Atrophic variants of dermatofibroma and dermatofibrosarcoma. The diagnosis is based on the clinical aspect, histopathological and immunohistochemical evaluation. Dfsp2 under therapy for recurrencemetastasis for recurrence. Dermal atrophy of more than 50% of the locoregional dermis may be the predominant histopathological feature in dermatofibroma and dermatofibrosarcoma protuberans. Pathological records were searched for cases of dfsp in the database of the department of pathology, faculty of medicine ramathibodi. Buck dw, kim jy, alam m, multidisciplinary approach to the management of dermatofibrosarcoma protuberans. The clinical features and histopathological appearances are described. Dermatofibrosarcoma protuberans dfsp typically presents during the early or midadult life, and the most. Dermatofibrosarcoma protuberans dfsp is a relatively unusual, locally aggressive cutaneous tumor, characterized by high rates of local recurrence, but low risk of metastasis. The detection of this translocation has become a useful. Incomplete resection is a risk factor for recurrence. A 26yearold male presented with multiple cutaneous nodular lesions of dfsp since 3 months along with distant metastasis to the brain, pleura, and muscles that were detected on investigations. Dermatofibrosarcoma protuberans dfsp is a rare tumor of the dermis layer of the skin, and is classified as a sarcoma.
Dermatofibrosarcoma protuberans in pediatric patients. Dermatofibrosarcoma protuberanslike tumor with col1a1. Cellular lesion composed of uniform small elongate cells. Original research diagnosis and treatment of dermato. Dermatofibrosarcoma protuberans overview mayo clinic. Dermatofibrosarcoma protuberans dfsp is a lowgrade tumor with rare metastasis. Spindle cell tumors occur in the pediatric population and can prove to be aggressive clinically, as in the case with dermatofibrosarcoma protuberans dfsp. Histopathologically, dfsp is characterized by proliferation of plump, spindle cells. The role of radiotherapy in the local management of dermatofibrosarcoma protuberans. The tumor cells are typically diffusely positive for cd34, but negative for factor xiiia. Dermatofibrosarcoma protuberans dfsp is a very rare type of skin cancer that begins in connective tissue cells in the middle layer of your skin dermis. Dermatofibrosarcoma protuberans is an uncommon skin tumour arising in the deeper layer of the skin the dermis.
Dfsps with sarcomatous areas have questionable biological behaviour. Infiltrates between and around individual fat cells. It is commonly found on the torso, usually in the shoulder and chest area. Dermatofibrosarcoma protuberans dfsp is easily diagnosed when it shows the classical growth pattern. Skin biopsies revealed a bland, storiform, spindlecell proliferation involving the deep dermis and subcutaneous fat.
A, the tumor shows adipocytic and spindle cell morphology mimicking dermatofibrosarcoma protuberans. The pigment is melanin which is positive with the fontanamasson stain, but not prussian blue. Jan 28, 2020 dermatofibrosarcoma protuberans dfsp is a very rare type of skin cancer that begins in connective tissue cells in the middle layer of your skin dermis. Dermatofibrosarcoma protuberans is a rare type of cancer that causes a tumor in the deep layers of skin. Dermatofibrosarcoma protuberans dfsp is not a common in most reports and originates from the dermis, it is a slow growing, nodular, polypoid neoplasm that invade the subcutaneous tissue and rarely metastasis. Fibrosarcomatous dermatofibrosarcoma protuberans a variant of dermatofibrosarcoma protuberans representing tumour progression. Two specific genes, the growthfactor gene and the collagen gene, fuse to produce a growth factor that stimulates the abnormal growth of the tumor. Cd34 and factor xiiia in the differential diagnosis of dermatofibroma and dermatofibrosarcoma protuberans. Combined modality had local control of 82% many of these who. This tumour was first described in 1924 by darier and ferrand as progressive and recurring dermatofibroma. Clinicopathological features of dermatofibrosarcoma protuberans. Pdf dermatofibrosarcoma protuberans dfsp is an uncommon malignant mesenchymal tumor. Radiation oncologysarcomadfsp wikibooks, open books. In these situations, immunohistochemistry can be of great value in arriving at the correct diagnosis.
