Biopsy and subsequent histopathology are key diagnostic tools. Dermatofibrosarcoma protuberans of the parotid gland journal of. Dermatofibrosarcoma protuberans dfsp is a rare tumor of the dermis layer of the skin, and is classified as a sarcoma. 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. Tissue blocks were reembedded with fresh paraffin wax and. A clinical, histologic, and molecular study of 9 cases of. Luckily, it rarely spreads to other sites beyond the skin.
Dermatofibrosarcoma protuberans dfsp is easily diagnosed when it shows the classical growth pattern. A unique case of dermatofibrosarcoma protuberans with. Fibrosarcomatous dermatofibrosarcoma protuberans a variant of dermatofibrosarcoma protuberans representing tumour progression. Dermatofibrosarcoma protuberans dfsp, although rare, is one of the most common dermal sarcomas1. Dermatofibrosarcoma protuberans in a black african population. Dermatofibrosarcoma protuberans in pediatric patients. More cellular than usual dermatofibrosarcoma protuberans with increased nuclear pleomorphism and mitotic figure count. 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. By immunohistochemistry, the tumor cells were diffusely positive for cd34 and caldesmon with multifocal reactivity for epithelial membrane antigen and focal, weak. Pathological records were searched for cases of dfsp in the database of the department of pathology, faculty of medicine ramathibodi. Cd34 and factorxiiia immunoreactivity in dermatofibrosarcoma protuberans and dermatofibroma. Soft tissue sarcomas represent less than 1% of malignant tumors overall,1 and the prevalence of dfsp before 20 years of age is 1. An interphase fluorescence in situ hybridization analysis for plateletderived growth factor b pdgfb gene rearrangement was negative.
Immunohistochemistry in dermatofibrosarcoma protuberans. In hospitals where only standard histopathological procedures are. Dermatofibrosarcoma protuberans dfsp represents a lowgrade soft tissue sarcoma that originates from the skin dermis layer. They can occur in all age groups and both sexes with young adults most frequently affected. Dermatofibrosarcoma protuberans updates in version 1. Dermatofibrosarcoma protuberans dfsp is a lowgrade tumor with rare metastasis. Pathology outlines dermatofibrosarcoma protuberans dfsp. The tumor cells are typically diffusely positive for cd34, but negative for factor xiiia.
Dermatofibrosarcoma protuberans genetics home reference. Superficial infiltrative soft tissue neoplasm composed of bland spindle cells with a regular storiform pattern. Histopathologically, dfsp is characterized by proliferation of plump, spindle cells. Immunohistochemistry in dermatofibrosarcoma protuberans dfsp. Fibrosarcomatous fs or malignant fibrous histiocytomatous mfh transformation of dermatofibrosarcoma protuberans dfsp is a rare, but well known, entity. Buck dw, kim jy, alam m, multidisciplinary approach to the management of dermatofibrosarcoma protuberans. 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%.
Nccn clinical practice guidelines in oncology nccn. The initial lesion is a skincoloured plaque which develops a redbrown colour and nodular surface histology of dermatofibrosarcoma protuberans. Dermatofibrosarcoma protuberans desp is a relatively rare and lowgrade tumor of the skin and soft tissue. In dermatofibrosarcoma protuberans, the tumor most often starts as a small, firm patch of skin, usually 1 to 5 centimeters. Dermatofibrosarcoma protuberans symptoms, prognosis. 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 genetics home reference nih.
Dermatofibrosarcoma protuberans dfsp is a locally invasive and slowgrowing tumor of the subcutaneous tissue. Dermatofibrosarcoma protuberans dfsp typically presents during the early or midadult life, and the most. Cd34 and factor xiiia in the differential diagnosis of dermatofibroma and dermatofibrosarcoma protuberans. 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. This study defines the clinical behavior of dfsp, evaluates the role of frozen section analysis, and identifies factors that predict local control. Read dermatofibrosarcoma protuberans with areas resembling giant cell fibroblastoma.
