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CASE REPORT
Year : 2010  |  Volume : 1  |  Issue : 1  |  Page : 24-26 Table of Contents     

Fibromyxoid sarcoma of the leg


1 Department of Dermatology and Allergology, Hospital Dresden-Friedrichstadt, Academic Teaching Hospital of the Technical University of Dresden, 01067 Dresden, Germany
2 Institute of Pathology, Hospital Dresden-Friedrichstadt, Academic Teaching Hospital of the Technical University of Dresden, 01067 Dresden, Germany

Date of Web Publication26-Nov-2010

Correspondence Address:
Uwe Wollina
Department of Dermatology and Allergology, Hospital Dresden-Friedrichstadt, Academic Teaching Hospital of the Technical University of Dresden, Dresden
Germany
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/2229-5178.73254

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   Abstract 

A 48-year-old female with an atypical plaque-like lesion of the lower leg is presented in this article. Histologic investigation revealed a rare low-grade fibromyxoid sarcoma (pT1a cN0 cM0; stage Ia) of suprafascial localization. Staging of the patient did not reveal metastatic spread. The tumor was surgically removed with wide safety margins. The defect was closed using a mesh graft transplant and vacuum-assisted closure. Healing was complete. Regular follow-up for at least 5 years is recommended. Besides the rareness of this tumor, this case is also remarkable because of the localization on the lower leg and the suprafascial soft tissue.

Keywords: Fibromyxoid sarcoma, histology, surgery


How to cite this article:
Wollina U, Runge J, Schonlebe J. Fibromyxoid sarcoma of the leg. Indian Dermatol Online J 2010;1:24-6

How to cite this URL:
Wollina U, Runge J, Schonlebe J. Fibromyxoid sarcoma of the leg. Indian Dermatol Online J [serial online] 2010 [cited 2020 Jul 10];1:24-6. Available from: http://www.idoj.in/text.asp?2010/1/1/24/73254


   Introduction Top


Sarcomas are mesenchymal neoplasms with various lines of differentiation, i.e., fibrocytic, myogenic, neurogenic, vascular, chondro-osseus, or undefined. The low-grade fibromyxoid sarcoma (LGFS) is a very rare entity. It is a spindle-cell tumor composed of collagen-rich and myxoid parts. [1],[2] About 40% of these tumors also develop collagen rosettes. [3]

LGFS prefers subfascial soft tissue layers on the trunk and proximal extremities in younger adults but can also develop in internal organs. Pediatric cases have also been reported. [1],[2],[3]

Herein, we present a 48-year-old female patient who developed a LGFS on the lower leg. The clinical presentation, histopathology, surgical treatment, and follow-up are discussed.


   Case Report Top


A 48-year-old woman was referred to our department because she had developed a slow-growing plaque below the left knee for 2 years. A diagnostic biopsy had been taken by the referring dermatologist that suggested an LGFS.

On examination we found an otherwise healthy, slim, woman with a symptomless firm subcutaneous plaque of about 2 cm size on the anterior aspect of the left lower leg [Figure 1].There was some bluish discoloration and circumscribed ulceration but no erythema or warmth.
Figure 1 :Low-grade fibromyxoid sarcoma of the left lower leg. (a) Overview and (b) the ulcerated honeycomb-like plaque

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Routine laboratory tests were unremarkable. Thoracic dual-energy x-ray and abdominal and lymph node ultrasound excluded a metastatic spread. Histology revealed a spindle-cell tumor, with mild atypia and fibroblast-like morphology [Figure 2]. The eosinophilic cells did not show increased mitotic activity. Cells were arranged in a whorled or plexifiorm pattern, with alternating collagenous stroma and myxoid zones. The cells stained positively for vimentin, but were negative for CD34 and S100. The covering epidermis was partially ulcerated. A final diagnosis of Low GradeF ibromyxoid Sarcoma was reached. Under general anesthesia, the tumor was surgically removed with a wide safety margin (>3 cm). The defect was covered by a mesh graft transplant covered by vacuum-assisted closure (VAC TM ; KCI International) [Figure 3]. Compression stockings were prescribed to protect the transplant and prevent leg edema. A regular follow-up for at least 5 years was recommended after consultation with the referring dermatologist.
Figure 2 :Histopathology. (a) Overview (H&E, ×4) and (b) detail (H&E, ×40)

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Figure 3 :Surgical procedure. (a) Wide excision of suprafascial soft tissue; (b) mesh graft transplantation; and (c) placement of microporous white sponge for vacuum-assisted closure above the transplant

