarcoma: A tumor not restricted to acral sites. Ann Diagn Pathol 2002;6:272-80.  Back to cited text no. 5
    

What is new?
These tumors are rare and require a high index of suspicion and extensive immunohistochemical work up for proper diagnosis.


    Figures

  [Figure 1], [Figure 2], [Figure 3]



 

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