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PubMed1986

Surgical treatment of dermatofibrosarcoma protuberans.

The clinical course and histopathologic factors of 50 consecutive patients treated for dermatofibrosarcoma protuberans were reviewed. Forty-eight patients were observed until the present time or death. No patient had distant metastases develop, although 16 patients had 18 recurrences of the dermatofibrosarcoma protuberans at the site of initial therapy. There was no correlation between the diameter of the primary lesion and the incidence of recurrence. There was no correlation between the histologic pattern of invasion and recurrence. However, a trend toward decreasing recurrence was noted with increasing minimal margins of resections (41 per cent less than 2 centimeters versus 24 per cent greater than or equal to 2 centimeters). The lowest incidence of recurrence (20 per cent) was noted with minimal margins of resection greater than or equal to 3 centimeters. Five year recurrence free survival rates increased with increasing margins of resection--59 per cent less than 1 centimeter; 66 per cent greater than or equal to 1 centimeter; 70 per cent greater than or equal to 2 centimeters, and 80 per cent greater than or equal to 3 centimeters. No patient had distant metastases and no change in histologic pattern was noted with progressive local recurrence.

Adolescent↗

PubMed1985

[Dermatofibrosarcoma protuberans: transition to fibrosarcoma].

We report on two patients suffering from dermatofibrosarcoma protuberans showing areas of fascicular fibrosarcoma. Whereas the mitotic rate was very low in the classic storiform parts of the tumor, the dedifferentiated areas presented an increased mitotic rate up to 10 to 15 mitoses/10 HPF. Metastases did not occur. Clinically, such a tumor may be treated like typical dermatofibrosarcoma protuberans as long as the sarcomatous component is small and not prevalent.

Back↗

PubMed1975

[DNA cytophotometry on cells of dermatofibrosarcoma protuberans (author's transl)].

Determination of DNA content was performed by Feulgen staining of tumour cell smears and DNA measurement with an integrating microdensitometer, In 3 cases of Dermatofibrosarcoma protuberans there was a DNA distribution in the range of 2 c: only a few cells showed a DNA content in the range between 2 c and 4 c; no atypical DNA distribution curves were found. The rate of proliferation in these cases of Dermatofibrosarcoma protuberans is small, there are no cytophotometric indices for malignancy.

Adult↗

PubMed1984

Dermatofibrosarcoma protuberans in black patients.

Twelve cases of dermatofibrosarcoma protuberans in Black patients admitted to Baragwanath Hospital, Johannesburg, between 1979 and 1983 are reviewed. The cause of this slow-growing, low-grade malignant fibrous histiocytoma is unknown, but there is some evidence that trauma may be a predisposing factor. The clinical and histological features of dermatofibrosarcoma protuberans are described, and treatment by wide tridimensional excision with local reconstruction is recommended.

Adolescent↗

PubMed1980

Dermatofibrosarcoma protuberans of the head and neck.

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. Four tumors were of the forehead, four were of the scalp, three were of the chin, two were of the anterior neck, one was of the lateral face, and one was of the postauricular region. Initial diagnosis was sometimes inaccurate, and initial treatment was often inadequate. The treatment of choice following diagnosis is wide local excision and careful follow-up examination.

Adolescent↗

PubMed1982

[Unusual dermatofibrosarcoma protuberans].

Case report of a recurrent histiocytic tumor with wide local spreading. Clinical findings and the course of the disease corresponded with the diagnosis of dermatofibrosarcoma protuberans. The histological findings did not correlate with this diagnosis. Signs of a typical histiocytoma with accumulation of hemosiderin and lipids were dominating. It will be discussed whether the clinical disorder of a dermatofibrosarcoma protuberans may be marked by distinctive histological criteria.

Aged↗

PubMed1980

[Dermatofibrosarcoma protuberans developing for 25 years with 27 recurrences. Histological and ultrastructural study].

The authors report the results of a histological and ultrastructure study of a case of a protuberant dermatofibrosarcoma evolving for the last 25 years with 27 relapses. Histologically the tumour is dermic, non-encapsulated, invading the hypodermis right from the beginning and mainly made up of fusiform cells which often have a storiform appearance and proliferate in a varying degree of collagenous stroma which includes an oedematous or myxoid component. During the course of the evolution of this tumour, there were zones of cystic degeneration, but in the last 10 relapses in 1978 and 1979, we noted the predominance of cellular and compact zones with an increase in the number of mitoses. The ultrastructure study showed tumour cells containing a fairly well developed endoplasmic reticulum in the cytoplasm, a prominent Golgi apparatus and some pinocytic vesicles. Some of the cells also contained lysosomes and phagolysosomes, whereas others contained cytoplasmic filaments and contractile elements. The nuclei were in general elongated and irregular. Sometimes the cytoplasmic membrane was covered by an amorphous substance with a membranoid appearance. Between the cells sometimes there were junctions of the tight kind. In conclusion, the authors consider that this tumour has the histological characteristics of a protuberant dermatofibrosarcoma and at the ultrastructure level, it is made up of cells which put it in the group of myofibroblastic and histiocytic proliferating lesions.

Fibroma↗

PubMed1993

Dermatofibrosarcoma protuberans of the hand: a case report.

Dermatofibrosarcoma protuberans is a slowly growing, low-grade sarcoma originating in the dermis and subcutaneous tissue. It generally occurs with equal frequency on the trunk and proximal extremities. Only three cases have been reported to occur on the hand. A case of dermatofibrosarcoma protuberans of the right hand anterior to Guyon's canal is reported, and the literature is reviewed.

Female↗

PubMed1977

Dermatofibrosarcoma of the face. Report of two cases from Nigeria.

