Abstract Background: Dermatofibrosarcoma protuberans (DFSP) of the head and neck is a rare, locally infiltrative, low-grade sarcoma. This study defines the clinical behavior of DFSP, evaluates the role of frozen section analysis, and identifies factors that predict local control Dermatofibrosarcoma protuberans of the head and neck. Barnes L, Coleman JA Jr, Johnson JT. To elucidate the natural history of dermatofibrosarcoma protuberans of the head and neck, 17 cases were reviewed and compared with 92 others contained within the English literature Dermatofibrosarcoma protuberans of the head and neck is an unusual, slowly growing malignant tumor of the dermis which is locally infiltrative and recurs persistently. Fifteen cases of dermatofibrosarcoma of the head and neck were studied in detail as to age and sex of patient, location of the tumor, clinical course, and ultimate outcome Knowledge of the imaging characteristics of dermatofibrosarcoma protuberans may alert neuroradiologists to include dermatofibrosarcoma protuberans in the differential diagnosis of lesions about the head and neck with similar imaging characteristics. Imaging Findings of Head and Neck Dermatofibrosarcoma Protuberans • Dermatofibrosarcoma protuberans of the head and neck is a rare neoplasm. Overall, sarcomas account for less than 1% of all head and neck malignancies. 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 dermatofibrosarcoma protuberans after pathologic review
OBJECTIVE: To evaluate the prognosis of dermatofibrosarcoma protuberans (DFSP) in head and neck after deferent treatment. METHODS: The clinical data of 28 cases of DFSP in head and neck from 1982 to 2005 were analyzed retrospectively. Eleven cases were treated with wide excision, and 17 with limited excision Dermatofibrosarcoma protuberans (DFSP) is a rare cutaneous neoplasm associated with a high cure rate. We present a case of aggressive DFSP with fibrosarcomatous areas in the head and neck. A 28-year-old Mediterranean female presented with a 45-day history of rapidly growing cutaneous lesion of the face BACKGROUND AND PURPOSE: Dermatofibrosarcoma protuberans is a rare, locally aggressive sarcoma of the skin in children and adults, usually involving the trunk and extremities and less commonly the head and neck • To elucidate the natural history of dermatofibrosarcoma protuberans of the head and neck, 17 cases were reviewed and compared with 92 others contained within the English literature. The prototypic case is seen as a firm, solitary, slowly enlarging, asymptomatic, cutaneous nodule between 2 and 5 cm in size and of two years' duration Dermatofibrosarcoma protuberans (DFSP) is a rare, low-grade mesenchymal skin tumor. Complete surgical excision remains the mainstay of treatment. Head and neck (HN) DFSP features high local recurrence (LR) rates with wide local excision (WLE) due to both anatomical limitations and functional and cosmetic concerns
Background: Dermatofibrosarcoma protuberans (DFSP) of the head and neck is a rare, locally infiltrative, low-grade sarcoma. This study defines the clinical behavior of DFSP, evaluates the role of frozen section analysis, and identifies factors that predict local control. Methods: Hospital records and pathological slides were reviewed for 33 patients with pathologically confirmed head and neck. The remaining 35% of tumours are found on the limbs and 10 to 15% on the head and neck region. D ermatofibrosarcoma protuberans is often diagnosed when it enters a more rapid growth phase giving rise to larger lesions. Neglected tumours may reach large proportions. More examples of dermatofibrosarcoma protuberans Stojadinovic A, Karpoff HM, Antonescu CR, et al. Dermatofibrosarcoma protuberans of the head and neck. Ann Surg Oncol 2000; 7:696. Hamid R, Hafeez A, Darzi MA, et al. Outcome of wide local excision in dermatofibrosarcoma protuberans and use of radiotherapy for margin-positive disease. Indian Dermatol Online J 2013; 4:93
DESIGN: Retrospective case series of 32 patients who were seen between 1960 and 1995 with a diagnosis of dermatofibrosarcoma protuberans of the head and neck The preferred initial treatment for a localized dermatofibrosarcoma protuberans (DFSP) is resection with pathologically negative margins. However, extensive excision may not be feasible due to either anatomic limitation or cosmetic concerns, especially if found in the head and neck region . Coleman, Jr, MD; Jonas T. Johnson, MD \s=b\To elucidate the natural history of dermatofibrosarcoma protuberans of the head and neck, 17 cases were reviewed and compared with 92 others contained within the English literature. The prototyp- ic case is seen as a firm, solitary, slowly enlarging, asymptomatic, cutaneous nod
