Dfsp variant represents a much more aggressive tumor with metastatic potential. Dermatofibrosarcoma protuberans dfsp is a rare soft tissue tumor with. Cd34 and factor xiiia in the differential diagnosis of dermatofibroma and dermatofibrosarcoma protuberans. Dermatofibrosarcoma protuberans genetics home reference nih.
Dermatofibrosarcoma protuberans dfsp is the most common dermal sarcoma. Dermatofibrosarcoma protuberans, lymphedema, and breast. Dermatofibrosarcoma protuberans dfsp is a locally aggressive tumour of fibroblastic origin. Dermoscopic features include whitish, red and purple areas unlike those of hemangiomas with linear and hairpin like vessels at the periphery. Full text get a printable copy pdf file of the complete article 2. This tumour was first described in 1924 by darier and ferrand as progressive and recurring dermatofibroma. It grows slowly but has a tendency to recur after excision. Dermatofibrosarcoma protuberans dfsp of the head and neck is a rare, locally infiltrative, lowgrade sarcoma. Clinicopathological features of dermatofibrosarcoma protuberans. The clinical features and histopathological appearances are described. In these situations, immunohistochemistry can be of great value in arriving at the correct diagnosis. The tumor usually starts as a small, firm patch of skin. Superficial infiltrative soft tissue neoplasm composed of bland spindle cells with a regular storiform pattern. An interphase fluorescence in situ hybridization analysis for plateletderived growth factor b pdgfb gene rearrangement was negative.
Dermatofibrosarcoma protuberans overview mayo clinic. The surgical resected margin was not diseasefree and the tumor. 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. The biopsy was reported as dermatofibrosarcoma protuberans dfsp. In 1962, taylor and helwig analyzed the histological characteristics of dfsp and in 1992 it was discovered that. Pathological records were searched for cases of dfsp in the database of the department of pathology.
As it grows, lumps of tissue protuberans may form near the surface of the skin. Nodules of histiocytes and giant cells in most cases. 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. A 57yearold woman presented with a 3year history of a progressive firm plaque on the right cheek. Clinicopathological features of dermatofibrosarcoma. Wide surgical excision is the cornerstone of treatment for dfsp. Immunohistochemistry in dermatofibrosarcoma protuberans dfsp. Combined modality had local control of 82% many of these who. Dermatofibrosarcoma protuberans genetics home reference. It is commonly found on the torso, usually in the shoulder and chest area. 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. The pigment is melanin which is positive with the fontanamasson stain, but not prussian blue. Dermatofibrosarcoma protuberanslike tumor with col1a1.
Dermatofibrosarcoma protuberans dfsp is a rare tumor of the dermis layer of the skin, and is classified as a sarcoma. For histopathology, sections were stained with hematoxylin and. Dermatofibrosarcoma protuberans consists of a proliferation of spindleshaped cells in the deep dermis with later lesions infiltrating the. Printable dermatofibrosarcoma protuberans surgical pathology. Skin biopsies revealed a bland, storiform, spindlecell proliferation involving the deep dermis and subcutaneous fat.
This is an example of pigmented dermatofibrosarcoma protuberans bednar tumor. Dermatofibrosarcoma protuberans dfsp is an uncommon locally aggressive cutaneous soft tissue sarcoma. Clinical aspect of the plaque in the lesion on the back. We report a dermatofibrosarcoma protuberans with lymph node involvement in a 27yearold woman.
A unique case of dermatofibrosarcoma protuberans with melano. Dermatofibrosarcoma protuberans dfsp accounts for less than 0. Dermatofibrosarcoma protuberans is an uncommon skin tumour arising in the deeper layer of the skin the dermis. Pdf dermatofibrosarcoma protuberans dfsp is an uncommon malignant mesenchymal. European consensusbased interdisciplinary guidelineq philippe saiaga. Dermatofibrosarcoma protuberans of the parotid gland journal of. Diagnosis and treatment of dermatofibrosarcoma protuberans. Apr 22, 2004 dermatofibrosarcoma protuberans dfsp is easily diagnosed when it shows the classical growth pattern. Dermatofibrosarcoma protuberans dfsp is a superficial, lowgrade, locally aggressive, spindle, fibroblastic, neoplastic lesion. Dermatofibrosarcoma protuberans with peculiar histologic features. Feb 09, 2016 dermatofibrosarcoma protuberans is an uncommon cancer in which tumors arise in the deeper layers of skin.
