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 Table of Contents  
Year : 2018  |  Volume : 7  |  Issue : 2  |  Page : 84-87

Dermatofibrosarcoma protuberans of the maxillofacial region: Report of a rare case and literature review

1 Department of Dental and Maxillofacial Surgery, Faculty of Basic Clinical Sciences, Usmanu Danfodiyo University Teaching Hospital, Sokoto, Nigeria
2 Department of Oral and Maxillofacial Surgery, Faculty of Dentistry, Lagos University Teaching Hospital, Lagos, Nigeria
3 Department of Histopathology, Faculty of Basic Clinical Sciences, Usmanu Danfodiyo University Teaching Hospital, Sokoto, Nigeria

Date of Web Publication1-Nov-2018

Correspondence Address:
Dr. Adebayo Aremu Ibikunle
Department of Oral and Maxillofacial Surgery, Usmanu Danfodiyo University Teaching Hospital, Sokoto
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/jdas.jdas_13_18

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Dermatofibrosarcoma protuberans is a low-grade malignancy that rarely occurs. When it occurs, it typically involves the trunk or extremities, but seldom occurs in the maxillofacial region. Its management in the maxillofacial region characteristically presents an esthetic challenge to the surgeon, especially because its surgical management often requires wide excision. We report a case of dermatofibrosarcoma protuberans of the lip and adjacent structures in a 42-year-old male. Wide excision of the tumor was done, and immediate reconstruction was achieved with a combination of platysma flap and Karapandzic lip reconstruction technique. The acceptable esthetic outcome was achieved, and the patient is currently being followed up. Although dermatofibrosarcoma protuberans infrequently occurs in the maxillofacial region, it may occur nonetheless; therefore, clinicians should be aware of this possibility. Surgical treatment of this tumor in the maxillofacial region often presents daunting reconstructive challenges.

Keywords: Dermatofibrosarcoma protuberans, maxillofacial tumor, reconstructive challenges

How to cite this article:
Taiwo AO, Ibikunle AA, Braimah RO, Michael A, Sambawa MU. Dermatofibrosarcoma protuberans of the maxillofacial region: Report of a rare case and literature review. J Dent Allied Sci 2018;7:84-7

How to cite this URL:
Taiwo AO, Ibikunle AA, Braimah RO, Michael A, Sambawa MU. Dermatofibrosarcoma protuberans of the maxillofacial region: Report of a rare case and literature review. J Dent Allied Sci [serial online] 2018 [cited 2023 Mar 26];7:84-7. Available from: https://www.jdas.in/text.asp?2018/7/2/84/244753

  Introduction Top

Dermatofibrosarcoma protuberans (DFSPs) is a rare low-grade malignancy that is slow growing but has a propensity for local aggression and recurrence.[1],[2],[3] The tumor rarely occurs regardless of the site of occurrence.[4],[5] When it occurs, it is very rarely seen in the maxillofacial region.[6] Its most of the frequent sites of incidence are the trunk and extremities, where it classically presents as a painless nodular growth.[2] Very rare reports of DFSP in other sites such as the vulva, the parotid, and the scalp have been reported.[2],[7]

Wide surgical excision remains an accepted mode of treatment; although, radiotherapy is advocated by some authors. More recently, the use of imatinib has been promoted in the management of advanced cases with metastatic lesions.[4],[8] To the best of our knowledge, this is the first report of a case involving the maxillofacial region from sub-Saharan Africa.

  Case Report Top

A 42-year-old male presented at our clinic with a slow growing, painless, and facial mass of 8-month duration [Figure 1]. He had surgical excision of a similar but smaller mass in the same anatomical region, 18 months before the presentation. However, the histological report was not available.
Figure 1: A globular right facial mass with hypopigmented overlying skin

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Examination revealed facial asymmetry which was due to a bulbous, firm, and nontender mass extending from the midline of the lower lip to the right oral commissure mediolaterally and from the lateral third of the upper lip to the mentum superoinferiorly [Figure 1]. The overlying skin exhibited areas of patchy hypopigmentation and also showed differential warmth. An area of ulceration which measured 0.9 cm × 1.4 cm was observed. The ulceration was covered by a dark-brown scab [Figure 1]. Examination of the submandibular, submental, and cervical lymph nodes revealed no clinically positive lymph node.