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   Discussion Top


LGFS is a very rare and distinctive type of fibrosarcoma that was first described by Evans in 1987. [1] There is a discrepancy between the bland histologic features with sparse mitotic figures and absent or mild nuclear and cellular pleomorphism and the anaplasia. The hyalinizing spindle-cell tumor with giant rosettes is considered a subtype of LGFS. [1],[2],[3] The tumor is further characterized by t(7;16)(q34;p11) translocation and fusion of FUS and CREB3L1 genes. [4],[5] Tumor cell phenotype is positive for vimentin, EMA, CD99, and bcl-2, but negative for CD34, SMA, S-100, desmin, keratins, neuron-specific enolase, and CD177. [5] The differential diagnosis includes other types of sarcomas, myxoma, neurofibroma, peripheral sheath tumor, histocytoma, desmoid tumor, and others. [2],[3],[5]

LGFS usually presents as a painless, slow-growing, soft tissue malignancy. The diagnosis often is delayed - mainly because the patients do not seek treatment early. Folpe et al. (2000) reported that in 15% of patients a histologic diagnosis was delayed by >5 years. In our case the delay was >2 years. [3]

Although the histopathologic features suggest a low-grade malignancy, local recurrence is seen in more than 50% of patients and metastasis occurs in 6% of patients. [3] Tumor cell dormancy is responsible for very late metastasis in some patients, with 45 years being the longest period observed between primary surgery and metastasis. [6],[7] Therefore, patients should be encouraged to have regular follow-up.

The present case did not show any subfascial involvement. Complete surgery with wide margins (>3 cm) is the most important procedure. We used a combination of mesh graft transplantation and vacuum-assisted closure to cover the large defect. It has been demonstrated recently that such a combination, with microporous sponge for vacuum-assisted closure, results in a significantly improved take rate. [8] The prognosis for superficial LGFS seems to be better than that for deep LGFS. [9]

 
   References Top

1.Evans HL. Low-grade fibromyxoid sarcoma. A report of two metastasizing neoplasms having a deceptively benign appearance. Am J Clin Pathol 1987;88:615-9.  Back to cited text no. 1
    
2.Goodland JR, Mentzel T, Fletcher CD. Low grade fibromyxoid sarcoma: Clinicopathological analysis of eleven new cases in support of a distinct entity. Histopathology 1995;26:229-33.  Back to cited text no. 2
    
3.Folpe AL, Lane KL, Paull G, Weis SW. Low-grade fibromyxoid sarcoma and hyalinizing spindle cell tumor with giant rosettes: A clinicopathologic study of 73 cases supporting their identity and assessing the impact of high-grade areas. Am J Surg Pathol 2000;24:1353-60.  Back to cited text no. 3
    
4.Panagopoulos I, Storlazzi CT, Fletcher CD, Fletcher JA, Nascimento A, Domanski HA, et al. The chimeric FUS/CREB3L2 gene is specific for low-grade fibromyxoid sarcoma. Genes Chromosomes Cancer 2004;40:218-28.  Back to cited text no. 4
    
5.Guillou L, Banhattar J, Gengler C, Gallagher G, Ranchθre-Vince D, Collin F, et al. Translocation-positive low grade fibromyxoid sarcoma: Clinicopathologic and molecular analysis of a series expanding the morphologic spectrum and suggesting potential relationship to sclerosing epitheloid fibrosarcoma: A study from the French Sarcoma Group. Am Surg Pathol 2007;31:1387-402.  Back to cited text no. 5
    
6.Wu X, Petrovic V, Torode IP, Chow CW. Low grade fibromyxoid sarcoma: Problems in the diagnosis and management of a malignant tumor with bland histological appearance. Pathology 2009;41:155-60.  Back to cited text no. 6
    
7.Lee BJ, Park WS, Jin JM, Ha CW, Lee SH. Low grade fibromyxoid sarcoma in thigh. Clin Orthop Surg 2009;1:240-3.  Back to cited text no. 7
    
8.Kφrber A, Franckson T, Grabbe S, Dissemond J. Vacuum assisted closure device improves the take of mesh grafts in chronic leg ulcer patients. Dermatology 2008;216:250-6.  Back to cited text no. 8
    
9.Billings SD, Giblen G, Fanburg-Smith JC. Superficial low-grade fibromyxoid sarcoma (Evans tumor): A clinicopathologic analysis of 19 cases with a unique observation in the pediatric population. Am J Surg Pathol 2005;29:204-10.  Back to cited text no. 9
    


    Figures

  [Figure 1], [Figure 2], [Figure 3]


This article has been cited by
1 A 10-year analysis of cutaneous mesenchymal tumors (sarcomas and related entities) in a skin cancer center
Uwe Wollina,André Koch,Gesina Hansel,Jaqueline Schönlebe,Thomas Kittner,Friedemann Pabst,Gunter Haroske,Andreas Nowak
International Journal of Dermatology. 2013; : n/a
[Pubmed] | [DOI]



 

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