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 Table of Contents  
CASE REPORTS
Year : 2021  |  Volume : 1  |  Issue : 2  |  Page : 95-97

Dermatofibrosarcoma protuberans of the lower extremity: A rare tumor


Department of Radiation Oncology and Nuclear Medicine, Dr Rajendra Prasad Government Medical College, Kangra at Tanda, Himachal Pradesh, India

Date of Submission19-Dec-2021
Date of Acceptance17-Feb-2022
Date of Web Publication24-Aug-2022

Correspondence Address:
Nitin Gupta
Department of Radiation Oncology and Nuclear Medicine, Dr Rajendra Prasad Government Medical College, Kangra at Tanda, Himachal Pradesh 176001
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/bjoc.bjoc_39_21

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  Abstract 

Dermatofibrosarcoma protuberans (DFSP) is a rare, uncommon, low-grade sarcoma of fibroblast origin. Clinical suspicion is confirmed by biopsy, and appropriate and confirmatory immunostaining with CD34, factor XIIIa, nestin, apolipoprotein D, and cathepsin K is to be performed in all cases of suspected DFSP. Initial treatment is surgical, and every effort should be made to completely remove the tumor at the time of initial therapy. If surgery yields positive margin, then re-resection is recommended whenever possible. For patients with DFSP in whom tumors cannot attain negative margins after surgery, adjuvant radiotherapy (RT) may be considered to improve local control. Herein, we describe a rare case of DFSP in the left leg of a 50-year-old woman treated with excision and adjuvant RT.

Keywords: Dermatofibrosarcoma protuberans, leg, rare, unusual


How to cite this article:
Raina D, Gupta N, Negi M. Dermatofibrosarcoma protuberans of the lower extremity: A rare tumor. Bengal J Cancer 2021;1:95-7

How to cite this URL:
Raina D, Gupta N, Negi M. Dermatofibrosarcoma protuberans of the lower extremity: A rare tumor. Bengal J Cancer [serial online] 2021 [cited 2022 Sep 27];1:95-7. Available from: http://www.bengaljcancer.org/text.asp?2021/1/2/95/354411




  Introduction Top


Dermatofibrosarcoma protuberans (DFSP) is a rare, uncommon, low-grade sarcoma of fibroblast origin. It rarely metastasizes.[1],[2] Clinical suspicion is confirmed by biopsy. In most cases, examination of hematoxylin and eosin results in an unequivocal diagnosis, so appropriate and confirmatory immunostaining with CD34, factor XIIIa, nestin, apolipoprotein D, and cathepsin K is be performed in all cases of suspected DFSP. As metastasis is very rare, an extensive workup is not routinely indicated unless highly suspected in history and physical examination. Initial treatment is surgical, and every effort should be made to completely remove the tumor at the time of initial therapy.[3] If surgery yields positive margin, then re-resection is recommended whenever possible. Mohs micrographic surgery (a technique of repeated surgery involving immediate microscopic examination of the tumor margin after excision until excision is complete) and wide surgical excision with 2–4 cm margins are recommended for DFSP.[4],[5] For patients with DFSP in whom tumors cannot attain negative margins after surgery, adjuvant radiotherapy (RT) may be considered to improve local control and prevent undesired cosmetic outcomes.[6] Herein, we describe a rare case of DFSP in the left leg of a 50-year-old woman treated with excision and adjuvant RT.


  Case Report Top


A 50-year-old female presented with the complaint of swelling in the left lower limb since 1 year. Swelling progressively increased in size in past 2 months with associated pain in swelling from past 1 month. There was no history of trauma, fever, weight loss, or appetite loss. There was no significant family history.

Physical examination revealed a soft, fluctuant, slightly tender swelling approximately 6 × 4 cm in the left lateral aspect of leg. There was no lymphadenopathy on examination.

Musculoskeletal ultrasound showed a soft tissue mass of 7 × 4 cm in the dermal and subdermal plane inseparable from the underlying fascia.

Computed tomography (CT) scan demonstrated a heterogeneously enhancing mass with nonenhancing areas suggestive of necrosis, overlying the anterolateral aspect of the left leg in the subcutaneous plane, with loss of fat planes with the adjacent muscle [Figure 1].
Figure 1: Contrast enhanced computed tomography axial (a and b) and sagittal (c) views of the left lower limb showing the heterogeneous mass (white arrow) in the subcutaneous plane with areas of necrosis (black arrow) and abutting the adjacent muscle with indistinct fat planes at places

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Fine needle aspiration cytology suggested pigmented spindle cell neoplasm with borderline malignant potential. Wide local excision of the tumor was performed, and intraoperative findings suggested that the underlying muscle was normal. Skin grafting was done from the opposite thigh.

Histopathology [Figure 2] showed a tumor arranged in short fascicles and a storiform pattern. The tumor cells were spindle shaped with mildly pleomorphic oval to elongated nuclei, fine chromatin, conspicuous nucleoli, and moderate amount of cytoplasm. Mitosis was 2–3/10 high-power field (HPF). Periphery showed hemosiderin deposition. The tumor was involving epidermis, and the resection margin was positive.
Figure 2: Histopathology of the tumor showing spindle cells with mildly pleomorphic oval to elongated nuclei arranged in fascicles (Hematoxylin and eosin 40×)

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Immunohistochemical staining was positive for CD34 antigen and vimentin and negative for S-100 and desmin. Based on histology and immunohistochemistry, diagnosis of DFSP was made.