Dermatofibrosarcoma protuberans dfsp is a rare, locally infiltrative, lowgrade sarcoma that rarely metastasizes in its conventional form. Advanced dermatofibrosarcoma protuberans treatment with. Dermatofibrosarcoma protuberans and cystadenofibromas hi, i was diagnosed in march with dermatofibrosarcoma protuberans which is a very rare sarcoma. Pdgfb study with therapeutic implications, histopathology on deepdyve, the largest online rental service for scholarly research with thousands of academic publications available at your fingertips. Several studies suggest that they have a higher risk for local recurrence and distant metastases than ordinary dfsps. Dermatofibrosarcoma protuberans genetic and rare diseases.
One year later, hoffman described the tendency of the dermatofibroma tumor to develop into protruding nodules and termed the condition dermatofibrosarcoma protuberans dfsp. Dermatofibrosarcoma protuberans risks, symptoms and. Dermatofibrosarcoma protuberans of the cheek sciencedirect. This condition is a type of soft tissue sarcoma, which are cancers that affect skin, fat, muscle, and similar tissues. 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 a very rare type of skin cancer that begins in connective tissue cells in the middle layer of your skin dermis. Dermatofibrosarcoma protuberanslike tumor with col1a1 copy. Giant dermatofibrosarcoma protuberans of the face and scalp. Dermatofibrosarcoma protuberans dfsp is a rare soft tissue tumour with an incidence rate of 4. Microscopically controlled excision appears to be the treatment of choice for dermatofibrosarcoma protuberans.
Dermatofibrosarcoma protuberans dfsp, a rare lowgrade sarcoma of fibroblast origin, tends to extend in a fingerlike fashion beyond macroscopic tumor margins. Wide local excision for dermatofibrosarcoma protuberans. Dermatofibrosarcoma protuberans is a rare type of cancer that causes a tumor in the deep layers of skin. There is only one other case in literature, where a truncal dermatofibrosarcoma was reported in association with breast cancer. A new algorithm for the management of dermatofibrosarcoma. By immunohistochemistry, the tumor cells were diffusely positive for cd34 and caldesmon with multifocal reactivity for epithelial membrane antigen and focal, weak. Dermatofibrosarcoma protuberans is an uncommon cancer in which tumors arise in the deeper layers of skin. Dermatofibrosarcoma protuberans dfsp is a locally aggressive, cutaneous, malignant tumor characterized by high propensity for local relapse and low metastatic potential. 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. It grows slowly and presents usually as a nodular superficial lesion on the trunk or the extremities. It is a malignant fibroblastic tumor that most frequently arises in middleaged adults and is typically a lowgrade sarcoma that grows slowly but is associated with a high rate.
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. Review open access dermatofibrosarcoma protuberans with. The study stands out owing to its clarity in methodology, discussion, and conclusion, as well as. In many respects, the disease behaves as a benign tumor, but in 25% of cases it can metastasize, so. Retinoblastoma protein expression was not detectable in tumor cells by immunohistochemistry. Deepak kumar diwakar, nikita wadhwani and shivani paruthi. A unique case of dermatofibrosarcoma protuberans with. Dermatofibrosarcoma protuberans with lymph node and. Dermatofibrosarcoma protuberans is the most common skin sarcoma, although its incidence is very low compared with other skin tumors. Luckily, it rarely spreads to other sites beyond the skin. Cellular lesion composed of uniform small elongate cells.
The initial lesion is a skincoloured plaque which develops a redbrown colour and nodular surface histology of dermatofibrosarcoma protuberans. Differential diagnosis dermatofibrosarcoma protuberans. Dermatofibrosarcoma protuberans dfsp is an exceedingly rare subtype of soft tissue sarcoma, constituting of 1% of all sarcomas. This study aimed to determine the efficacy of wide local excision wle for controlling local recurrence of dfsp. Dermatofibrosarcoma protuberans surgical pathology criteria. It is a malignant fibroblastic tumor that most frequently arises in middleaged adults and is typically a lowgrade sarcoma that grows slowly but is associated with a high rate of local recurrence but low metastatic potential. In 1924, darier and ferrand first reported a case of progressive and recurrent dermatofibroma.
Dermatofibrosarcoma protuberans risks, symptoms and leading. 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. Radiation oncologysarcomadfsp wikibooks, open books. Pdf dermatofibrosarcoma protuberans of the breast researchgate. Pathology of dermatofibrosarcoma protuberans dfsp dr.
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. Fibrosarcomatous fs or malignant fibrous histiocytomatous mfh transformation of dermatofibrosarcoma protuberans dfsp is a rare, but well known, entity. Dermatofibrosarcoma protuberans dfsp, although rare, is one of the most common dermal sarcomas1. It presents as a slowgrowing indurated plaque on which nodules. A clinical, histologic, and molecular study of 9 cases of. 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. Dfsp is a fibrosarcoma, more precisely a cutaneous soft tissue sarcoma. Dermatofibrosarcoma protuberans dfsp is a rare softtissue neoplasm that was first. Media in category histopathology of dermatofibrosarcoma protuberans the following 15 files are in this category, out of 15 total.
