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 Table of Contents  
CASE REPORT
Year : 2018  |  Volume : 5  |  Issue : 3  |  Page : 228-231

Management of low-grade malignant dermatofibrosarcoma protuberans without compromising oncologic clearance despite financial constraints


1 Department of General Surgery, Christian Medical College and Hospital, Ludhiana, Punjab, India
2 Department of Pathology, Christian Medical College and Hospital, Ludhiana, Punjab, India
3 Consultant Plastic Surgeon, Queen Elizabeth University Hospital, Birmingham, UK

Date of Web Publication17-Jul-2018

Correspondence Address:
Vibhushit Kaul
Christian Medical College and Hospital, Ludhiana, Punjab
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/cjhr.cjhr_15_18

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  Abstract 


Dermatofibrosarcoma protuberans (DFSP) is a rare, locally aggressive soft-tissue tumor with high recurrence. Tissue biopsy, imaging, and staging form the diagnostic pathway, but sometimes investigations deter patients from low socioeconomic background. We present a case of malignant DFSP with adequate oncologic clearance despite financial constraints. A young male presented with a swelling over the right hip for 8 years. Although fine-needle aspiration, core biopsy, magnetic resonance imaging, and staging computerized axial tomography scan are the ideal management pathway, the patient refused these for an excision biopsy which would have sufficed if histology was benign. Histology reported a malignant fibrosarcomatous variant of DFSP. Wide excision of previous biopsy site and flap reconstruction was done as advised by the skin oncology multidisciplinary team (MDT) which confirmed oncologic clearance. We would ideally advocate a proper oncologic pathway, but in patients where finances restrict investigations oncologic clearance can still be achieved.

Keywords: Fibrosarcomatous variant of dermatofibrosarcoma protuberans, financial constraints, informed consent, oncologic clearance, skin oncology multidisciplinary team


How to cite this article:
Kaul V, Haque PD, Calton N, Thomas SS. Management of low-grade malignant dermatofibrosarcoma protuberans without compromising oncologic clearance despite financial constraints. CHRISMED J Health Res 2018;5:228-31

How to cite this URL:
Kaul V, Haque PD, Calton N, Thomas SS. Management of low-grade malignant dermatofibrosarcoma protuberans without compromising oncologic clearance despite financial constraints. CHRISMED J Health Res [serial online] 2018 [cited 2019 Oct 19];5:228-31. Available from: http://www.cjhr.org/text.asp?2018/5/3/228/236888




  Introduction Top


Dermatofibrosarcoma protuberans (DFSP) is a rare soft-tissue tumor that originates in the reticular dermis. It is locally aggressive and is associated with a high recurrence rate after surgical excision. A review from the Surveillance, Epidemiology, and End Results Program of the National Cancer Institute reported a 4.2 per million DFSP incidence (0.1% of all cancers).[1] Third and fifth decades are commonly affected with an equal sex ratio and slight male preponderance.[2] Afro-Caribbeans are affected more and there are no other known risk factors. It is often misdiagnosed clinically,[1] and a high index of clinical suspicion with definitive biopsy confirms diagnosis. Treatment should be within a skin oncology/sarcoma multidisciplinary team (MDT). Our patient presented with a malignant DFSP, treated with oncologic clearance but without imaging and core biopsy due to financial constraints.


  Case Report Top


A 29-year-old male patient with no comorbidities, presented with an 8-year history of swelling over the right greater trochanter. This was insidious with no local or systemic features of malignancy. A fine-needle aspiration cytology (FNAC) done at another hospital a year earlier reported “Mesenchymal cells with no definite evidence of malignancy.” This clouded the patient's view that it was “benign” and he wanted it removed to relieve local discomfort when he sat cross-legged on the floor for his job as a 'cobbler'.

Local examination showed a 5 cm diameter swelling over the right hip, which was firm, non-tender, and mobile over the fascia and muscle, but adherent to the overlying skin, with no lymphadenopathy [Figure 1].
Figure 1: Five centimeter diameter swelling over the right lateral thigh

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Clinically, this was suspicious of a malignant tumor, but the patient refused a core biopsy and imaging as he could not afford these. With the patient's consent, an excision biopsy was done with full understanding that if histopathology was malignant, he would need another operation.

