|Year : 2014 | Volume
| Issue : 1 | Page : 85-87
Dermatofibrosarcoma protuberans: Male breast
Vinod V Prabhu1, Aslam Shivani1, SH Kulkarni1, Vishrabdha R Pawar2
1 Department of Surgery, B.V.D.U. Medical College, Sangli, Maharashtra, India
2 Department of Pathology, B.V.D.U. Medical College, Sangli, Maharashtra, India
|Date of Web Publication||10-Dec-2013|
Vinod V Prabhu
Department of Surgery, B.V.D.U. Medical College, Sangli-Miraj Road, Wanlesswadi, Sangli - 416 414, Maharashtra
Source of Support: None, Conflict of Interest: None
Dermatofibrosarcoma protuberans (DFSP) is a rare skin tumor of the dermal layer. Incidence is only about one case per million per year. It is a cutaneous soft tissue sarcoma that rarely metastasizes but it has got a malignant potential in 2% of cases. The challenge with DFSP is that the malignant potential of this tumor depends on the components histologically and on immunohistochemistry. The second challenge is that these tumors cannot be diagnosed on fine needle aspiration and require a core or incisional biopsy. Only in nodular stage we have to diagnose DFSP. This case presented in a male patient simulating malignancy of breast and fine needle aspiration cytology was inconclusive. The treatment also poses a challenge as not all surgeons are well versed with Moh's surgery and hence here local wide resection was done. All margins were free. Although chemotherapy forms a part of treatment, it is reserved for large and recurrent tumors.
Keywords: CD 34, dermatofibrosarcoma protuberans, skin
|How to cite this article:|
Prabhu VV, Shivani A, Kulkarni S H, Pawar VR. Dermatofibrosarcoma protuberans: Male breast. Med J DY Patil Univ 2014;7:85-7
| Introduction|| |
Soft tissue sarcomas form a unique group of tumors which pose diagnostic and treatment challenges. Dermatofibrosarcoma protuberans (DFSP) is a rare tumor that is seen to occur in adults in age of 30s. It accounts for 2-6% of all soft tissue sarcomas. The incidence is one in one million per year.
There are several variants of DFSP depending upon the cell types. Melanin containing cells are called as Bednar's tumor and cells containing myxoid tissue are called myxoid DFSP. Juvenile DFSP occurring in children are called as giant fibroblastoma. Rarely, the tumors in the different types of DFSP can have regions that look similar to fibrosarcoma which is a more aggressive type of soft tissue sarcoma called as fibrosarcomatous dermatofibrosarcoma protuberans (FS-DFSP) which frequently metastasize. Only 5% of DFSP metastasize. Local recurrence is common. Immunohistochemistry  forms an important tool to categorize DFSP. CD 34, Factor xiiia, and apo-d form some diagnostic tools. Out of these, CD 34 forms a better indicator.
The British Sarcoma Group  provides a framework for the multidisciplinary care of patients with soft tissue sarcomas.
Surgical management forms the mainstay of treatment along with radiotherapy and chemotherapy as a pre- or post-operative adjunct.
| Case Report|| |
A 55-year-old male presented with a nodule on left breast near the axillary fold since 2 years. The nodule gradually increased in size to attain present size.
On examination, there was a nodular swelling 5 × 5 cm in size along with smaller satellite nodules. The skin over the nodules was red, shiny. It was non-tender and freely mobile over the pectoralis muscle [Figure 1]. There was no discharge from nipple and no palpable axillary nodes. The fine needle aspiration cytology (FNAC) report was inconclusive. All the routine blood parameters and chest X-ray were normal.
A wide local excision with 1 cm margin and primary closure were done. Histopathologically, the slide showed a storiform pattern of spindle cells with greater interstitial collagenization [Figure 2]. There was sparse mitosis <5/hpf. The CD 34 histology shows uniform expression [Figure 3].
|Figure 2: Densely packed, monomorphous, plump spindle cells arranged in a storiform pattern|
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| Discussion|| |
DFSP presents as a firm area of skin most commonly about 1-5 cm in diameter. It is a slow growing tumor and is usually found on the trunk. It is also found in arms, legs, head, and neck. Its presence in the male breast is rare  and when present, it resembles malignancy. About 90% of DFSPs are low-grade sarcomas. About 10% are mixed and they contain a high-grade sarcomatous component (DFSP-FS, FS-fibrosarcoma).
There has been considerable discussion about the diagnosis and management difficulties of DFSP. The first one is the failure of FNAC to detect these tumors, which makes a core needle or incisional biopsy mandatory. The second involves absence of a consensus staging system which takes into account the lymph node involvement or the compartment involved. However, the UK sarcoma group has given recommendations in management of soft tissue sarcomas whereby in resectable tumors, resection can be done with 1 cm margin which was done in this case. ,
In diagnosis of these tumors, histopathology and immunohistochemistry play an important role. The intense staining for CD 34  in this case favors a diagnosis of DFSP. It should be kept in mind that only at the nodular stage does this hold good, as in the plaque stage it bears similarity to many fibrohistiocytic tumors. Increased age, a high mitotic index, and increased cellularity are predictors of poor clinical outcome. 
Last but not the least, the treatment also lacks consensus. Although Moh's surgery has been found to be more effective, lack of such expertise in all centers results in using the technique of wide local excision with or without plastic reconstruction, the surgery of choice. We used this technique with all margins free of tumor. As the length of tumor was greater than breadth, we could achieve a primary closure. Moh's surgery can be used in head and neck areas and wide excision in other areas. ,
Chemotherapy in the form of Imatinib has been tried with encouraging results. Similarly, radiotherapy has been tried in a limited series and the results do not favor using it regularly.
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[Figure 1], [Figure 2], [Figure 3]