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Table of Contents
CLINICAL CASE
Year : 2018  |  Volume : 20  |  Issue : 1  |  Page : 42-44

Dermatofibrosarcoma Protuberans: An Unusual Parotid Swelling - A Case Report


1 Otolaryngology Department, Faculty of Medicine, Almajmaah University, Almajmaah, Saudi Arabia
2 Otolaryngology Department, King Faisal Specialist Hospital and Research Centre, Jeddah, Saudi Arabia
3 Otolaryngology Department, Faculty of Medicine King Abdulaziz University, Rabigh, Saudi Arabia

Date of Web Publication23-Dec-2019

Correspondence Address:
Ahmad K Alnemare
Faculty of Medicine, Almajmaah University, Almajmaah
Saudi Arabia
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/1319-8491.273918

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  Abstract 


Background: Dermatofibrosarcoma protuberans (DFSP) is a slowly growing tumor. It has a high recurrence rate. The tumor is characterized by dermal spindle cell proliferation with infiltration of subcutaneous tissue, and expression of CD34 on immunnohistochemistry is pathgnomic.
Main observation: We observed a 46-years-old female patient complaining of recurrent fixed swelling in the parotid region that was diagnosed as DSFP. The tumor had been surgically excised and the patient was referred for adjuvant radiotherapy and a follow-up every month for 2 years with no reported recurrence.
Conclusion: Although rare, DFSP requires a high index of suspicion if there is recurrent painless, cutaneous, and multinodular lesion in the parotid region. In this context, wide local excision (>3 cm margins) combined with post-operative radiotherapy plays a fundamental role in the management of DFSP patients.

Keywords: Dermatofibrosarcoma protuberans, CD34, recurrence, parotid


How to cite this article:
Alnemare AK, Alabidi A, Alhasan N, Abumansour KA. Dermatofibrosarcoma Protuberans: An Unusual Parotid Swelling - A Case Report. Saudi J Otorhinolaryngol Head Neck Surg 2018;20:42-4

How to cite this URL:
Alnemare AK, Alabidi A, Alhasan N, Abumansour KA. Dermatofibrosarcoma Protuberans: An Unusual Parotid Swelling - A Case Report. Saudi J Otorhinolaryngol Head Neck Surg [serial online] 2018 [cited 2021 May 5];20:42-4. Available from: https://www.sjohns.org/text.asp?2018/20/1/42/273918




  Introduction Top


Dermatofibrosarcoma protuberans (DFSP) is a slowly growing tumor, which usually presents as a nodular distinct dermal/ subcutaneous neoplasm. It is known for its high recurrence rate [1]. DFSP is highly aggressive locally, rarely metastasizes to regional lymph nodes or distant sites [2]. The commonest sites of DFSP are the trunk and the extremities; however, it may occur at any site of the body. Histologically, it is characterized by the typical storiform pattern [1]. Although DFSP represents less than 0.1% of all malignant neoplasms, it constitutes the most frequent skin sarcoma (approaching 1% of all soft tissue sarcomas), exceeds 1% of all head and neck malignancies and 7% of all head and neck sarcomas [3],[4]. Positive staining for CD34 on immunnohistochemistry is pathgnomic for DFSP [5]. Wide and deep local excision is the treatment of choice [6]. We report an unusual presenting case of DFSP in the parotid gland. We also describe the MRI findings of this tumor and correlate it with the histopathological findings.


  Case Study Top


Clinical History

A 46-years-old Saudi female patient presented at Combined Head and Neck Clinic, King Faisal Specialist Hospital and Research Centre, Jeddah ; Saudi Arabia complaining of recurrent swelling in front of the left ear since two years. She had undergone left superficial parotidectomy in periphery hospital seven months prior to presentation at our hospital. But the swelling showed up again and started to increase in size gradually.

Examination

Examination revealed swelling in left parotid region fixed to deep tissues, and having a firm to hard consistency which was associated with the surgical scar over the parotid area. The lesion (sized 4 × 6 × 3cm) was non-mobile and non-tender with propensity to bleed. Left facial nerve involvement was noted, grade 4 according to The House-Brackmann scale. Regional lymph nodes were not enlarged. Clinical examination did not reveal any other abnormality.