. We present a case of aggressive DFSP with fibrosarcomatous areas in the head and neck. A 28-year-old Mediterranean female presented with a 45-day history of rapidly growing cutaneous lesion of the face. Surgical biopsy confirmed the diagnosis. To elucidate the natural history of dermatofibrosarcoma protuberans of the head and neck, 17 cases were reviewed and compared with 92 others contained within the English literature. The prototypic.. Dermatofibrosarcoma protuberans (DFSP) is an uncommon tumor of the skin that grows slowly. The cell of origin in DFSP is a dermal stem cell or an undifferentiated mesenchymal cell with fibroblastic, muscular, and neurologic features. The tumor was first described by Darier and Ferrand in 1924 Mark RJBailet JWTran LMPoen JFu YSCalcaterra TC Dermatofibrosarcoma protuberans of the head and neck: a report of 16 cases. Arch Otolaryngol Head Neck Surg. 1993;119891- 896 Google Scholar Crossre Check out our super affordable Head range. 100-day risk-free returns. Shop our great deals. Explore now
Background: Dermatofibrosarcoma protuberans (DFSP) of head and neck is rare and presents a unique challenge as surgery may be associated with poor cosmetic and functional outcomes or inadequate surgical margins. There is a paucity of data regarding the use and effectiveness of imatinib in head and neck DFSP. We retrospectively analyzed head and neck DFSP cases treated with imatinib Dermatofibrosarcoma Protuberans: an unusual case of neck swelling Naeem Sultan Ali,1 Maliha Kazi,2 Beena Umar,3 Mumtaz Jamshed Khan4 Department of Otorhinolaryngology-Head & Neck Surgery,1,2,4 Department of Pathology,3 Aga Khan University Hospital, Karachi. Corresponding Author: Maliha Kazi. Email: email@example.com Case Repor Dermatofibrosarcoma protuberans is a rare locally aggressive cutaneous tumor of intermediate malignancy. It is a slow-growing neoplasm with a marked propensity to recur after resection. Head and neck involvement is unusual and distant metastases are quite rare but tend to be more frequent in tumors that undergo fibrosarcomatous degeneration
Dermatofibrosarcoma protuberans (DFSP) is a rare, infiltrative, skin soft tissue tumour of intermediate malignancy. The most common site was trunk followed by head and neck followed by extremities. Anterior abdominal wall was the most common site in our cohort. Nearly half of the tumours were between 5 and 10 cm in size. The majority (66·7. Dermatofibrosarcoma protuberans (DFSP) is a rare low-grade malignant cutaneous tumor. It was first reported by Darier and Ferrand and called progressive and recurrent skin fibroma in 1924. 1 In 1925, Hoffman named it as DFSP. 2 DFSP often occurs in the dermis and subcutaneous layers. Typical DFSP shows superficial skin nodules or masses, and often protrudes from the skin surface Analysis of Head and Neck Dermatofibrosarcoma Protuberans Christiana M. Hayward, Vikas Mehta, MD, MPH Department of Otolaryngology Louisiana State University Health Sciences Center at Shreveport INTRODUCTION MATERIALS & METHODS DISCUSSION REFERENCES SUMMARY. From 1973 to 2011, there were 7827 DFSP cases, wit
Dermatofibrosarcoma protuberans (DFSP) is an uncommon, slow growing malignant tumor of the skin. DFSP often arises in the skin of the trunk or proximal limbs, but it is rare in the head and neck. We report two cases of DFSP in the head and neck. Case 1: A 61-year-old female had a slow growing mass for 20 years in the center of the anterior neck . MATERIALS AND METHODS: We retrospectively reviewed the demographics and imaging findings in all 24 patients with pathologically proven dermatofibrosarcoma protuberans of the head and neck. Dermatofibrosarcoma Protuberans was coined by Hoffman  in 1925. DFSP are most commonly found on the trunk, followed by the extremities and the head and neck . Here we present a case of a Dermatofibrosarcoma Protuberans of the temporal region. Case Report A 64-year-old female patient, with a history of right paroti