Dermatofibrosarcoma protuberans dfsp is an uncommon cutaneous tumor of intermediate malignancy that is known to be locally aggressive. In the present study 26 cases of atrophic dermatofibroma were compared with three cases of atrophic dermatofibrosarcoma protuberans. More cellular than usual dermatofibrosarcoma protuberans with increased nuclear pleomorphism and mitotic figure count. Sclerosing dermatofibrosarcoma protuberans dfsp wiley online. Pdf variants of dermatofibroma a histopathological study.
The tumor usually starts as a small, firm patch of skin. Pdf dermatofibrosarcoma protuberans in children and. A clinical, histologic, and molecular study of 9 cases of. Dfsp tumors occur in the dermis layer of the skin see picture on 3rd page. S1 guidelines for dermatofibrosarcoma protuberans dfsp.
Tissue blocks were reembedded with fresh paraffin wax and. This is an example of pigmented dermatofibrosarcoma protuberans bednar tumor. Dermatofibrosarcoma protuberans dfsp is an uncommon, lowgrade sarcoma of fibroblast origin with an incidence rate of 4. Clinical features, pathological findings and treatment of. Dermatofibrosarcoma protuberans surgical pathology. It was first described in 1924 as a progressive and recurrent dermatofibroma. Dermatofibrosarcoma protuberans is a slowgrowing tumor and does not usually spread to distant parts of the body metastasize. Dermatofibrosarcoma protuberans dfsp is a tumor of the skin. The overall rate of distant metastasis is estimated to be around 4%, and the rate of metastasis to nearby lymph nodes is 1%. Dermatofibrosarcoma protuberans dfsp is an uncommon locally aggressive cutaneous soft tissue sarcoma. It grows slowly but has a tendency to recur after excision. To begin with, i would like to congratulate the authors of the work entitled, extended resection in the treatment of dermatofibrosarcoma protuberans, published in the brazilian journal of plastic surgery volume 29 number 3, pages 395403, 2014. Soft tissue sarcomas represent less than 1% of malignant tumors overall,1 and the prevalence of dfsp before 20 years of age is 1.
The tumor looks like a small harmless bump under the skin in. Clinicopathological features of dermatofibrosarcoma. Dermatofibrosarcoma protuberanslike tumor with col1a1 copy. B, the spindle cells are immunoreactive to cluster of differentiation 34 cd34. In many respects, the disease behaves as a benign tumor, but in 25% of cases it can metastasize, so it should be considered to have malignant potential. Feb 09, 2016 dermatofibrosarcoma protuberans is an uncommon cancer in which tumors arise in the deeper layers of skin. Immunohistochemistry in dermatofibrosarcoma protuberans. Obviously, accurate diagnosis is important, since the natural history and treatment of dfsp differs greatly from that of dffh. The initial lesion is a skincoloured plaque which develops a redbrown colour and nodular surface histology of dermatofibrosarcoma protuberans. Summary while dermatofibrosarcoma protuberans dfsp is a rare cancer entity overall.
As a relatively uncommon neoplasm and locally aggressive cutaneous tumor, it is characterized by high rates of local recurrence, but a low risk of metastasis 14. Dermatofibrosarcoma protuberans updates in version 1. They can occur in all age groups and both sexes with young adults most frequently affected. The study stands out owing to its clarity in methodology, discussion, and conclusion, as well as. Dermatofibrosarcoma protuberans dfsp is a rare superficial tumor characterized by high rates of local recurrence and low risk of metastasis. Several studies suggest that they have a higher risk for local recurrence and distant metastases than ordinary dfsps. Dermatofibrosarcoma protuberans genetics home reference. Dermatofibrosarcoma protuberans tumors arise due to a rapid division of cells which eventually results in an abnormal mass on the skin. In hospitals where only standard histopathological procedures are.