It grows slowly but has a tendency to recur after excision. Diagnosis and treatment of dermatofibrosarcoma protuberans. Dermatofibrosarcoma protuberans dfsp is a tumor of the skin. Dermatofibrosarcoma protuberans dfsp is a rare superficial tumor characterized by high rates of local recurrence and low risk of metastasis. Although metastasis is rare, dfsp can be locally aggressive, resulting in. The detection of this translocation has become a useful. Two specific genes, the growthfactor gene and the collagen gene, fuse to produce a growth factor that stimulates the abnormal growth of the tumor. 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. Spindle cell tumors occur in the pediatric population and can prove to be aggressive clinically, as in the case with dermatofibrosarcoma protuberans dfsp.
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. Dermatofibrosarcoma protuberans is an uncommon cancer in which tumors arise in the deeper layers of skin. Dfsp typically presents with a purple or pink asymptomatic plaque or nodule, with a. Dermatofibrosarcoma protuberanslike tumor with col1a1 copy. Dermatofibrosarcoma protuberans dfsp pathology outlines. Dermatofibrosarcoma protuberans is an uncommon skin tumour arising in the deeper layer of the skin the dermis. The clinical features and histopathological appearances are described. 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.
The tumor typically grows slowly but has a tendency to recur. A unique collaboration of multidisciplinary experts from the european dermatology forum edf, the. B, typical spindle cell proliferation in the subcutis these spindle cells were positive when labeled with anticd34 antibodies not shown hematoxylineosin, original magnification. 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 may at first appear as a bruise or scar. Radiation oncologysarcomadfsp wikibooks, open books. Obviously, accurate diagnosis is important, since the natural history and treatment of dfsp differs greatly from that of dffh.
Nodules of histiocytes and giant cells in most cases. Dermatofibrosarcoma protuberans with peculiar histologic features. Dermatofibrosarcoma protuberans dfsp is a locally aggressive tumour of fibroblastic origin. A unique case of dermatofibrosarcoma protuberans with melano. Dermatofibrosarcoma protuberans in children and adolescents. Pdf dermatofibrosarcoma protuberans dfsp is an uncommon malignant mesenchymal tumor. Dermatofibrosarcoma protuberans tumors arise due to a rapid division of cells which eventually results in an abnormal mass on the skin. It begins as a small flat lesion, rarely more than 5 cm across. Dfsp is a fibrosarcoma, more precisely a cutaneous soft tissue sarcoma. Pdf variants of dermatofibroma a histopathological study. Dermatofibrosarcoma protuberanslike tumor with col1a1. Dermatofibrosarcoma protuberans with areas resembling. The tumor usually starts as a small, firm patch of skin. Dermatofibrosarcoma protuberans dfsp is a fibrohistiocytic tumour of intermediate malignancy, characterized by a distinctive storiform growth pattern and frequent local recurrences.
Dermatofibrosarcoma protuberans dfsp is an uncommon infiltrative dermal and subcutaneous tumor, characterized by high rates of local recurrence and low risk of metastasis. Dfsp occurs most commonly on the trunk and proximal extremities, affects all races, and often develops between the second and fifth decade of life. A clinicopathologic study with emphasis on the predictors of recurrence and outcome proceedings. Dermal atrophy of more than 50% of the locoregional dermis may be the predominant histopathological feature in dermatofibroma and dermatofibrosarcoma protuberans. Dermatofibrosarcoma protuberans of the head and neck. Clinicopathological features of dermatofibrosarcoma. Dermatofibrosarcoma protuberans dfsp is a rare, locally infiltrative, lowgrade sarcoma that rarely metastasizes in its conventional form. 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. Feb 26, 2019 dermatofibrosarcoma protuberans dfsp is a rare skin tumor arising in the dermal layer of skin. Dermatofibrosarcoma protuberans overview mayo clinic. Infiltrates between and around individual fat cells. A focal fascicular or herringbone pattern is present. Dermatofibrosarcoma protuberans dfsp is an uncommon cutaneous tumor of intermediate malignancy that is known to be locally aggressive.