Background . Dermatofibrosarcoma protuberans (DFSP), a rare low-grade sarcoma of fibroblast origin, tends to extend in a finger-like fashion beyond macroscopic tumor margins. Therefore, incomplete removal and subsequent recurrence are common. This study aimed to determine the efficacy of wide local excision (WLE) for controlling local recurrence of DFSP.<i> Methods</i> The trunk is the most common site of involvement for dermatofibrosarcoma protuberans, accounting for almost half of all cases [4, 8, 9]. The extremities followed by the head and neck are the next most common sites, but tumors may occur on any part of the body . Patients often ignore these tumors because of their slow growth It is most often localized to the trunk (50%), proximal extremities (35%), and head and neck (15%).1 Dermatofibrosarcoma protuberans appears as a firm, nodular, or plaque-like fixated lesion of.
Dermatofibrosarcoma protuberans of the head and neck: treatment with Mohs surgery using inverted horizontal paraffin sections. Laryngoscope 2003; 113:1289. Tan WP, Barlow RJ, Robson A, et al. Dermatofibrosarcoma protuberans: 35 patients treated with Mohs micrographic surgery using paraffin sections Dermatofibrosarcoma protuberans (DFSP) represents a low-grade soft tissue sarcoma that originates from the skin dermis layer. It is a rare tumor which accounts for about 5% of soft tissue sarcoma. They can occur in all age groups and both sexes with young adults most frequently affected. The incidence is also slightly higher in men than women Dermatofibrosarcoma protuberans (DFSP) is a low-grade malignant tumour arising from dermal and subcutaneous tissues and is the most common cutaneous sarcoma (although overall still quite rare).It is most commonly found at the trunk and proximal extremities 6.. Its behaviour is notable for a high reported rate of local recurrence, although outcomes overall are favourable
Cutaneous sarcomas are a heterogeneous group of rare mesenchymal neoplasms representing less than 1% of malignant tumors. Histology report remains the cornerstone for the diagnosis of these tumors. The most important clinicopathologic parameters related to prognosis include larger tumor size, high mitotic index, head and neck location, p53 mutations, depth of infiltration and histological. Dermatofibrosarcoma Protuberans (DFSP) is treated within the GW Cancer Center's Cutaneous Oncology Program. DFSPs are a locally aggressive soft tissue tumor considered an intermediate-to-low grade malignancy with a high recurrence rate. DFSPs originate from the connective tissue and are most often due to a chromosomal mutation. DFSPs rarely metastasis, but often can be larg
Dermatofibrosarcoma protuberans (DFSP) is a rare type of neoplasm, a locally aggressive, cutaneous, malignant tumor characterized by high propensity for local relapse and low metastatic potential. It compromises <0.1% of all neoplasms and about 1% of all soft tissue sarcomas. Approximately 85-90% of all DFSPs represent low-grade tumors Dermatofibrosarcoma protuberans (DFSP) is a rare soft-tissue malignancy of cutaneous mesenchymal cell origin representing less than 0.1% of all cancers. 1,2 DFSP is characterized by slow, infiltrative growth with substantial local invasion. Broad subclinical extension is common, with infiltration of subcutaneous tissue, fascia, or muscle. DFSP is associated with a high rate of local recurrence. Dermatofibrosarcoma protuberans (DFSP) is a slow-growing neoplasm in the dermis that appears as a firm, indurated plaque, usually either red or blue in hue. [ 78] DFSP typically appears on the. Dermatofibrosarcoma Protuberans Information for Patients What is DFSP? Dermatofibrosarcoma Protuberans (DFSP) is a tumor of the skin. It is a rare type of sarcoma (soft tissue cancer). It comprises fewer than 0.1% of all cancers and 1% of all soft tissue sarcomas. DFSP tumors occur in the dermis layer of the skin (see picture on 3rd page)
Pigmented dermatofibrosarcoma protuberans (Bednar tumor) is a rare neoplasm accounting for approximately 1-5% of all cases of dermatofibrosarcoma protuberans. The majority occurs on the trunk, and the remainders are more or less equally distributed in the upper and lower extremities and the head and neck. Microscopically it is characterized by spindled cells arranged in a tight storiform. Dermatofibrosarcoma protuberans (DFSP) is a soft tissue neoplasm of intermediate to low-grade malignancy. Although metastasis rarely occurs, DFSP has a locally aggressive behavior with a high recurrence rate. In the head and neck area, resection involving a wide margin of healthy tissue can be difficult because of functional and cosmetic considerations Season 3: OHNI Case Files - Episode 7: ImeldaImelda is a woman with a rare condition called recurrent dermatofibrosarcoma protuberans. After undergoing numer..
