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 Table of Contents  
CASE REPORT
Year : 2014  |  Volume : 13  |  Issue : 2  |  Page : 119-121

Dermatofibrosarcoma protuberans of the breast in a male: A case report and review of the literature


1 Department of Surgery, University of Benin Teaching Hospital, Benin City, Edo State, Nigeria
2 Department of Pathology, University of Benin Teaching Hospital, Benin City, Edo State, Nigeria

Date of Web Publication13-Nov-2014

Correspondence Address:
Prof. Clement Osime
Department of Surgery, University of Benin Teaching Hospital, PMB - 1111, Benin City, Edo State
Nigeria
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/2384-5589.144583

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  Abstract 

Dermatofibrosarcoma protuberans (DFSP) of the breast is quite rare. Even rarer is DFSP of the male breast. The purpose of this article is to report a case of DFSP of the left breast in a 32-year-old man. This is with a view to creating awareness about this rare clinical condition. This is expected to aid clinicians in making an early diagnosis and ensuring prompt management of the condition.

Keywords: A rare malignant condition, dermatofibrosarcoma protuberans, literature review, males, rare clinical condition


How to cite this article:
Osime C, Irowa O, Obaseki D, Osaigbovo E. Dermatofibrosarcoma protuberans of the breast in a male: A case report and review of the literature. Afr J Med Health Sci 2014;13:119-21

How to cite this URL:
Osime C, Irowa O, Obaseki D, Osaigbovo E. Dermatofibrosarcoma protuberans of the breast in a male: A case report and review of the literature. Afr J Med Health Sci [serial online] 2014 [cited 2019 Sep 16];13:119-21. Available from: http://www.ajmhs.org/text.asp?2014/13/2/119/144583


  Introduction Top


Dermatofibrosarcoma protuberans (DFSP) is an extremely rare and slow-growing neoplasm that involves the soft tissue and was first described by Darier and Ferrand in 1924. [1] At the time they described the pathology, it was called a progressive recurrent dermatofibroma. However, in 1925, Hoffmann characterized the pathology better and renamed it DFSP. [2] It has a variable degree of malignancy with a high propensity for local invasion and a high recurrence rate, particularly if a negative margin is not be achieved in the primary resection surgery. [1],[2],[3] Most studies indicate that metastasis is a rare finding. [4],[5] Patients are commonly affected between the fourth and sixth decades of life. [6] While most studies report a higher preponderance of DFSP in females, other studies claim a higher incidence in males. This may be due to regional variations in the epidemiology of this pathology. [6]

DFSP may affect virtually any part of the body, but especially the trunk, the extremities, the head and neck, the vulva, the toes, etc. [5],[6] Of these sites, the trunk is the most commonly affected. [6] Occurrence in the female breast is extremely rare. [6] However, even rarer is occurrence of DFSP in the male breast. [6] Thus, the purpose of this article is to present a case of DFSP involving the male breast, and also to review the literature on the subject.


  Case Report Top


Mr. O.D. is a 32-year-old accountant who presented with a 3-month history of swelling in the left breast. The painless swelling slowly increased in size over time. Examination revealed a freely mobile lump, measuring about 2 × 2 cm, located in the lower outer quadrant of the left breast. There was no axillary lymphadenopathy. Ultrasound scan of the left breast showed a dense solid mass with a regular margin and without cavitations or calcifications in the lower outer quadrant of the left breast, measuring about 1.5 × 2.1 cm. At the time of operation, the lump was found to be a firm yellowish lump, was well encapsulated, and freely mobile within the tissue of the breast. The histology report confirmed DFSP of the left breast with the features as shown in [Figure 1]a and b.
Figure 1: (a and b) Both figures show a proliferation of a uniform population of fibroblasts arranged in a distinct storiform pattern around vascular channels. Figure 1a shows the tumor cells infiltrating subcutaneous fat

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The plan was to carry out a wide local excision of the tissues at the site of the mass with a margin of 3 cm. The patient, however, opted for simple mastectomy for fear of recurrence. The patient was subsequently counseled for simple mastectomy of the left breast. Histological examination of the tissue excised during mastectomy did not reveal any residual tumor. He has twice visited the outpatient clinic since then and the postoperative period has been largely uneventful 9 months post operation.


  Discussion Top


DFSP, even though a rare disease, accounts for about 6% of all soft tissue sarcomas, with an incidence of about 0.8 cases per million per year. [3],[4],[5],[6] In Maiduguri, DFSP accounted for 3.05% of all malignant tumors. Most cases of DFSP occur between the second and fourth decades of life, with a male preponderance. Nggada et al. reported a male:female incidence of 2:1. [7] Some cases have been reported in children, and there have also been reports on congenital cases of DFSP. [1],[2],[6] A few studies have reported equal prevalence in males and females or a slight female preponderance. [6] After a variable period of time, features consistent with a malignant lesion may then become noticeable. It is usually not easy to make a preoperative diagnosis of DFSP. However, after surgery, the characteristic histological findings and the immunohistochemical staining are usually diagnostic. [6] Histopathologically, the hallmark of DFSP is spindle cells arranged in storiform pattern with minimal atypia, a few mitotic figures, and the absence of necrosis. Immunohistochemically, DFSP shows strong positivity for CD34 and vimentin, and negative staining for cytokeratin, S-100, epithelial membrane antigen, and variable staining for smooth muscle actin (SMA). The expression of CD34 is almost a consistent finding, and it is extremely useful in the differentiation of DFSP from benign fibrous histiocytoma, dermatofibroma, and other soft tissue tumors. The sensitivity of CD34 staining in DFSP ranges 84-100%. [8]