Baseline investigations such as electrolyte urea and creatinine and full blood count gave satisfactory results that were within the normal range. Posteroanterior view and true lateral views of the jaws did not reveal any bone involvement. Based on the history, which was suggestive of recurrence, an excisional biopsy with wide margins was done under general anesthesia [Figure 2]. The resulting defect was surgically reconstructed by utilizing a combination of the Karapandzic technique of lip reconstruction and the platysma flap [Figure 3] and [Figure 4].
Figure 2: The operative site following wide surgical excision of the tumor

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Figure 3: Raised platysma flap

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Figure 4: The platysma and karapandzic flaps inset

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The excised tissue specimen was sent to the histopathologist in our center, who received an ulcerated nodular tissue with a gray solid cut surface. Sections were partially embedded after routine processing with paraffin embedding and hematoxylin and eosin stains which on light microscopy showed a deep-dermal nodular cellular mesenchymal neoplasm with spindle cells admixed with histiocytic cells having bland nuclei and disposed in fascicles with storiform areas, consistent with a DFSPs [Figure 5]. Subsequently, the patient was referred to the oncologist for radiotherapy. Postoperative reviews have been satisfactory with a patient having acceptable aesthetics [Figure 6]. The patient has been followed up for 13 months without any sign of recurrence, and he is still being followed up.
Figure 5: Photomicrograph showing spindle-shaped cells mixed with histiocytic cells having bland nuclei and dispersed in fascicles with storiform pattern [H and E, (a) ×40 magnification,(b) ×100 magnification, (c) ×200 magnification and (d) ×400 magnification]

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Figure 6: Eight-month review of the patient showing acceptable postoperative aesthetics

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  Discussion Top

DFSP is a rare low-grade malignancy of the cutaneous and subcutaneous tissues that typically starts as a painless plaque; it may also progress to infiltrate deeper tissues.[2],[4]

Stivala et al. in a 12-year study of 59 cases of DFSP, reported an overall female preponderance.[2] However, this report features a male patient. Of the 59 cases studied, only two involved the oral and maxillofacial region. This further validates the rarity of this tumor in the oral/maxillofacial region. It is frequently seen on the trunks (42%–72%) and the proximal extremities (20%–30%), when it occurs.[2],[3] Proposed predisposing factors to the development of DFSP include trauma and previous irradiation.[5] There was no history of previous irradiation in this patient; although, he had previous surgical excision of an unknown tumor in an adjacent site before the presentation. Perhaps, repeated low-grade trauma which the patient may have overlooked could have played a role in its occurrence.

Several histological subtypes of DFSP have been described, these include, the classic, giant-cell fibroblastoma, Bednar tumor, sclerotic, myxoid, atrophic, and fibrosarcomatous subtypes.[2] The most frequently diagnosed subtype is the classic subtype, accounting for over 90% of the cases seen.[2] The classic subtype was observed in this case. This probably lends credence to the regularity of occurrence of the classic histological subtype. Histologically, DFSP characteristically consists of highly cellular tissue with uniform spindle-shaped cells predominating.[5],[6] The spindle-shaped cells have large elongated nuclei and are arranged in a storiform pattern.[2],[3],[5],[7]

Immunohistochemical markers are also used in characterizing DFSP; prominent among them is the CD34 antigen. Although, a nonspecific marker, it is expressed in as much as 90% of cases.[2] Other immunohistochemical markers that have been utilized are stromelysin III, apolipoprotein D, D2–D40, CD163, and factor XIIIa, all of which are negative in DFSP.[2],[5],[8]

DFSP has an inclination for local aggressiveness and recurrence, especially when inadequately excised.[3],[7],[9] Its propensity for recurrence is believed to be associated with the manner in which the cells are arranged in a radial fashion around an acellular core.[2] These peripherally positioned cells may be the loci for recurrence.