Dermatofibrosarcoma Protuberans Clinical. Wide age range but most common 20-40 years of age; Trunk most common, followed by proximal extremities and head and neck Very rare cases of similar appearing tumors in deep tissues and retroperitoneum Same clinical behavior ; Slow growth. What happens if dermatofibrosarcoma protuberans is left untreated? » Left untreated, dermatofibrosarcoma protuberans tend to grow into surrounding fat, muscle, and even bone. This is most common on the torso, but it can also be seen on the arms, legs, head, and neck . It is most commonly seen on the trunk followed by the proximal extremities and the head and neck.[1
Dermatofibrosarcoma protuberans is a rare fibrous tumour of skin which is slow growing and locally aggressive. DFSP is a malignant are location over head and neck, histological subtype, cellularity, size, and high mitotic rate. The treatment of choice for DFSP is surgical. Dermatofibrosarcoma Protuberans Dermatofibrosarcoma protuberans (DFSP) is a locally aggressive tumor of fibroblasts . It often presents in the second through fifth de-cades with slight male predominance . The head and neck, particularly the scalp, is the third most common location in the body with an estimated incidence of 10-15% [41.
Dermatofibrosarcoma protuberans (DFSP) is a locally aggressive tumor, uncommonly occurring on the head and neck where these deeply infiltrating tumors might violate underlying neurovascular structures. Treatment is typically surgical, whether by Mohs micrographic surgery (MMS) or wide local excision (WLE) Dermatofibrosarcoma protuberans. As dermatofibrosarcoma protuberans grows, lumps of tissue (protuberans) may form near the surface of the skin. Your skin has three layers that house your sweat and oil glands, hair follicles, melanocytes, and blood vessels. Dermatofibrosarcoma protuberans (DFSP) is a very rare type of skin cancer that begins in. There is inconclusive evidence for any advantage of MMS in non-primary cases, while MMS was most effective in treating head and neck tumors. These data may be useful to guide clinicians in the choice of the more appropriate surgical treatment for DFSP patients. KW - Dermatofibrosarcoma protuberans. KW - Mohs micrographic surgery. KW. L Barnes, JA Coleman Jr, JT Johnson: Dermatofibrosarcoma protuberans of the head and neck Arch Otolaryngol 110: 398 - 404, 1984 Crossref, Medline, Google Scholar: 10. DF Roses, Q Valensi, G LaTrenta, etal: Surgical treatment of dermatofibrosarcoma protuberans Surg Gynecol Obstet 162: 449 - 452, 1986 Medline, Google Scholar: 11
An Overview of Dermatofibrosarcoma Protuberans. DFSP is rare type of soft tissue sarcoma (cancer) that grows in the depths of the skin. It is often described as possessing tentacles that encroach onto local fat, muscle and bones. Most instances of DFSP are located in the torso; however, it has also been found on the arms, legs, head and neck Dermatofibrosarcoma protuberans (DFSP) is a malignant fibroblastic tumor with a low metastatic potential.1 These tumors most often form a nodule or ill-defined dermal plaque with an infiltrating honeycomb pattern.1 Although most tumors remain confined to the dermis, longstanding tumors can invade fascia, muscle, periosteum, and bone.1 They are most commonly located on the trunk, followed by. DERMATOFIBROSARCOMA PROTUBERANS (DFSP) What are the aims of this leaflet? This leaflet has been written to help you understand more about dermatofibrosarcoma and shoulders; however, it can also affect the limbs, head and neck and rarely the genitalia. It starts in the deep layer of the skin (the dermis) and can spread to surrounding. Dermatofibrosarcoma protuberans with fibrosarcomatous transformation: A tale of unbridled expansion Alexander T. Reid, MD, Anthony Berger, MD, MPH, and Hillary Johnson-Jahangir, MD, PhD, MHCDS then the head/ neck area.2 In the United States, the incidence is 4.2 per million people per year.3 The Nationa