Histopathology

We reviewed slides and paraffin blocks from previous surgery in our hospital which showed: spindle cells neoplasm with frequent mitosis arranged in a storiform pattern, highly suggestive of dermatofibrosarcoma protuberance with possible minimal residual tumor cells at the margin.

Radiology

MRI was done and showed: a partial resection of the left parotid gland. The gland itself was hyperintense when compared to the right one. No evidence of regional and distant metastasis was seen [Figure 1] and [Figure 2],

Decision

The decision was made, in a multi-disciplinary meeting for revision surgery with post-operative radiotherapy. The tumor was surgically excised together with the scar of previous surgery. Some branches of facial nerve were involved in the tumor. These branches were sacrificed to allow complete resection. There were enlarged lymph nodes in upper jugular chain, which were dissected and sent for histopathology. Histopathology post operative course

Histopathological examination, of the excised tumor, showed a nodular lesion composed of large spindle-shaped cells with prominent nuclei, showing high mitotic figures and infiltrating into the subcutaneous fat and muscles [Figure 3].

Immunohistochemical markers were positive for CD34. (Fig. 4) They were negative for S100, desmin, CD117 and BcL-2. These findings were suggestive of residual DFSP in the parotid gland. Lymph node pathology results were all negative for tumor metastasis including its margins. The patient was referred for adjuvant radiotherapy. We followed-up her every month for 2 years till now and no recurrence has been reported.


  Discussion Top


Typical presentation of DFSP is in early or middle adult life with a slowly growing soft nodular cutaneous mass on the trunk, although any part of the body may be involved [7].

CD34 is a highly specific tumor marker for DFSP diagnosis [7]. Its sensitivity has been ranged between 84 and 100% [8].

It was reported in the literature that wide excision with 3cm margins lowers the recurrence rate of DFSP significantly [9]. However, particularly in head and neck region, MOHS’ micrographic surgery which focuses on minimal removal of normal tissues also showed low recurrence rate [10].

The occurrence of DFSP in the parotid region as in our case poses great difficulty and challenge in its management due to the critical structures and difficulties in reconstruction [11]. Although surgery is the recommended standard in its treatment, adjuvant radiotherapy has been suggested in recurring lesions, when the margins are positive or close to the tumor or in cases where wider excision is not possible due to anatomic limitations, especially in head and neck regions [12].

In the present case report, after two years of follow-up, no recurrence was reported. In a series of 27 patients of DFSP by McPeak et al., recurrence rate was 11.11% [13]. Roses et al. reported a recurrence rate of 32% (41% when resection margin was less than 2 cm and 24% if resection margin exceeded 2 cm) [14]. Hamid et al., concluded in their study that because of the potential local recurrence, therapy for DFSP should be directed toward adequate local excision of the primary lesion. Minimal resection should include a surrounding margin, comprising 3-cm margin of normal skin and removal of underlying deep fascia [15]. Furthermore, Lindner et al. reported 2.5-3 cm resection margin improved local control of disease [16].

As a feedback of treatment of 260 cases in China, patients underwent wide excision with margins >3 cm were found to have lower recurrence rate compared with those margins 1.5-2 cm [17]. In the light of findings described in our case as well as from above findings, we also recommend 3 cm or more resection margins wherever feasible to improve the local control of the disease with low recurrence rate.

The use of post-operative radiotherapy in the treatment of DFSP has been discussed in many studies [18]. It is particularly recommended if resection of the tumour is inadequate. There is limited objective data to encourage its routine use; however, successful application has been observed in few small case series [19].


  Conclusion Top


DFSP is a rare tumor, and clinicians must suspect its presentation in parotid region if there is recurrent painless, cutaneous, and multinodular lesion in this region. Wide local excision (>3 cm margins) combined with post-operative radiotherapy was a golden standard.



 
  References Top

1.
Enzinger FM, Weiss SW. Soft tissue tumors. 3rd ed. St Louis: Mosby-Year Book; 1995. p 201–225.  Back to cited text no. 1
    
2.
Haycox CL, Odland PB, Olbricht SM, Piepkorn M. Immunohistochemical characterization of dermatofibrosarcoma protruberans with practical applications for diagnosis and treatment. J Am Acad Dermatol. 1997;37:438–444.  Back to cited text no. 2
    