In view of positive resection margin and that the subsequent resection was not feasible, she was subsequently administered image guided radiotherapy of 50 Gy @ 2 Gy/# for tumor bed with wide margins followed by 16 Gy @ 2 Gy/# for tumor bed boost. She is currently on regular follow-up since last 6 months and is disease free.


  Discussion Top


DFSP is a rare, uncommon, low-grade sarcoma of fibroblast origin. It is a slow-growing tumor that constitutes less than 0.1% of all malignant neoplasms and 1% of all soft tissue sarcomas.[7] On ultrasound, DFSPs have been found to be mostly hypoechoic or mixed hyperechoic, with mostly well-defined margins or irregular margins.[8] In our case, ultrasound showed a soft tissue mass in the dermal and subdermal plane inseparable from the underlying fascia; however, CT scan showed a heterogeneously enhancing mass lesion with nonenhancing areas suggestive of necrosis with loss of fat planes with the muscle. Microscopically, DFSP is characterized by diffuse infiltration of the dermis and subcutis, usually sparing the epidermis. Atypia is minimal, and mitoses are rare.[9] However, in our case, there was infiltration of epidermis, mitosis was 1–2/10 HPF, and the final diagnosis was made by immunohistochemical analysis. Treatment is surgical, and if surgery yields positive margin, then re-resection is recommended whenever possible. However, in our case, after surgery, resection margins were positive but re-resection was not feasible as grafting was already done, so patient was taken for adjuvant treatment with a total dose of 66 Gy. Postoperative RT is reported to have a cure rate of 85%.[10] The recurrence rate is high. Most local recurrences appear within the first 3 postoperative years, with 50% presenting within the first year of surgery. However, recurrences after 5 years are also reported.[11]


  Conclusion Top


DFSP is a rare and uncommon sarcoma, and is characterized by diffuse infiltration of the dermis, usually sparing the epidermis. In our case, however, there was infiltration of epidermis and tumor was CD34 and vimentin positivity. Wide local excision of the tumor was performed, and in view of positive resection margin, the patient was subsequently taken for adjuvant RT. As the recurrence rate in these tumors is high, close follow-up of these patients is necessary.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Acosta AE, Vélez CS Dermatofibrosarcoma protuberans. Curr Treat Options Oncol 2017;18:56.  Back to cited text no. 1
    
2.
Li Y, Wang C, Xiang B, Chen S, Li L, Ji Y Clinical features, pathological findings and treatment of recurrent dermatofibrosarcoma protuberans. J Cancer 2017;8:1319-23.  Back to cited text no. 2
    
3.
Rutgers EJ, Kroon BB, Albus-Lutter CE, Gortzak E Dermatofibrosarcoma protuberans: Treatment and prognosis. Eur J Surg Oncol 1992;18:241-8.  Back to cited text no. 3
    
4.
Molina AS, Duprat Neto JP, Bertolli E, da Cunha IW, Fregnani JHTG, Figueiredo PHM, et al. Relapse in dermatofibrosarcoma protuberans: A histological and molecular analysis. J Surg Oncol 2018;117:845-50.  Back to cited text no. 4
    
5.
Chang CK, Jacobs IA, Salti GI Outcomes of surgery for dermatofibrosarcoma protuberans. Eur J Surg Oncol 2004;30: 341-5.  Back to cited text no. 5
    
6.
Rutkowski P, Debiec-Rychter M Current treatment options for dermatofibrosarcoma protuberans. Expert Rev Anticancer Ther 2015;15:901-9.  Back to cited text no. 6
    
7.
Gloster HM Jr. Dermatofibrosarcoma protuberans. J Am Acad Dermatol 1996;35:355-74.  Back to cited text no. 7
    
8.
Shih PY, Chen CH, Kuo TT, Yang CY, Huang YH, Yang CH Deep dermatofibrosarcoma protuberans: A pitfall in the ultrasonographic diagnosis of lipoma-like subcutaneous lesions. Dermatol Sin 2010;28:32-5.  Back to cited text no. 8
    
9.
Fletcher CDM, Bridge JA, Hogendoorn P, Mertens F, editors. WHO Classification of Tumors of Soft tissue and Bone. Lyon: IARC; 2013. p. 77-9.  Back to cited text no. 9
    
10.
Mendenhall WM, Zlotecki RA, Scarborough MT Dermatofibrosarcoma protuberans. Cancer 2004;101:2503-8.  Back to cited text no. 10
    
11.
Ratner D, Thomas CO, Johnson TM, Sondak VK, Hamilton TA, Nelson BR, et al. Mohs micrographic surgery for the treatment of dermatofibrosarcoma protuberans. Results of a multiinstitutional series with an analysis of the extent of microscopic spread. J Am Acad Dermatol 1997;37:600-13.  Back to cited text no. 11
    


    Figures

  [Figure 1], [Figure 2]



 

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