Under local anesthesia, an excision biopsy was done, including the overlying skin, deep until the fascia lata with a minimum (8–10 mm) margin. The specimen was marked superiorly with a silk suture and inked on the deep margin. The defect was closed directly without undermining and healed well [Figure 2]a and [Figure 2]b.
Figure 2: (a) Specimen resected after the first surgery with the skin and superior marker stitch. (b) Cut section showing excision deep to fascia lata

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The histopathology showed “Fibrosarcomatous (FS) variant of DFSP with the French Federation of Cancer Centres Sarcoma Group (FNCLCC); Grade III,” incomplete on the inked deep fascia lata margin.[3] CD34 was diffusely positive, and smooth muscle actin was focally positive with a 15%–20% Ki67 proliferative index on immunohistochemistry.

The Skin Oncology MDT meeting advised a wider 3 cm excision deep to periosteum. The defect was reconstructed with a local posterior lumbar transposition flap [Figure 3]. The specimen included the entire previous suture line, deep to the greater trochanter periosteum [Figure 4]a and [Figure 4]b. The biopsy confirmed complete excision including the residual fascia lata tumor. He was discharged on the 3rd post-operative day with weekly review and resumed work in 8 weeks [Figure 5].
Figure 3: Second surgery after wide local excision deep to periosteum over greater trochanter and the planned lumbar transposition flap for cover

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Figure 4: (a) Wide local excision specimen after the second surgery with previous suture line. (b) Inked deep aspect of wide local excision specimen until periosteum

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Figure 5: Three months post wide local excision and flap reconstruction

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  Discussion Top


DFSP most commonly arises in the trunk (42%), upper extremities (23%), lower extremities (18%), and head and neck (13%).[1],[2] The differential diagnoses include Dermatofibroma (DF), neurofibroma, leiomyoma, malignant melanoma, morpheic basal cell carcinoma, keloid, dermoid tumors, Kaposi sarcoma, fibrosarcoma, nodular fasciitis, and sarcoidosis.[4] DFSP with FS change represents 7%–16% of diagnosed DFSPs.[5]

Due to financial constraints and a previous benign FNA, the patient refused a core biopsy, magnetic resonance imaging (MRI), and staging computerized tomography (CT). An excision biopsy was agreed with informed consent, as this would remove the lump, alleviate discomfort allowing him to sit comfortably, and continue his profession and would have sufficed if the histology was benign.

FNAC or core biopsy can sometimes be inadequate or non-representative of an entire tumor resulting in false-negatives.[6] The intraoperative frozen section can be unreliable with a false-negative rate of 57%.[7] Any previous biopsy scar needs to be excised entirely within the final wide local excision (WLE) to ensure adequate oncologic clearance, as was done in this case.

The average monthly gross national income per capita, Purchasing Power Parity (PPP) is Indian rupees (INR) 34,845, (USD 540).[8] As a cobbler, our patient earned INR 5000 (USD 75) monthly and supported his non-earning wife and two school-aged children equating to INR 1250 (USD 19) per capita monthly income. This when corrected for his 4 member family was below the poverty line. He did not have medical insurance and lost 8 weeks earning during the treatment.

The ideal treatment of a core biopsy, immunohistochemistry, MRI, and staging CT scan would cost an additional INR 38,000 (USD 594), while the two surgeries cost him INR 53,760 (INR 10,500 + INR 43,260) [USD 840] saving 41.4% of the total expenses, amounting to 7½ months earnings. (Numerical Figures quoted approximately reflecting PPP & exchange rate at time of operation in 2017).

The treatment of choice in DFSP is WLE, as done in our patient. The option of Mohs micrographic surgery (MMS) is only available at specialized centers.[6] Anatomical site, depth, stage, and margin status (R0 resection) are important factors predicting management and local recurrence.[6],[7] Management planning is within a Skin Oncology/Sarcoma MDT to achieve oncologic clearance and ensure maximum functional preservation. A 3-5 cms (peripheral margin), down to the periosteum of the greater trochanter (deep margin) was adequate oncologic clearance in our patient.

