Dermatofibrosarcoma protuberans genetics home reference nih. This condition is a type of soft tissue sarcoma, which are cancers that affect skin, fat, muscle, and similar tissues. Variants of dermatofibroma a histopathological study. Dfsp is most often diagnosed in individuals between the ages of 20 and 50 years and is seen mainly on the trunk and the proximal extremities. This study defines the clinical behavior of dfsp, evaluates the role of frozen section analysis, and identifies factors that predict local control. Question what is the prognostic significance of patient demographics, tumor characteristics, andor treatment modalities for patients with dermatofibrosarcoma protuberans dfsp findings in this populationbased cohort study of 3686 dfsp cases, older age, male sex, and tumor size were significantly associated with worse overall survival in a controlled analysis. 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. Clinicopathological features of dermatofibrosarcoma. Media in category histopathology of dermatofibrosarcoma protuberans the following 15 files are in this category, out of 15 total. It begins in the middle layer of skin, and tends to grow slowly. The tumor cells are typically diffusely positive for cd34, but negative for factor xiiia. Dermatofibrosarcoma protuberans dfsp is a rare tumor of the dermis layer of the skin, and is classified as a sarcoma.
The study stands out owing to its clarity in methodology, discussion, and conclusion, as well as. Pathology of dermatofibrosarcoma protuberans dfsp dr. Dermatofibrosarcoma protuberans dfsp is the most common dermal sarcoma. Dermatofibrosarcoma protuberans is a superficial and locally invasive mesenchymal tumor, which undergoes fibrosarcomatous transformation, portending a metastatic potential in 10% of. Obviously, accurate diagnosis is important, since the natural history and treatment of dfsp differs greatly from that of dffh. Dermatofibrosarcoma protuberans of the parotid gland journal of. Dermatofibrosarcoma protuberans is an uncommon spindle cell tumor, which was originally described as a distinct clinicopathologic entity in 1924 by darier and ferrand. Pathology outlines dermatofibrosarcoma protuberans dfsp. Printable dermatofibrosarcoma protuberans surgical pathology. 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. Alternative pdgfd rearrangements in dermatofibrosarcomas.
It grows slowly and presents usually as nodular superficial lesion on the trunk or the extremities. Dermatofibrosarcoma protuberans radiology reference article. Dermatofibrosarcoma protuberans dfsp is a rare tumour of skin and soft tissue. Although many times the distinction of dermatofibroma a. In histopathology, the tumor is located in the dermis and consists of uniform. Mar 06, 2020 dermatofibrosarcoma protuberans dfsp accounts for less than 0. Diagnosis and treatment of dermatofibrosarcoma protuberans. Dermatofibrosarcoma protuberans pathology dermnet nz.
Cd34 and factorxiiia immunoreactivity in dermatofibrosarcoma protuberans and dermatofibroma. Case report atrophic dermatofibrosarcoma protuberans with the. Dermatofibrosarcoma protuberans dfsp of the head and neck is a rare, locally infiltrative, lowgrade sarcoma. In the present study 26 cases of atrophic dermatofibroma were compared with three cases of atrophic dermatofibrosarcoma protuberans. Dermatofibrosarcoma protuberans dfsp is a distinctive cutaneous spindle cell neoplasm that invariably infiltrates the subcutaneous tissue. Dermatofibrosarcoma protuberans dfsp pathology outlines. Role of postoperative radiotherapy in dermatofibrosarcoma. Discussion dermatofibrosarcoma protuberans was first described by darier and ferrand in 1924 and named by hoffman one year later. Dermatofibrosarcoma protuberans dfsp is a rare skin tumor that accounts for dermatofibrosarcoma protuberans dfsp is a rare, locally infiltrative, lowgrade sarcoma that rarely metastasizes in its conventional form. Prognostic factors in dermatofibrosarcoma protuberans jama. A rare case report from north india bb mahajan 1, kumar sumir 1, monika singla 2 1 department of dermatology, guru gobind singh medical college and hospital, faridkot, punjab, india 2 department of dermatology, government medical college and hospital, chandigarh, india.
