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 Table of Contents  
Year : 2017  |  Volume : 20  |  Issue : 2  |  Page : 204-207

Breast dermatofibrosarcoma protuberans in an adolescent male: A case report and extensive review of the literature

1 Department of Histopathology, Nnamdi Azikiwe University Teaching Hospital, Nnewi, Anambra State, Nigeria
2 Department of Surgery, Paediatric Surgery Unit, Nnamdi Azikiwe University Teaching Hospital, Nnewi, Anambra State, Nigeria

Date of Web Publication14-Nov-2017

Correspondence Address:
I F Ezejiofor
Department of Histopathology, Nnamdi Azikiwe University Teaching Hospital, Nnewi, Anambra State
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DOI: 10.4103/tjmr.tjmr_52_16

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Dermatofibrosarcoma protuberans (DFSP) is a nodular cutaneous tumor with high recurrence rate characterized by a prominent storiform pattern. DFSP of the breast is extremely rare and even rarer is DFSP of the male breast. The aim of this article is to present a case report of right breast DFSP (T1N0M0 Stage 1B) in a 13-year-old male. The diagnosis of this case report was based on clinical examination, histology of core, and excisional biopsies as well as immunohistochemistry.

Keywords: Adolescent male, breast dermatofibrosarcoma protuberans, literature review, trauma

How to cite this article:
Ezejiofor I F, Onwukamuche M E, Anyiam D, Ugwu J O, Ndukwe C O, Madubuike K C, Menkiti F E, Ogbu C C, Ozor N S. Breast dermatofibrosarcoma protuberans in an adolescent male: A case report and extensive review of the literature. Trop J Med Res 2017;20:204-7

How to cite this URL:
Ezejiofor I F, Onwukamuche M E, Anyiam D, Ugwu J O, Ndukwe C O, Madubuike K C, Menkiti F E, Ogbu C C, Ozor N S. Breast dermatofibrosarcoma protuberans in an adolescent male: A case report and extensive review of the literature. Trop J Med Res [serial online] 2017 [cited 2020 Aug 7];20:204-7. Available from: http://www.tjmrjournal.org/text.asp?2017/20/2/204/218219

  Introduction Top

Taylor first identified dermatofibrosarcoma protuberans (DFSP) in 1890 as a sarcoma resembling a keloid.[1] In 1924, Ferrand[2] recognizes it as a unique entity and described it as a progressive recurrent dermatofibroma and was later termed DFSP by Hoffman[3] in 1925. This rare tumor usually presents during early or middle adult life as erythematous indurated firm subcutaneous nodules. It is a slow growing, nodular, polyploidy neoplasm that invades the subcutaneous tissue and rarely metastasizes.[4] DFSP is predominant in the trunk, rare in the breast, and even extremely rare in male breast.

  Case Report Top

A 13-year-old Nigerian male of Hausa tribe presented to the surgical outpatient department with a 2-year history of painless right breast swelling following a fall. The lesion was noticed as a nodule in the upper outer quadrant, which gradually increased in size extending to the inner upper quadrant and central areas sparing the nipple [Figure 1]. There were alternating areas of hyper- and hypo-pigmentation of the overlying skin. His physical examination revealed a healthy adolescent with body mass index of 17.62 kg/m2. His vital signs showed pulse rate of 72 beat/min and respiratory rate of 24 cycles/min. Breast examination revealed a multinodular irregular mass of 12 cm × 10 cm, firm in consistency, and attached to skin but not to the underlying structures. The mass was not tender and there was neither differential warmth nor lymphadenopathy.
Figure 1: Clinical examination showing a firm, multinodular, irregular mass in the upper outer quadrant, extending to the inner upper quadrant, and central areas sparing the nipple

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Ultrasound examination of abdomen and chest X-ray revealed no abnormalities. Other investigations revealed pack cell volume of 38.08%, white blood cell of 4.1 × 103/L, and AS genotype. Electrolyte/urea/creatinine were within normal ranges. He underwent excisional biopsy of the right breast. Tumor was removed and submitted to the histopathology department for further analysis and diagnosis. The patient was discharged on satisfactory condition 13th day postsurgery but was lost in follow-up.