3.
Laskin WB. Dermatofibrosarcoma protuberans. CA Cancer J Clin. 1992;42(2):116–25.  Back to cited text no. 3
    
4.
Stojadinovic A, Karpoff HM, Antonescu CR, Shah JP, Singh B, Spiro RH, et al. Dermatofibroma protuberance of the head and neck. Ann Surg Oncol. 2000; 7(9):696–704.  Back to cited text no. 4
    
5.
Mentzel T, Beham A, Katenkamp D, Dei Tos AP, Fletcher CD. Fibrosarcomatous “high grade” dermatofibrosarcoma protuberans: clinicopathologic and immunohistochemical study of a series of 41 cases with emphasis on prognostic significance. Am J Surg Pathol. 1998;22:576–587.  Back to cited text no. 5
    
6.
Sardesai VR, Patil RM, Agarwal TD. Dermatofibrosarcoma Protuberans at an Uncommon Site. Indian J Dermatol. 2014 ; 59(6): 635.  Back to cited text no. 6
    
7.
Palmerini E, Gambarotti M, Staals EL, Zanella L, Sieberova G, Longhi A, et al. Fibrosarcomatous changes and expression of CD34+ and apolipoprotein-D in dermatofibrosarcoma protuberans. Clin Sarcoma Res. 2012 Jan 27;2(1):4. doi: 10.1186/2045-3329-2-4.  Back to cited text no. 7
    
8.
Haycox CL, Odland PB, Olbricht SM, Piepkorn M. Immunohistochemical characterization of dermatofi brosarcoma protuberans with practical applications for diagnosis and treatment. J Am Acad Dermatol. 1997;37:438-44.  Back to cited text no. 8
    
9.
Burkhardt BR, Soule EH, Winkelmann RK. Dermatofibrosarcoma protuberans. Study of 56 cases. Am J Surg. 1966 May;111(5):638-44.  Back to cited text no. 9
    
10.
Gloster HM, Harris KR, Roenigk RK. A comparison between Moh’s micrographic surgery and wide surgical excision for the treatment of dermatofibrosarcoma protuberans. J Am Acad Dermatol. 1996;35:82.  Back to cited text no. 10
    
11.
Hegde U, Shetty SK, Sreeshyla HS, Doddawada VG. Dermatofibrosarcoma Protuberans – A recurrent lesion with unusual presentation in the parotid Region. J Clin Diagn Res. 2014;8(3): 130-131.  Back to cited text no. 11
    
12.
Suit H, Sprior I, Mankin HJ, et al. Radiation in management of patients with dermatofibrosarcoma protuberans. J Clin Oncol. 1996;145:2365.  Back to cited text no. 12
    
13.
McPeak CJ, Cruz T, Nicastri AD. Dermatofibrosarcoma Protuberans: An analysis of 86 cases-Five with metastasis. Ann Surg. 1967;166:803–16.  Back to cited text no. 13
    
14.
Roses DF, Valensi Q, LaTrenta G, Harris MN. Surgical treatment of Dermatofibrosarcoma Protuberans. Surg Gynecol Obstet. 1986;162:449–52.  Back to cited text no. 14
    
15.
Hamid R, Hafeez A, Darzi AM, Zaroo I, Owais H, Akhter A. Dermatofibrosarcoma protuberans: Role of wide local excision. South Asian J Cancer. 2013; 2(4): 232–238.  Back to cited text no. 15
    
16.
Lindner NJ, Scarborough MT, Powell GJ, Spanier S, Enneking WF. Revision surgery in dermatofibrosarcoma protuberans of the trunk and extremities. Eur J Surg Oncol. 1999;25:392–7.  Back to cited text no. 16
    
17.
Cai H, Wang Y, Wu J, Shi Y. Dermatofibrosarcoma protuberans: Clinical diagnoses and treatment results of 260 cases in China. J Surg Oncol. 2012;105:142–8.  Back to cited text no. 17
    
18.
Heuvel ST, Suurmeijer A, Pras E, Van Ginkel RJ, Hoekstra HJ. Dermatofibrosarcomaprotuberans: recurrence is related to the adequacy of surgical margins. Eur J Surg Oncol. 2010; 36: 89-94.  Back to cited text no. 18
    
19.
Dagan R, Morris CG, Zlotecki RA, Scarborough MT, Mendenhall WM. Radiotherapy in the treatment of dermatofibrosarcoma protuberans. Am J Clin Oncol. 2005; 28: 537-9.  Back to cited text no. 19
    




 

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