Sarcomas arising in dermatofibrosarcoma protuberans. This is an example of pigmented dermatofibrosarcoma protuberans bednar tumor. Childhood dermatofibrosarcoma protuberans, giant cell. Dermatofibrosarcoma protuberans in children and adolescents. 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 is usually easily distinguished from dermatofibroma, if an adequate biopsy is provided. A unique collaboration of multidisciplinary experts from the european dermatology forum edf, the. We present a typical case of dermatofibrosarcoma protuberans with local. Aug 23, 2015 dermatofibrosarcoma protuberans dfsp, although rare, is one of the most common dermal sarcomas1. Of 229 patients with sarcomas of the head and neck seen at our institution between 1955 and 1988, 14 7% were assigned the diagnosis of. Congenital atrophic dermatofibrosarcoma protuberans.
Dermatofibrosarcoma protuberans dfsp is a locally aggressive, cutaneous, malignant tumor characterized by high propensity for local relapse and low metastatic potential. 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. Dermatofibrosarcoma protuberans is a rare type of cancer that causes a tumor in the deep layers of skin. A focal fascicular or herringbone pattern is present.
Dermatofibrosarcoma protuberans dfsp typically presents during the early or midadult life, and the most. Immunohistochemistry in dermatofibrosarcoma protuberans. Dermatofibrosarcoma protuberans surgical pathology. How do dermatologists diagnose dermatofibrosarcoma protuberans dfsp. 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. Dermatofibrosarcoma protuberans an overview sciencedirect. In these cases, the condition is called fibrosarcomatous dermatofibrosarcoma protuberans or fsdfsp. Dermatofibrosarcoma protuberans dfsp is a lowgrade tumor with rare metastasis. The role of radiotherapy in the local management of dermatofibrosarcoma protuberans.
Other reports have suggested that exceptional cases of dfsp may be confined to the subcutaneous tissue and lack dermal involvement. Clinical diagnoses included dermatofibrosarcoma protuberans, dermatofibroma, metastasis, and lymphoma. Dermatofibrosarcoma protuberans dfsp pathophysiology. Multiple histological subtypes of dermatofibrosarcoma. Dermatofibrosarcoma protuberans dfsp is a superficial, lowgrade, locally aggressive, spindle, fibroblastic, neoplastic lesion.
As it grows, lumps of tissue protuberans may form near the surface of the skin. Cd34 and factor xiiia are difficult to interpret in many cases because of numerous entrapped reactive cells. Dermatofibrosarcoma protuberans of the head and neck is a rare neoplasm. Atrophic variant of dermatofibrosarcoma protuberans dfsp is a distinct form of dfsp. The term dermatofibrosarcoma protuberans was coined by hoffman in 1925 and, to this day, remains the preferred name among many proposed for this lesion dermatofibrosarcoma protuberans typically arises in the dermis. The detection of this translocation has become a useful. Dermatofibrosarcoma protuberans has an infiltrative pattern of growth and extensively permeates the subcutaneous fat lobules. If an excisional biopsy is available, the distinction can usually be made at scanning magnification. Treatment of d ermatofibrosarcoma protuberans and d ermatofibrosarcoma protuberans with fibrosarcomatous transformation consists of wide excision of the lesion including deep fascia, with cm margin of normal skin. Fibrosarcomatous change in dermatofibrosarcoma protuberans represents a form of tumor progression in dfsp and is associated with a significantly more aggressive clinical course than in ordinary dfsp, indicating a possible need for treatment intensification in such cases.