Gross examination [Figure 2] showed an excisional biopsy specimen, which comprises skin and subcutaneous tissue with firm, multiple nodular grayish-white masses of variable sizes below the skin, altogether measured 11.2 cm × 9.0 cm × 4 cm and weighed 120 g. The largest mass measured 2.5 cm × 2.5 cm × 1 cm, whereas the smallest measured 1.8 cm × 1 cm × 0.5 cm. Serial cut sections revealed tumors of circumscribed nodular, yellowish-white tissues with fibrous wall. Microscopy showed poorly circumscribed intermediate-grade, malignant, mesenchymal neoplasm composed of proliferating plump spindle cells with low mitotic activity disposed largely in storiform pattern. There were few foci of myxoid stroma [Figure 3]b and localized areas showing trapped adipocytes; however, the overlying skin appeared thinned out, whereas the resection margins showed tumor involvement. Overall features were those of incompletely resected DFSP.
Figure 2: Gross tumor displaying well-circumscribed multinodular masses

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Figure 3: (a) Photomicrograph shows a proliferation of a uniform population of fibroblasts arranged in a distinct storiform pattern around vascular channels. (b) Photomicrograph shows myxoid change

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  Discussion Top

DFSP is a highly recurrent, infiltrating intermediate-grade, malignant, soft-tissue sarcoma, which originates from the dermis of the skin and historically from fibroblast, histiocyte, or neuroectoderm.[4] DFSP has capacity to differentiate into all these cells suggesting undifferentiated mesenchymal cells to be their origin.[4] More than 90% of DFSP exhibit either ring chromosome or an unbalanced chromosomal translocation between chromosomes 17 (COL1A1) and 22 (platelet-derived growth factor B) with response to tyrosine-kinase inhibitor (imatinib).[5] The prevalence of DFSP is roughly 0.01% of all malignant tumors and approximately 2%–6% of all malignant soft-tissue sarcoma.[6] Primary sarcomas of the breast are extremely rare, <0.1% of all malignant tumors of the breast,[5] whereas the dermatofibrosarcoma of the breast is even rare. DFSP affects male more frequently than female although some authors report higher rate in female than male.[4],[7]

The estimated incidence of DFSP is 0.8–5 cases per 1 million persons/year, which are roughly 1000 new cases/year in America.[8] The incidence among negro (6.5/million) almost double that among Caucasian (3.9/million).[8] Valdivielso-Ramos et al. reported that the average annual incidence of DFSP in the pediatric population corresponded to 1.02 cases/million persons/year.[9]

DFSP commonly affects patients between 20 and 50 years of age but can sometimes be seen in both children and elderly. Congenital DFSP is a recognized entity but is extremely uncommon. DFSP occurs at almost any site, but the predominant sites are trunk (47%) followed by lower extremities (20%), proximal extremities (18%), and finally head and neck (14%).[4]

In Maiduguri, North-eastern Nigeria, Nggada et al. reported 36 cases of DFSP with a median age of 37 years, and 48.8% of the tumors present in the trunk, none in the breast, with male to female ratio of 2:1.[7] In University of Benin Teaching Hospital, Benin City, Nigeria, Osime et al. reported one case of DFSP of the left breast in an older male of 32-year-old.[10] In India, Jawaharlal Nehru Medical College, Kafil et al. also reported a case of DFSP of the left breast in a 22-year-old male,[11]whereas in Doha, Qatar, Al Tarakji et al. reported a case of breast DFSP in yet another adult male of 27-year-old.[12] After extensive research in literature, this index case in a 13-year-old will be the youngest reported age of male breast DFSP.

The etiology of this tumor is not well known, but antecedent trauma is an associated predisposing factor in the development of this malignancy.[13] This association with trauma is also documented in the index case. This is also supported by Bukhari et al. who reported a case of DFSP at an unusual site in a 9-year-old child at previous central venous line insertion in the left supraclavicular area.[14]

The hallmark of diagnosis is histology and immunohistochemistry. Histological section of DFSP shows uniform population of slender fibroblast arranged in a distinct monotonous storiform pattern [Figure 3]a around inconspicuous vasculature and interdigitate with lobules of subcutaneous fat creating honeycomb effect. Immunohistochemically, DFSP has diffuse and extensive positivity for CD34 but negative for S-100, CD44, factor XIIIa, and cytokeratins. In addition, staining for hyaluronate and vimentin is expected to be positive in DFSP.[4],[15]