The FS variant of DFSP has higher local and distant metastases (36% recurrence with 13% metastasis) compared to classic DFSP (13.7% recurrence with 1.1% metastasis).[9],[10]

Radiotherapy and tyrosine kinase inhibitors are other treatment options.[11] Local recurrences are more within 5 years which is the recommended minimum follow-up for DFSP, at 3–6 monthly intervals, as was planned for our patient. MRI may be done if clinically indicated for local recurrence or as a comparative baseline for future recurrence or metastasis.[6]


  Conclusion Top


DFSP and DFSP-FS are uncommon soft-tissue tumors characterized by a relatively high risk of local recurrence.[6],[10] Although ideally an initial core biopsy with MRI and staging CT would be the proper pathway, these may not always be possible due to financial constraints. Histopathological diagnosis is mandatory. An excision biopsy may be considered as an exception. We do not advocate by-passing the proper oncologic pathway; however, in selected patients, who decline treatment altogether for financial reasons, adequate oncologic clearance can be achieved with the patient agreement and informed consent.

Declaration of patient consent

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

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Criscione VD, Weinstock MA. Descriptive epidemiology of dermatofibrosarcoma protuberans in the United States, 1973 to 2002. J Am Acad Dermatol 2007;56:968-73.  Back to cited text no. 1
    
2.
Monnier D, Vidal C, Martin L, Danzon A, Pelletier F, Puzenat E, et al. Dermatofibrosarcoma protuberans: A population-based cancer registry descriptive study of 66 consecutive cases diagnosed between 1982 and 2002. J Eur Acad Dermatol Venereol 2006;20:1237-42.  Back to cited text no. 2
    
3.
Trojani M, Contesso G, Coindre JM, Rouesse J, Bui NB, de Mascarel A, et al. Soft-tissue sarcomas of adults; study of pathological prognostic variables and definition of a histopathological grading system. Int J Cancer. 1984;33:37-42.  Back to cited text no. 3
    
4.
Bogucki B, Neuhaus I, Hurst EA. Dermatofibrosarcoma protuberans: A review of the literature. Dermatol Surg 2012;38:537-51.  Back to cited text no. 4
    
5.
Kuzel P, Mahmood MN, Metelitsa AI, Salopek TG. A clinicopathologic review of a case series of dermatofibrosarcoma protuberans with fibrosarcomatous differentiation. J Cutan Med Surg 2015;19:28-34.  Back to cited text no. 5
    
6.
Reha J, Katz SC. Dermatofibrosarcoma protuberans. Surg Clin North Am 2016;96:1031-46.  Back to cited text no. 6
    
7.
Stojadinovic A, Karpoff HM, Antonescu CR, Shah JP, Singh B, Spiro RH, et al. Dermatofibrosarcoma protuberans of the head and neck. Ann Surg Oncol 2000;7:696-704.  Back to cited text no. 7
    
8.
Gross National Income (GNI) Per Capita, Purchasing Power Parity (Current International $). World Bank, International Comparison Program Database. 2016. Available from: http://www.data.worldbank.org/indicator/NY.GNP.PCAP.PP.CD. [Last accessed on 2017 Jul 20].  Back to cited text no. 8
    
9.
Liang CA, Jambusaria-Pahlajani A, Karia PS, Elenitsas R, Zhang PD, Schmults CD. A systematic review of outcome data for dermatofibrosarcoma protuberans with and without fibrosarcomatous change. J Am Acad Dermatol 2014;71:781-6.  Back to cited text no. 9
    
10.
Voth H, Landsberg J, Hinz T, Wenzel J, Bieber T, Reinhard G, et al. Management of dermatofibrosarcoma protuberans with fibrosarcomatous transformation: An evidence-based review of the literature. J Eur Acad Dermatol Venereol 2011;25:1385-91.  Back to cited text no. 10
    
11.
McArthur G. Dermatofibrosarcoma protuberans: Recent clinical progress. Ann Surg Oncol 2007;14:2876-86.  Back to cited text no. 11
    


    Figures

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



 

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