Pathology outlines dermatofibrosarcoma protuberans dfsp. Fibrosarcomatous fs or malignant fibrous histiocytomatous mfh transformation of dermatofibrosarcoma protuberans dfsp is a rare, but well known, entity. In dermatofibrosarcoma protuberans, the tumor most often starts as a small, firm patch of skin, usually 1 to 5 centimeters. Dermatofibrosarcoma protuberans dfsp is a rare soft tissue tumor, presenting on the trunk in about 50% of cases, the proximal extremities in 2035% of cases, and the head and neck in about 1015. Dermatofibrosarcoma protuberans dfsp is a fibrohistiocytic tumour of intermediate malignancy, characterized by a distinctive storiform growth pattern and frequent local recurrences. Biopsy and subsequent histopathology are key diagnostic tools. A unique case of dermatofibrosarcoma protuberans with. Dermatofibrosarcoma protuberans in a black african population. Superficial infiltrative soft tissue neoplasm composed of bland spindle cells with a regular storiform pattern. Dermatofibrosarcoma protuberans dfsp is an uncommon infiltrative dermal and subcutaneous tumor, characterized by high rates of local recurrence and low risk of metastasis. Dermatofibrosarcoma protuberans of the parotid gland journal of. Dermatofibrosarcoma protuberans dfsp is a rare, locally infiltrative, lowgrade sarcoma that rarely metastasizes in its conventional form. A clinicopathologic study with emphasis on the predictors of recurrence and outcome proceedings.
Dermatofibrosarcoma protuberans dfsp is a rare dermal tumor comprising approximately 1% of soft tissue sarcomas with typically indolent growth over years and a probability of regionaldistant metastases of less than 23%. Mar 14, 2011 dermatofibrosarcoma protuberans tumors arise due to a rapid division of cells which eventually results in an abnormal mass on the skin. A unique case of dermatofibrosarcoma protuberans with melano. Dermatofibrosarcoma protuberans genetic and rare diseases.
Dermatofibrosarcoma protuberans of the head and neck. Cd34 and factorxiiia immunoreactivity in dermatofibrosarcoma protuberans and dermatofibroma. An interphase fluorescence in situ hybridization analysis for plateletderived growth factor b pdgfb gene rearrangement was negative. Pathology of dermatofibrosarcoma protuberans dfsp dr. This condition is a type of soft tissue sarcoma, which are cancers that affect skin, fat, muscle, and similar tissues in dermatofibrosarcoma protuberans, the tumor most often starts as a small, firm patch of skin, usually 1 to 5 centimeters in diameter, that is usually purplish, reddish. Case reprot open access gigantic dermatofibrosarcoma protuberans of the scalp with fibrosarcomatous transformation 1section of plastic and reconstructive surgery, department of surgery king faisal specialist hospital and research center jeddah, saudi arabia 2department of pathology and laboratory medicine king faisal specialist hospital and research center jeddah, saudi arabia. Dermatofibrosarcoma protuberans dfsp is a locally invasive and slowgrowing tumor of the subcutaneous tissue.
Immunohistochemical staining of thirteen cases with antisera to lysozyme. It begins as a small flat lesion, rarely more than 5 cm across. Read dermatofibrosarcoma protuberans with areas resembling giant cell fibroblastoma. Feb 26, 2019 dermatofibrosarcoma protuberans dfsp is a rare type of cancer in which a soft tissue sarcomatous tumor forms deep in the skin. Excision biopsy of lump was done and histopathology reults stated a spindle celled neoplasm, consisting of monomorphic spindle cells forming whorls and are arranged in storiform pattern. Diagnosis and treatment of dermatofibrosarcoma protuberans. Nccn clinical practice guidelines in oncology nccn. Dermatofibrosarcoma protuberans dfsp, although rare, is one of the most common dermal sarcomas1. Dermatofibrosarcoma protuberans desp is a relatively rare and lowgrade tumor of the skin and soft tissue.