Dermal atrophy of more than 50% of the locoregional dermis may be the predominant histopathological feature in dermatofibroma and dermatofibrosarcoma protuberans. Dermatofibrosarcoma protuberans dfsp is a locally aggressive tumour of fibroblastic origin. The tumor usually starts as a small, firm patch of skin. Dermoscopic features include whitish, red and purple areas unlike those of hemangiomas with linear and hairpin like vessels at the periphery. For every one million people, about five to eight will develop dfsp. Dermatofibrosarcoma protuberans dfsp is a fibrohistiocytic tumour of intermediate malignancy. Radiation oncologysarcomadfsp wikibooks, open books. An incisional biopsy was performed under local anesthesia and tissue was sent for histopathology.
Dermatofibrosarcoma protuberans can begin as a minor firm area of skin most commonly about to 1 to 5 cm in diameter. Dermatofibroma, also known as fibrous histiocytoma, is one of the most common cutaneous softtissue lesions, accounting for approximately 3% of skin lesion specimens received by dermatopathology laboratories. The term dermatofibrosarcoma protuberans was coined by hoffman in 1925 and. Dfsp is a locally invasive, slowgrowing tumor of the subcutaneous tissue that rarely metastasizes but recurs frequently after surgical excision. Although these tumors are locally aggressive with high rate of recurrence following surgery. Feb 26, 2019 dermatofibrosarcoma protuberans dfsp is a rare type of cancer in which a soft tissue sarcomatous tumor forms deep in the skin. Skin nonmelanocytic tumor dermatofibrosarcoma protuberans dfsp.
A 35 years old male presented in december, 2005 with a recurrent soft tissue swelling on the left anterior chest wall which had been increasing in size for the last one year. Dermatofibrosarcoma protuberans in pediatric patients. Dermatofibrosarcoma protuberans overview mayo clinic. It begins as a small flat lesion, rarely more than 5 cm across. 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.
Controversy exists regarding margin width and excision techniques, with some advocating mohs surgery and others wide excision we. Dermatofibrosarcoma protuberans dfsp is a rare cutaneous neoplasm associated with a high cure rate. It may take more than one surgical procedure to ensure complete removal of a tumour. Immunohistochemistry in dermatofibrosarcoma protuberans dfsp. Dermatofibrosarcoma protuberans dfsps is an uncommon dermal tumor of intermediate to lowgrade malignancy. A primary squamous cell carcinoma of the orbit canadian journal. Your dermatologist can safely perform a skin biopsy. Dermatofibrosarcoma protuberans dfsp is a fibroblastic tumor with lowgrade malignant potential. 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%. More cellular than usual dermatofibrosarcoma protuberans with increased nuclear pleomorphism and mitotic figure count. Dermatofibrosarcoma protuberans dfsp is a rare skin cancer. It is a slow growing tumor and is usually found on the torso but can also be found on the arms, legs, head and neck.
Dermatofibrosarcoma protuberans with fibrosarcomatous. Overall, sarcomas account for less than 1% of all head and neck malignancies. It was first recognized by taylor 1 in 1890, and described by darrier 2 in 1924, but the term dermatofibrosarcoma protuberans was coined by hoffman 3 in 1925. Dermatofibrosarcoma durmahtoefybrosarcoma protuberans protoburanz dfsp is a rare skin. Dermatofibrosarcoma protuberans genetics home reference. Feb 26, 2019 dermatofibrosarcoma protuberans dfsp is a rare tumor that affects the deep layers of the skin. We report herein a case of highly recurrent, locally invasive dfsp that failed both postoperative radiation therapy and complete trial of imatinib, but was successfully treated with sorafenib, which showed unprecedented response. Dermatofibrosarcoma protuberans surgical pathology criteria. Dermatofibrosarcoma protuberans, giant cell fibroblastoma, and hybrid lesions in children.
Dermatofibrosarcoma protuberans dfsp is a rare soft tumor which originally represents a cutaneous sarcoma. Mar 06, 2020 a case of dermatofibrosarcoma protuberans with a ring chromosome 5 and a rearranged chromosome 22 containing amplified col1a1 and pdgfb sequences. Dermatofibrosarcoma protuberans genetic and rare diseases. 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. Dfsp is a fibrosarcoma, more precisely a cutaneous soft tissue sarcoma. It develops in the dermis, which is our second layer of skin. 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 pathology orthobullets.