Dfsp typically presents with a purple or pink asymptomatic plaque or nodule, with a. Obviously, accurate diagnosis is important, since the natural history and treatment of dfsp differs greatly from that of dffh. Dermatofibrosarcoma protuberans is a locally aggressive histiocytic tumor that, on rare occasions, may metastasize to regional lymph nodes or even to distant sites. 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.
I have just had surgery to remove cystadenofibromas 17817. Dfsps with sarcomatous areas have questionable biological behaviour. The detection of this translocation has become a useful. Original research diagnosis and treatment of dermato. A woman with postmastectomy lymphedema presented with a progressive nodular swelling on the affected limb. Mar 06, 2020 dermatofibrosarcoma protuberans dfsp accounts for less than 0. Feb 26, 2019 dermatofibrosarcoma protuberans dfsp is a rare type of cancer in which a soft tissue sarcomatous tumor forms deep in the skin. Fibrosarcomatous dermatofibrosarcoma protuberans a variant of dermatofibrosarcoma protuberans representing tumour progression. This open access thesis is brought to you for free and open access by the school of.
Infiltrates between and around individual fat cells. Pdf dermatofibrosarcoma protuberans is an uncommon, locally aggressive but rarely metastasizing tumor of the deep dermis and subcutaneous. Dermatofibrosarcoma definition of dermatofibrosarcoma by. 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. Behaviorally, dfsp rarely metastasizes occurring in 5% of all cases, and the literature on metastatic dfsp remains conspicuously sparse. Immunohistochemistry in dermatofibrosarcoma protuberans. The surgical resected margin was not diseasefree and the tumor invaded the. Dermatofibrosarcoma protuberans may at first appear as a bruise or scar. It is initially located on the skin from where it is able to infiltrate the deep structures and has a tendency to recur locally following inadequate excision. Dermatofibrosarcoma protuberans dfsp is a lowgrade malignant tumor arising from dermal and subcutaneous tissues and is the most common cutaneous sarcoma although overall still quite rare. Dermal atrophy of more than 50% of the locoregional dermis may be the predominant histopathological feature in dermatofibroma and dermatofibrosarcoma protuberans. Prospectively collected data and pathology slides were available for. It begins as a small flat lesion, rarely more than 5 cm across.
A pathology report revealed residual dfsp in the lower dermis and. Pathology outlines dermatofibrosarcoma protuberans dfsp. It is most commonly found at the trunk and proximal extremities 6. Dermatofibrosarcoma protuberans dfsp is a locally invasive and slowgrowing tumor of the subcutaneous tissue. Dermatofibrosarcoma protuberans of the head and neck. Dermatofibrosarcoma protuberans, a rare but locally aggressive. This study defines the clinical behavior of dfsp, evaluates the role of frozen section analysis, and identifies factors that predict local control. Buck dw, kim jy, alam m, multidisciplinary approach to the management of dermatofibrosarcoma protuberans. Cd34 and factorxiiia immunoreactivity in dermatofibrosarcoma protuberans and dermatofibroma. The tumor cells are typically diffusely positive for cd34, but negative for factor xiiia. Dermatofibrosarcoma protuberans dfsp typically presents during the early or midadult life, and the most. Dermatofibrosarcoma protuberans surgical pathology. 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 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%. Dermatofibrosarcoma protuberans, giant cell fibroblastoma, and hybrid lesions in children. A focal fascicular or herringbone pattern is present. Dermatofibrosarcoma protuberans rapid growth after. Therefore, incomplete removal and subsequent recurrence are common. Treatment of dermatofibrosarcoma protuberans with mohs. Interestingly, dfsp grew on a former site of nodular fasciitis and former site of a fibrous histiocytoma in the same patient.
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. Department of general surgery, vardhman mahavir medical college and safdarjung hospital, new delhi 110 029, india. The tumor typically grows slowly but has a tendency to recur. The role of radiotherapy in the local management of dermatofibrosarcoma protuberans.
Dermatofibrosarcoma protuberans pathology dermnet nz. B, typical spindle cell proliferation in the subcutis these spindle cells were positive when labeled with anticd34 antibodies not shown hematoxylineosin, original magnification. It was first described in 1924 as a progressive and recurrent dermatofibroma. Aug 30, 2012 dermatofibrosarcoma protuberans dfsp is a rare soft tissue tumor with intermediate malignancy.
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