Clinicopathological features of dermatofibrosarcoma protuberans. Several studies suggest that they have a higher risk for local recurrence and distant metastases than ordinary dfsps. Dermatofibrosarcoma protuberans dfsp is a relatively unusual, locally aggressive cutaneous tumor, characterized by high rates of local recurrence, but low risk of metastasis. Original research diagnosis and treatment of dermato. Dermatofibrosarcoma protuberans is a slowgrowing tumor and does not usually spread to distant parts of the body metastasize. This tumour was first described in 1924 by darier and ferrand as progressive and recurring dermatofibroma. Dermatofibrosarcoma protuberans dfsp is an uncommon, lowgrade sarcoma of fibroblast origin with an incidence rate of 4. Media in category histopathology of dermatofibrosarcoma protuberans the following 15 files are in this category, out of 15 total. Dedifferentiated liposarcoma misdiagnosed as dermatofibrosarcoma protuberans. 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.
Summary while dermatofibrosarcoma protuberans dfsp is a rare cancer entity overall. The role of radiotherapy in the local management of dermatofibrosarcoma protuberans. 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. Retinoblastoma protein expression was not detectable in tumor cells by immunohistochemistry. Dermatofibrosarcoma protuberans dfsp is an uncommon locally aggressive cutaneous soft tissue sarcoma. 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. Immunohistochemical staining of thirteen cases with antisera to lysozyme. It is commonly found on the torso, usually in the shoulder and chest area.
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. Combined modality had local control of 82% many of these who. S1 guidelines for dermatofibrosarcoma protuberans dfsp. Dermatofibrosarcoma protuberans is a rare type of cancer that causes a tumor in the deep layers of skin. This condition is a type of soft tissue sarcoma, which are cancers that affect skin, fat, muscle, and similar tissues. It was first described in 1924 as a progressive and recurrent dermatofibroma.
Dermatofibrosarcoma protuberans consists of a proliferation of spindleshaped cells in the deep dermis with later lesions infiltrating the. Feb 09, 2016 dermatofibrosarcoma protuberans is an uncommon cancer in which tumors arise in the deeper layers of skin. Dermatofibrosarcoma protuberans dfsp of the head and neck is a rare, locally infiltrative, lowgrade sarcoma. Dfsps with sarcomatous areas have questionable biological behaviour. Fibrosarcomatous transformation fibrosarcoma exdfsp imparts an increased risk of. The tumor looks like a small harmless bump under the skin in. The pigment is melanin which is positive with the fontanamasson stain, but not prussian blue. Dermatofibrosarcoma protuberans dfsp is a superficial, lowgrade, locally aggressive, spindle, fibroblastic, neoplastic lesion. The diagnosis is based on the clinical aspect, histopathological and immunohistochemical evaluation. Dermatofibrosarcoma protuberans genetic and rare diseases. Differential diagnosis dermatofibrosarcoma protuberans. In these situations, immunohistochemistry can be of great value in arriving at the correct diagnosis. Sclerosing dermatofibrosarcoma protuberans dfsp wiley online. Dfsp tumors occur in the dermis layer of the skin see picture on 3rd page.
It is a rare tumor which accounts for about 5% of soft tissue sarcoma. Dermatofibrosarcoma protuberans surgical pathology. The overall rate of distant metastasis is estimated to be around 4%, and the rate of metastasis to nearby lymph nodes is 1%. Mar 14, 2011 dermatofibrosarcoma protuberans tumors arise due to a rapid division of cells which eventually results in an abnormal mass on the skin. Cellular lesion composed of uniform small elongate cells.
Incomplete resection is a risk factor for recurrence. Clinical features, pathological findings and treatment of. Pathology of dermatofibrosarcoma protuberans dfsp dr. Dermatofibrosarcoma protuberans rapid growth after. In the present study 26 cases of atrophic dermatofibroma were compared with three cases of atrophic dermatofibrosarcoma protuberans. Pdf dermatofibrosarcoma protuberans in children and. Dermatofibrosarcoma protuberans surgical pathology criteria. Dfsp2 under therapy for recurrencemetastasis for recurrence. The incidence is also slightly higher in men than women. B, the spindle cells are immunoreactive to cluster of differentiation 34 cd34. A 57yearold woman presented with a 3year history of a progressive firm plaque on the right cheek. Printable dermatofibrosarcoma protuberans surgical.
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