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Table of Contents
CASE REPORT
Year : 2021  |  Volume : 29  |  Issue : 1  |  Page : 62-65

Giant dermatofibrosarcoma protuberans in the forearm


Department of Plastic Reconstructive and Aesthetic Surgery, Ankara Training and Research Hospital, Altindag, Ankara, Turkey

Date of Submission19-Dec-2019
Date of Acceptance29-Feb-2020
Date of Web Publication31-Dec-2020

Correspondence Address:
Dr. Ferhat Kargalioglu
Department of Plastic Reconstructive and Aesthetic Surgery, Ankara Training and Research Hospital, Ulucanlar Street 89, Altındağ/ Ankara, 06230
Turkey
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/tjps.tjps_94_19

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  Abstract 


Dermatofibrosarcoma protuberans (DFSP) is a rare, slow-growing, locally aggressive malignant fibroblastic mesenchymal skin tumor, which is considered as the most common skin sarcoma. As a treatment modality, local excision with 4–5 cm border is commonly replaced by Mohs micrographic surgery. A 24-year-old woman with a giant DFSP in the forearm is presented. The patient was admitted to clinic with a large protuberant mass on the left forearm. The preliminary diagnosis was soft-tissue sarcoma due to a history of rapid growth and absence of any skin lesion. DFSP originates from the dermis and commonly infiltrates underlying muscles, tendons, fascia, and bones. In this case, there was no skin infiltration. Mohs micrographic surgery was not used in the treatment since the tumor was confined to subcutaneous tissue and did not infiltrate to the skin. Although the Mohs micrographic surgery was not used, the tumor did not recur postoperative 6 months' follow-up. A long-term follow-up is strongly recommended in DFSP cases.

Keywords: Dermatofibrosarcoma, giant dermatofibrosarcoma protuberans, local excision, protuberans, recurrence


How to cite this article:
Kargalioglu F, Ustun GG, Gursoy K, Kocer U. Giant dermatofibrosarcoma protuberans in the forearm. Turk J Plast Surg 2021;29:62-5

How to cite this URL:
Kargalioglu F, Ustun GG, Gursoy K, Kocer U. Giant dermatofibrosarcoma protuberans in the forearm. Turk J Plast Surg [serial online] 2021 [cited 2021 Dec 9];29:62-5. Available from: http://www.turkjplastsurg.org/text.asp?2021/29/1/62/305914




  Introduction Top


Dermatofibrosarcoma protuberans (DFSP) was originally described by Darier and Ferrand in 1924, but the term “DFSP” was established by Hoffman in 1925.[1] DFSP is a rare, slow growing fibrohistiocytic neoplasm considered as low to medium malignancy. Although it rarely metastasizes, 20%–50% of DFSP cases recur locally.[2] It usually occurs between the third and fourth decades of life. A slight male predominance has been reported among patients with DFSP.[3] It favors the trunk (40%–60%), followed by the proximal extremities (20%–30%) and the head and neck (10%–16%).[4] The tumor has a low chance of metastasis, either to regional lymph nodes or distantly, but is aggressive locally.

History, clinical and histopathological examination takes part during the diagnosis of these tumors. Lesions tend to grow slowly and may originally present as a painless, skin-colored plaque with dark red or blue discoloration.[3] DFSPs can increase in size and become protuberant or ulcerative. Although distant metastasis is rare, they are usually characterized with locally aggressive behavior. DFSPs tend to infiltrate adjacent subcutaneous tissue, muscles, tendons, and bone structures. These tumors infiltrated into deep tissues such as forearm or hand. This infiltration can cause loss of functions, amputations and disabilities, so early diagnosis is paramount. A patient with 8 cm × 6 cm mass in the left forearm is presented.


  Case Report Top


A 23-year-old female patient presented with a giant mass in the left anterior side of the forearm. History reveals no additional systemic diseases.

Mass in the anterior side of the forearm was measured to be 8 cm × 6 cm [Figure 1] and had a history of 6 months' growth before presentation. The motor and sensory examination was normal.
Figure 1: The mass was seen in the forearm

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In the ultrasound, a hypoechoic solid lesion with necrotic areas with a lobulated contour of approximately 70 mm × 35 mm is observed between the muscle structures in the anteromedial part of the left forearm. In color Doppler ultrasound, the mass showed a marked increase in blood supply. In the magnetic resonance imaging (MRI), a hypervascular mass measuring 88 mm × 50 mm, extending to the midline, taking nourishing arterial branches from the radial artery into the mass is noted [Figure 2]. Local and distant metastases were eliminated by clinical examination and imaging.
Figure 2: Magnetic resonance imaging of the dermatofibrosarcoma protuberans

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The operation was performed under tourniquet control using loupe magnification. The subcutaneous mass was reached by lazy S incision. Intraoperative exploration showed an approximately 10 cm × 8 cm irregular bounded vascular mass [Figure 3]. The mass was observed to be fed by multiple branches emerging from the radial and ulnar artery and associated with flexor carpi ulnaris and flexor digitalis superficialis muscle bulks [Figure 4]. The mass was isolated from the vascular structures. The ulnar and radial artery is preserved [Figure 5] and [Figure 6]. The mass was excised without sacrification of any neurovascular and muscle tissue. Following hemostasis, the skin was primary closed after 1 Jackson Pratt drain.
Figure 3: Intraoperative image of the mass

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Figure 4: Intraoperative image of the mass which was associated with flexor digitalis superficial is muscle and nourished by ulnar artery

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Figure 5: Ulnar nerve and artery after excision of the mass

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Figure 6: Radial artery after excision of the mass

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The macroscopic appearance of the tumor was 8 cm × 6 × cm × 2.5 cm irregular bounded creamy dirty yellow nodular tissue. In the cross-section, a homogeneous appearance with orange-colored soft viscous mass around necrosis was observed.