The traditional mainstay of treatment is wide surgical excision of the mass but adjuncts to this are fast emerging.[8],[9] To curb recurrence, the frozen section may be utilized in an adjunctive manner to ensure the complete removal of the tumor.[10] Frozen section was not done in this case due to the limited resources in our climes. Moreover, some researchers have touted the effectiveness and precision of Mohs micrographic surgery in reducing the rate of recurrence.[6]

Metastasis rarely occurs with DFSP and local wide excision often suffices in controlling the disease; however, when metastasis occurs, it worsens the prognosis significantly.[5],[9] Molecular therapy with imatinib mesylate has also been reported to improve prognosis, especially in patients with recurrent or metastatic tumors.[2],[8],[9] Imatinib acts by preventing the autophosphorylation of the tyrosine kinase receptor, thereby resulting in part reinstatement of normal intracellular signaling.[2],[8]

Surgical management of soft-tissue orofacial tumors often poses an esthetic challenge.[6],[7] This is especially so in patients with DFSP, where wide tissue excision is advocated. Surgical excision, in this case, resulted in a gaping tissue loss, which was challenging to close. Reconstruction of the postexcision defect was done using a combination of the Karapandzic technique for lip reconstruction and the platysma flap. This resulted in acceptable esthetics postoperatively.

  Conclusion Top

Although dermatofibrosarcoma of the maxillofacial region is rare, the possibility of its occurrence should never be underestimated. Surgical excision aimed at eliminating all tumor cells remains the principal mode of treatment. Nevertheless, other treatment modalities of treatment are emerging.

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


Conflicts of interest

There are no conflicts of interest.

  References Top

Chang YH, Chen YW. Surgical treatment of dermatofibrosarcoma protuberans on right face. J Dent Sci2017;12:311-2.  Back to cited text no. 1
Stivala A, Lombardo GA, Pompili G, Tarico MS, Fraggetta F, Perrotta RE, et al. Dermatofibrosarcoma protuberans: Our experience of 59 cases. Oncol Lett 2012;4:1047-55.  Back to cited text no. 2
Lee OJ, Pi DY, Jo DH, Cho KJ, Kim SY, Ro JY. Dermatofibrosarcoma protuberans of the parotid gland. Korean J Pathol2004;38:276-9.  Back to cited text no. 3
Van Der Rijt R, Moradi P. Dermatofibrosarcoma protuberans: A rare cutaneous tumour. J Surg Case Rep 2012;2012:8.  Back to cited text no. 4
Baker PA, O'Dowd GJ, Khan IU. Dermatofibrosarcoma protuberans arising in a decorative tattoo. Sarcoma 2005;9:37-41.  Back to cited text no. 5
Kumar L, Bhandari V, Singh S, Garg P, Kumar A. Giant dermatofibrosarcoma protuberans: A rare presentation over face. J Cancer Res Ther 2015;11:1038.  Back to cited text no. 6
Gupta AK, Singh HA, Manalel AM, Jaiswal NK. A rare case of scalp dermato-fibrosarcoma-protuberans with intracranial extension and distant soft tissue and liver metastasis. Calicut Med J2011;9:8-11.  Back to cited text no. 7
McArthur GA, Demetri GD, van Oosterom A, Heinrich MC, Debiec-Rychter M, Corless CL, et al. Molecular and clinical analysis of locally advanced dermatofibrosarcoma protuberans treated with imatinib: Imatinib Target Exploration Consortium Study B2225. J Clin Oncol 2005;23:866-73.  Back to cited text no. 8
Kim GK. Status report on the management of dermatofibrosarcoma protuberans: Is there a viable role for the use of imatinib mesylate? In which cases may it be therapeutically helpful and in which cases not? J Clin Aesthet Dermatol 2011;4:17-26.  Back to cited text no. 9
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. 10


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


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