The present case showed spindle cells arranged in storiform pattern with minimal atypia, a few mitotic figures, and the absence of necrosis, and these histological features are consistent with DFSP. However, we lacked the relevant antibody panels at our center to do immunohistochemistry to further characterize the lesion. Some other cutaneous lesions may mimic DFSP. Examples of such lesion include dermatofibromas, keloid, or morphea in early lesions, or cutaneous metastasis, malignant lymphoma, and Kaposi sarcoma in advanced states. One of the ways of making an accurate diagnosis is with the aid of immunohistochemistry in the hands of a skilled pathologist. [8]

The breast is a very rare site for DFSP. [5],[6] These sarcomas are found more commonly in the trunk, the extremities, and the head and neck. Of these sites, the trunk is most commonly affected. Of the 36 cases presented by Nggada et al., there was no case involving the breast. [7] Even rarer is dermatofibrosarcoma involving the male breast. A few cases of female breast area involvement have been reported but involvement of male breast is less likely supported by the medical literature. [5],[6] A recent case report by Cavusoglu et al. affirms that breast involvement even in females is a very rare incidence. [3] A study by Karcnik et al. similarly found very little involvement of the breast. [4] There are very few cases of DFSP affecting the male breast found in the literature. [6],[7]

In most cases of DFSP, the lesion is often from the dermal layer. However, some variants have been observed in the subcutaneous layer. [1],[2],[3],[4] This is the finding in the case presented here. Some authors have classified these variants occurring within the breast tissue as atypical forms of DFSP. And these variants can readily be confused with fibroadenomas or other primary lesions arising from the breast. [5],[6] Some studies have noted that most cases of DFSP arising from the subcutaneous layer of the breast usually have a smooth and regular margin, well-circumscribed and without calcifications. [5],[6]

Treatment for DFSP is primarily by surgical excision of the swelling with about 3 cm margin. [9] Simple excision, however, is fraught with the risk of local recurrence. Varying degrees of recurrence rates following excision have been reported by different authors. Consequently, a technique of surgery that has been recommended by several studies in order to minimize chances of recurrence is the Mohs micrographic surgical technique. [9] This technique, however, demands a lot of expertise on the part of the surgeon and on the part of the pathologist. Kamar et al. have reported success with use of sorafenib after the failure of imatinib. [10] Our patient opted for a simple mastectomy, even though the option of wide local excision of the site where the lump was removed initially was suggested. The patient's main concern for not accepting conservative surgery was fear of recurrence, because he claimed that he had read extensively about the pathology. He was then counseled, and informed consent for simple mastectomy was obtained. He then underwent a simple mastectomy. This point emphasizes the place of the patient's autonomy (one of the core values in medical ethics) in Medicare. The specimen that was examined after the mastectomy did not show any residual tumor. The patient is presently being followed up at the outpatient department and there has not been any challenge. However, it is opined that 9 months is too short a period to determine whether there will be recurrence.

In conclusion, we have presented a case of DFSP of the breast in a 32-year-old man, which is a very rare finding. Another point highlighted in this case report is the unusual nature of the lesion presented. In most cases of DFSP, the lesion is of dermal origin. However, in the case presented, the lesion was found in the subcutaneous layer. The case presented also highlights the place of the patient's autonomy in Medicare.

 
  References Top

1.
Darier S, Ferrard M. Dermatofibrosarcomes progressife et ricidivantes en fibrosarcomes de lapeau. Ann Dermatol Venereol 1924;5:545-62.  Back to cited text no. 1
    
2.
Hoffmann E. Ueber das knollentribende fibrosarkom der Haunt (dermatofibrosarcoma protuberans). Dermatol Z 1925;43:1-28.  Back to cited text no. 2
    
3.
Cavuþoðlu T, Yavuzer R, Tuncer S. Dermatofibrosarcoma protuberans of the breast. Aesthetic Plast Sur 2003;27:104-6.   Back to cited text no. 3
    
4.
Karcnic TJ, Miller JA, Fromountz F, Abujudek H, Hertz MB. Dermatofibrosarcoma of the breast: A rare malignant tumor simulating benign disease. Breast J 1999;5:262-3.  Back to cited text no. 4
    
5.
Bague S, Folpe AL. Dermatofibrosarcoma protuberans presenting as a subcutaneous mass: A clinicopathological study of 15 cases with exclusive or near-exclusive subcutaneous involvement. Am J Dermatopathol 2008;30:327-32.  Back to cited text no. 5
    
6.
Ruiz-Tovar J, Fernández Guarino M, Reguero Callejas ME, Aguilera Velardo A, Arano Bermejo J, Cabañas Navarro L. Dermatofibrosarcoma protuberans: Review of 20-years experience. Clin Transl Oncol 2006;8:606-10.  Back to cited text no. 6
    
7.
Nggada HA, Gali BM, Na'aya HU. A clinocopathological study of dermatofibrosarcoma protuberans in Maiduguri, Northeastern Nigeria. Nig J Sur Res 2006;8:78-80.  Back to cited text no. 7
    
8.
Sigel JE, Bergfeld WF, Goldblum JR. A morphologic study of dermatofibrosarcoma protuberans: Expansion of a histologic profile. J Cutan Pathol 2000;27:159-63.  Back to cited text no. 8
    
9.
Roh MR, Bae B, Chong KY. Mohs' micrographic surgery for dermatofibrosarcoma protuberans. Clin Exp Dermatol 2010;35:849-52.  Back to cited text no. 9
    
10.
Kamar FG, Kairouz VF, Sabri AN. Dermatofibrosarcoma protuberans (DFSP) successfully treated with Sorafenib: Case report. Clin Sarcoma Res 2013;3:5.  Back to cited text no. 10
    


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