Retinoblastoma protein expression was not detectable in tumor cells by immunohistochemistry. Dermatofibrosarcoma protuberans may at first appear as a bruise or scar. Luckily, it rarely spreads to other sites beyond the skin. Printable dermatofibrosarcoma protuberans surgical. This study defines the clinical behavior of dfsp, evaluates the role of frozen section analysis, and identifies factors that predict local control. Dermatofibrosarcoma protuberans dfsp represents a lowgrade soft tissue sarcoma that originates from the skin dermis layer. The tumor typically grows slowly but has a tendency to recur. Feb 26, 2019 dermatofibrosarcoma protuberans dfsp is a rare skin tumor arising in the dermal layer of skin.
This condition is a type of soft tissue sarcoma, which are cancers that affect skin, fat, muscle, and similar tissues. By immunohistochemistry, the tumor cells were diffusely positive for cd34 and caldesmon with multifocal reactivity for epithelial membrane antigen and focal, weak. Dermatofibrosarcoma protuberans symptoms, prognosis. Dermatofibrosarcoma protuberans dfsp of the head and neck is a rare, locally infiltrative, lowgrade sarcoma.
Dermatofibrosarcoma protuberans rapid growth after. Dermatofibrosarcoma protuberans in children and adolescents. Immunohistochemistry in dermatofibrosarcoma protuberans dfsp. Dermatofibrosarcoma protuberans with areas resembling. Dermatofibrosarcoma protuberans dfsp pathology outlines. A unique collaboration of multidisciplinary experts from the european dermatology forum edf, the. Dermatofibrosarcoma protuberans consists of a proliferation of spindleshaped cells in the deep dermis with later lesions infiltrating the. Dermatofibrosarcoma protuberans surgical pathology criteria. Dfsp typically presents with a purple or pink asymptomatic plaque or nodule, with a. Pdf dermatofibrosarcoma protuberans of the breast researchgate.
Fibrosarcomatous transformation fibrosarcoma exdfsp imparts an increased risk of. European consensusbased interdisciplinary guidelineq philippe saiaga. Although metastasis is rare, dfsp can be locally aggressive, resulting in. Dfsp occurs most commonly on the trunk and proximal extremities, affects all races, and often develops between the second and fifth decade of life. Dermatofibrosarcoma protuberans genetics home reference nih. Dermatofibrosarcoma protuberans is an uncommon cancer in which tumors arise in the deeper layers of skin.
Dedifferentiated liposarcoma misdiagnosed as dermatofibrosarcoma protuberans. Dfsp is a fibrosarcoma, more precisely a cutaneous soft tissue sarcoma. Media in category histopathology of dermatofibrosarcoma protuberans the following 15 files are in this category, out of 15 total. A focal fascicular or herringbone pattern is present. Dermatofibrosarcoma protuberans dfsp is a superficial, lowgrade, locally aggressive, spindle, fibroblastic, neoplastic lesion. Dermatofibrosarcoma protuberans with peculiar histologic features. Approximately 85 to 90 percent of dfsps are low grade, while the remainder contain a highgrade sarcomatous component which is usually a fibrosarcoma, designated dfspfs and are considered to be intermediategrade sarcomas 1.
Differential diagnosis dermatofibrosarcoma protuberans. As it grows, lumps of tissue protuberans may form near the surface of the skin. It is a lowgrade, slow growing fibroblastic malignant neoplasm most frequently affecting middle aged adults and characterized by a high local recurrence rate and a low propensity for metastasis. It is a rare tumor which accounts for about 5% of soft tissue sarcoma. The incidence is also slightly higher in men than women. B, typical spindle cell proliferation in the subcutis these spindle cells were positive when labeled with anticd34 antibodies not shown hematoxylineosin, original magnification. Nodules of histiocytes and giant cells in most cases. In many respects, the disease behaves as a benign tumor, but in 25% of cases it can metastasize, so.
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