In the index case, the tumor showed strong positivity to CD34 [Figure 4] but negative stains to S-100, pan cytokeratin, and CD117. Some mimics of DFSP include phyllodes, dermatofibroma, fibrosarcoma, neurofibroma, benign fibrous histiocytoma, cellular fibroadenoma, nodular fascitis, and inflammatory myofibroblastic tumor.[4] Benign fibrous histiocytoma, dermatofibroma, and phyllodes show focal/patchy stain in occasional cases with CD34, whereas fibrosarcoma display negative stain with CD34. The histology of the tumor and S-100 negativity ruled out neurofibroma.
Figure 4: Photomicrograph of dermatofibrosarcoma protuberans showing strong positivity with CD34 stain

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The mainstay of treatment of DFSP is Mohs microscopic controlled surgery which has no recurrence at 5 years or wide margin excision surgery (3–5 cm) which has a recurrence rate of 13% in combination with chemotherapy (imatinib) with or without radiotherapy.[6],[16] The index case had simple tumor excision and was meant to start chemotherapy but was lost in follow-up. DFSP has excellent prognosis except in metastatic tumor but with aid of imatinib these cases show better outcome.

  Conclusion Top

We have reported an extremely rare case of DFSP of the right breast in a 13-year-old male with antecedent trauma associated. This report has highlighted the rare anatomical location in an adolescent male, the risk factor, and extensive review of literature on the patient.

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.

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Conflicts of interest

There are no conflicts of interest.

  References Top

Taylor RW. Sarcomatous tumour resembling in some respect keloids. J Cutan Genitourin Disord 1890;8:384.  Back to cited text no. 1
Ferrand D. Dermatofibrosarcomas progressive and recurrent or fibrosarcoma of the few. Ann Dermatol Syphiligr 1924;5:545-62.  Back to cited text no. 2
Hoffman E. Over the knoll driving fibrosarcoma of the skin (Dermatofi brosarcoma protuberans). Dermatol Ztschr 1925;43:1-28.  Back to cited text no. 3
Schmitt W, Black S, MacSween L. Fibrohistiocytic tumors of intermediate malignancy. In: Weiss and Goldblum: Enzinger and Weiss's Soft Tissue Tumors. 5th ed. Philadelphia: Mosby Elsevier; 2008. p. 371-82.  Back to cited text no. 4
Pollard SG, Marks PV, Temple LN, Thompson HH. Breast sarcoma. A clinicopathologic review of 25 cases. Cancer 1990;66:941-4.  Back to cited text no. 5
Kransdorf MJ. Malignant soft-tissue tumors in a large referral population: Distribution of diagnoses by age, sex, and location. AJR Am J Roentgenol 1995;164:129-34.  Back to cited text no. 6
Nggada HA, Gali BM, Na'aya HU. A clinicopathological study of dermatofibrosarcoma protuberans in Maiduguri, Northeastern Nigeria. Niger J Surg Res 2006;8:78-80.  Back to cited text no. 7
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. 8
Valdivielso-Ramos M, Torrelo A, Campos M, Feito M, Gamo R, Rodriguez-Peralto JL. Pediatric dermatofibrosarcoma protuberans in Madrid, Spain: Multi-institutional outcomes. Pediatr Dermatol 2014;31:676-82.  Back to cited text no. 9
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.  Back to cited text no. 10
  [Full text]  
Kafil A, Rana KS, Prasenjit SR. Dermatofibrosarcoma Protuberans of Male Breast: An Unusual Presentation. India: Oman Medical Specialty Board; 2012. p. 1-3.  Back to cited text no. 11
Al Tarakji M, Toro A, Di Carlo I, Junejo K. Unusual presentation of dermatofibrosarcoma protuberans in a male patient's breast: A case report and review of the literature. World J Surg Oncol 2015;13:158.  Back to cited text no. 12
Ding J, Hashimoto H, Enjoji M. Dermatofibrosarcoma protuberans with fibrosarcomatous areas. A clinicopathologic study of nine cases and a comparison with allied tumors. Cancer 1989;64:721-9.  Back to cited text no. 13
Bukhari I, Al Akloby O, Bedaiwi Y. Dermatofibrosarcoma protuberans at the site of a central venous line. Case report. Am J Clin Dermatol 2005;6:61-4.  Back to cited text no. 14
Haycox CL, Odland PB, Olbricht SM, Piepkorn M. Immunohistohemical characteristic of dermatofibrosarcoma protuberans with practical applications for diagnosis and treatment. J Am Acad Dermatol 1997;37:438-44.  Back to cited text no. 15
Lemm D, Mügge LO, Mentzel T, Höffken K. Current treatment options in dermatofibrosarcoma protuberans. J Cancer Res Clin Oncol 2009;135:653-65.  Back to cited text no. 16


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


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