Dermatofibrosarcoma protuberans consists of a proliferation of spindleshaped cells in the deep dermis with later lesions infiltrating the. A study on the prognostic value of clinical and surgical features of dermatofibrosarcoma protuberans in korean patients. 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. What is the treatment for dermatofibrosarcoma protuberans. Dermatofibrosarcoma protuberans may at first appear as a bruise or scar. Dermatofibrosarcoma protuberans dfsp is a rare dermal tumor with local recurrence rates ranging from 0 to 50%. Bednar tumor or pigmented dermatofibrosarcoma protuberans is a very rare tumor. Dermatofibrosarcoma protuberans dfsp is a rare malignant tumor of cutaneous soft tissue, with a prevalence of 0.
The lesion remained atrophic for more than 10 years. Cd34 and factor xiiia in the differential diagnosis of dermatofibroma and dermatofibrosarcoma protuberans. The standard treatment of primary tumors is surgical exci sion with safety margins of 1 to. It begins as a rough, reddish or purple patch of skin.
It was first recognized by taylor in 1890, and described by darrier in 1924, but the term dermatofibrosarcoma protuberans was coined by hoffman in 1925. 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. 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. If your dermatologist suspects you have dfsp, you will need a skin biopsy. A read is counted each time someone views a publication summary such as the title, abstract, and list of authors, clicks on a figure, or views or downloads the fulltext. Fibrosarcomatous dermatofibrosarcoma protuberans a variant of dermatofibrosarcoma protuberans representing tumour progression. Dermatofibrosarcoma definition of dermatofibrosarcoma by. Printable dermatofibrosarcoma protuberans surgical. In these situations, immunohistochemistry can be of great value in arriving at the correct diagnosis. Superficial infiltrative soft tissue neoplasm composed of bland spindle cells with a regular storiform pattern. The initial lesion is a skincoloured plaque which develops a redbrown colour and nodular surface histology of dermatofibrosarcoma protuberans. Dermatofibrosarcoma protuberans differential diagnoses.
Feb 09, 2016 dermatofibrosarcoma protuberans is an uncommon cancer in which tumors arise in the deeper layers of skin. Fibrosarcomatous change in dermatofibrosarcoma protuberans. Most tumors were asymptomatic, multinodular, protuberant masses. Differential diagnosis dermatofibrosarcoma protuberans. Combined modality had local control of 82% many of these who. Biopsy and histopathology is the es sential method to diagnose dfsp. Clinicopathological features of dermatofibrosarcoma protuberans.
Rarely, the tumors involved in the different types of dermatofibrosarcoma protuberans can have regions that look similar to fibrosarcoma, a more aggressive type of soft tissue sarcoma. Soft tissue sarcomas represent less than 1% of malignant tumors overall,1 and the prevalence of dfsp before 20 years of age is 1. We present a case of aggressive dfsp with fibrosarcomatous areas in the head and neck. Dermatofibrosarcoma protuberans of the head and neck. Spindle cell tumors occur in the pediatric population and can prove to be aggressive clinically, as in the case with dermatofibrosarcoma protuberans dfsp. Histopathology report revealed fibrosarcomatous transformation and the patient underwent adjuvant radiotherapy. Pdf dermatofibrosarcoma protuberans in children and. Dermatofibrosarcoma protuberans dfsp is a relatively unusual, locally aggressive cutaneous tumor, characterized by high rates of local recurrence, but low risk of metastasis.
Here, we report the case of a 19yearold woman with a small congenital atrophic plaque on the right precordium. 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. It may take more than one surgical procedure to ensure complete. Hematoxylin and eosin h and e stain showed spindle. 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. The pigment is melanin which is positive with the fontanamasson stain, but not prussian blue. It is most commonly found at the trunk and proximal e. Histopathology for dfsp shows a poorlycircumscribed, dense collection of spindle cells in the dermis arranged in a storiform pattern.
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