Q) What is not true about dermatofibroma Protuberans (MCQS In Onco Skin) a) It arises from the fibroblasts. b) Highly malignant. c) Characterstic is translocation of 17:22. d) Imatinab is useful Background:Giant dermatofibrosarcoma protuberans (DFSP) of the scalp is a rare case, which is an intermediate grade soft tissue neoplasm originating from the dermal layer of the skin, which usually occurs in adults. Case Description:We describe such a case in a 26-year-old male.A wide local excision of the tumor with a generous tissue margin was performed; microscopic and immunohistochemical. Dermatofibrosarcoma protuberans (DFSP) is a rare type of cancer in which a soft tissue sarcomatous tumor forms deep in the skin. It begins as a small flat lesion, rarely more than 5 cm across.
Dermatofibrosarcoma protuberans (DFSP) is a rare, locally aggressive tumor arising from the skin. DFSP has an indolent growth and a high tendency to give local recurrences after excision. 1,2,3 DFSP arises from the rearrangement of chromosomes 17 and 22, with the fusion between the collagen type Iα1 gene (COL1A1) and the platelet-derived growth factor (PDGF) β-chain gene (PDGFB). 4 This. Read Dermatofibrosarcoma protuberans with regional lymph node metastasis: A case report, Head & Neck: Journal for the Sciences & Specialties of the Head and Neck on DeepDyve, the largest online rental service for scholarly research with thousands of academic publications available at your fingertips Dermatofibrosarcoma protuberans (DFSP) is a rare soft tissue tumor with intermediate malignancy. 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. A t(17;22)(q22;q13) chromosome translocation is the main cytogenetic alteration responsible for the onset of DFSP Dermatofibrosarcoma protuberans (DFSP) is a fibroblast-derived intermediate-grade soft tissue sarcoma. It is usually observed in young to middle-aged adults, although it can occur in children as either a congenital or acquired lesion. DFSP is almost twice as common in individuals of African descent and has a slight female predominance
Dermatofibrosarcoma Protuberans (DFSP) is a locally invasive and slow-growing tumor of the subcutaneous tissue. It rarely metastasizes. It was first described in 1924 as a progressive and recurrent dermatofibroma .DFSP has an annual incidence of only 0.8 cases per million and presents typically at mid-adult life with a slight male predominance  NCCN Clinical Practice Guidelines in Oncology for Dermatofibrosarcoma Protuberans. NCCN Categories of Evidence and Consensus. Category 1: Based upon high-level evidence, there is uniform NCCN consensus that the intervention is appropriate. Dermatofibrosarcoma protuberans of the head and neck Dermatofibrosarcoma protuberans (DFSP) is a slow growing, low- to intermediate-grade dermal soft-tissue tumor. It has a high local recurrence rate but low metastatic potential. It is characterized by a uniform spindle cell arrangement, classically with a storiform pattern and CD34 immunoreactivity. The histomorphology and immunophenotype overlap with a broad range of other neoplasms