Immunohistochemically, positive staining with vimentin, CD34, CD68, a1 antitrypsin and negative staining with HMB45, Melan-A, myosin was observed. The Ki-67 proliferation index is about 4%.


  Discussion Top


DFSP is a rare, slow-growing, low-grade, malignant fibroblastic mesenchymal skin tumor. It is most commonly seen in the third or fourth decade and favors most commonly the trunk (40%–60%), followed by the proximal extremities (20%–30%) and the head and neck (10%–16%).[5] The tumor is initially limited to the skin and then evolves into multiple “protuberant” nodules that may infiltrate the subcutaneous tissue, fascia, and muscles. In our case, no infiltration was observed and lesion was protuberant from the skin. The tumor has a low chance of metastasis but is locally aggressive. A local recurrence rate of DFSP of up to 60% has been reported.[6] DFSP usually presents as an asymptomatic, skin-colored plaque with possible dark red or blue discoloration. As the disease progress, there may be irregular nodules that can increase in size and become protuberant or ulcerative. Laboratory tests are not used in the diagnosis of DFSP. In most cases, no imaging studies are needed unless metastatic disease is suspected. In this case, the patient was admitted without any color change or ulceration in the skin so there was suspicion from the malignant soft-tissue sarcomas. The axillary ultrasonoghraphy and MRI of the extremity showed no metastasis. There was no clinical finding of regional or distant metastasis.

The American Joint Committee on Cancer has published a staging system. Stage I – localized disease, Stage II – lymph node metastasis, and Stage III – distant metastasis.[7] In this case, the patient had a Stage I disease. It was localized without any finding of clinical or radiographic evidence of metastasis.

The treatment of choice for a DFSP is wide local excision. Wide radical excision and Mohs micrographic surgery are the most frequently used surgical techniques. Surgical excision with 2–4 cm wide margins was the recommended treatment. In a series of 204 patients with DFSP, showed a very low local recurrence rate (1%) using wide excision (median 2 cm margin). If the pathology report shows positive margins, re excision, is recommended.

Mohs micrographic surgery is considered by some as the treatment of choice for DFSP.[8] The technique consists of sequential horizontal sectioning and immediate frozen microscopic examination until a tumor-free margin is obtained. The local cure rates with Mohs surgery range from 93% to 100%.[6] In this case, Mohs micrographic surgery was not used in the treatment, since the tumor was confined to subcutaneous tissue and did not infiltrate to the skin. The other reasons were the suspicion of soft-tissue sarcoma due to the absence of any skin lesion.

The majority of local recurrences of DFSP occur within the first 3 years, so it is important to follow these patients, especially in the first 3 years after surgery.

In summary, we report a case of DFSP who presented clinically with a firm 8 cm × 5 cm subcutaneous mass without any skin lesion. The diagnosis was achieved on the basis of pathological and immunohistochemical features, and there was no clinical or radiographic evidence of metastasis. In this case, the tumor was different from the other DFSPs due to a lack of skin lesion and excision without Mohs micrographic surgery. The tumor was excised without any skin excision because there was no skin lesion. The patient was referred to medical oncology after surgery and clinical control was recommended at 3 months' interval.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Bhambri S, Desai A, Del Rosso JQ, Mobini N. Dermatofibrosarcoma protuberans: A case report and review of the literature. J Clin Aesthet Dermatol 2008;1:346.  Back to cited text no. 1
    
2.
RuizTovar J, Fernández Guarino M, Reguero Callejas ME, Aguilera Velardo A, Arano Bermejo J, Cabañas Navarro L. Dermatofibrosarcoma protuberans: Review of 20years experience. Clin Transl Oncol 2006;8:60610.  Back to cited text no. 2
    
3.
Asquo ME, Umoh MS, Ebughe G. Dermatofibrosarcoma protuberans: Case reports. Ann Afr Med 2007;6:80-3.  Back to cited text no. 3
    
4.
Bhambri S, Desai A, Del Rosso JQ, Mobini N. Dermatofibrosarcoma protuberans: a case report and review of the literature. J Clin Aesthet Dermatol 2008;1:34-6.  Back to cited text no. 4
    
5.
Gloster HM Jr. Dermatofibrosarcoma protuberans. J Am Acad Dermatol 1996;35:355-74.  Back to cited text no. 5
    
6.
Nouri K, Lodha R, Jimenez G, Robins P. Mohs micrographic surgery for dermatofibrosarcoma protuberans: University of Miami and NYU experience. Dermatol Surg 2002;28:1060-4.  Back to cited text no. 6
    
7.
Amin MB, Edge SB, Greene FL, Byrd DR, Brookland RK, Washington MK, et al., editors. Ajcc Cancer Staging Manual. 8th ed. Switzerland: Springer; 2017.  Back to cited text no. 7
    
8.
Dim-Jamora KC, Perone JB. Management of cutaneous tumors with mohs micrographic surgery. Semin Plast Surg 2008;22:247-56.  Back to cited text no. 8
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6]



 

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