Two Nigerian patients with dermatofibrosarcoma protuberans of the face are described. Both patients developed histologically typical tumours of unusual size. The condition is discussed in relation to previous reports of this unusual location and the frequency of dermatofibrosarcoma protuberans in Africa.

Adult↗

PubMed2003

Congenital dermatofibrosarcoma protuberans: variability in presentation.

BACKGROUND: Dermatofibrosarcoma protuberans (DFSP) is an uncommon low-grade fibrohistiocytic tumor that usually occurs on the trunk or proximal extremities and typically appears during the second to fifth decade of life. It most commonly begins as a red-blue plaque that grows slowly and ultimately becomes nodular. The tumor is associated with a high recurrence rate but low metastatic potential. It rarely presents in childhood and is even more rarely present at birth. The clinical diagnosis of DFSP in infancy or childhood may be difficult because, in its early stages, the tumor often resembles a vascular birthmark. OBSERVATIONS: We studied 6 patients with congenital DFSP who were initially thought to have other diagnoses, highlighting the potential clinical variability in presentation. Half of the cases in this series occurred in areas of the body outside of the typically reported distribution pattern of acquired DFSP and in locations that, therefore, may not arouse suspicion of congenital DFSP. CONCLUSIONS: Given the aggressive local potential and high recurrence rate of DFSP, early diagnosis is preferable to facilitate appropriate excision. We recommend that any infant or child presenting with a cutaneous plaque or nodule, even congenital, that does not have characteristic or diagnostic clinical features undergo tissue biopsy for histologic evaluation.

Biopsy, Needle↗

PubMed1998

Multispecialty approach to complex dermatofibrosarcoma protuberans of the forehead.

We describe a 38-year-old woman with extensive dermatofibrosarcoma protuberans of the forehead and discuss the advantages of using a multispecialty approach in the treatment of this case. The patient had had an incomplete resection 15 years previously, with a misdiagnosis of the actual tumor type. After undergoing a biopsy and a computed tomographic scan, the patient underwent Mohs micrographic surgery, followed by split-thickness grafting. Ultimate reconstruction was performed 15 months later using bilateral temporoparietal fascial flaps, in addition to split-thickness grafting. The patient remains tumor-free 3 years after resection.

Adult↗

PubMed1999

Dermatofibrosarcoma protuberans metastasizing to lymph nodes: a case report and review of literature.

Dermatofibrosarcoma Protuberans (DFSP) is an uncommon cutaneous soft tissue tumor. It is locally invasive and is known to recur. Metastases are rare and occur most commonly to the lungs. Metastasis to lymph nodes is extremely rare with only a few case reports in literature. The management strategy for such lymph node metastases remains controversial because of rarity of the condition. We report a case of DFSP of the lower limb with definite inguinal node secondaries managed by loco-regional surgery. We recommend that block dissection of lymph nodes should be performed in all cases with secondaries. J. Surg. Oncol. 1999;72;178-180.

Adult↗

PubMed1996

Cytogenetic and immunohistochemical evidence that giant cell fibroblastoma is related to dermatofibrosarcoma protuberans.

From cytogenetic and immunohistochemical findings in a primary giant cell fibrosarcoma and its recurrence, it is further demonstrated that a close relationship exists between this tumor and dermatofibrosarcoma protuberans. Both tumors express CD34 and show rearrangements of chromosomes 17 and 22.

Antigens, CD34↗

PubMed1997

Cellular fibrous histiocytoma of the skin: evidence of a clonal process with different karyotype from dermatofibrosarcoma.

Recently, a distinct variant of cutaneous fibrous histiocytoma (FH) has been histologically characterized as a "cellular" subtype. This variant is often mistaken for sarcoma, including dermatofibrosarcoma protuberans. We report a case of cellular FH of the skin in which the cytogenetic analysis demonstrated a novel chromosome pattern, possibly allowing distinction from its histologic simulants.

Adult↗

PubMed2002

Cytologic features of primary, recurrent, and metastatic dermatofibrosarcoma protuberans.

BACKGROUND: Dermatofibrosarcoma protuberans (DFSP) is a low-grade spindle cell neoplasm involving both dermis and subcutis. Its diagnosis may be difficult to render from cytologic smears, as it shares some features with other spindle cell lesions occurring in the skin and soft tissue. METHODS: Fourteen aspiration smears from 12 patients with primary, recurrent, or metastatic DFSP, examined by fine-needle aspiration biopsy (FNAB), were reviewed and compared with corresponding surgical specimens (13 aspirates) and clinical data (one aspirate). The cytologic features of DFSP were evaluated. Other spindle cell lesions in the differential diagnoses were discussed. RESULTS: Unequivocal spindle cell sarcoma diagnoses were rendered in nine aspirates, six of which were labeled correctly as DFSP in the original reports. In three aspirates, the preoperative diagnoses were inconclusive with regard to whether the tumors were benign or malignant. Two aspirates were diagnosed erroneously as benign spindle cell lesions. Cytologic features included tight clusters of bland spindle cells embedded in a collagenous/fibrillar and, often, metachromatic matrix along with dissociated, uniform, or slightly atypical spindle cells or bare nuclei. Tissue fragments showing a storiform pattern and entrapped fat tissue, reported in previous series, were less characteristic, presenting in nine and seven aspirates, respectively. CONCLUSIONS: Correct subtyping of DFSP in fine-needle aspiration smears can be difficult, due to its morphologic overlapping with other spindle cell lesions. A combination of cytology with ancillary studies and appropriate clinical information is crucial to establishing a correct diagnosis.

Adult↗

PubMed2004

Low recurrence rate after surgery for dermatofibrosarcoma protuberans: a multidisciplinary approach from a single institution.

BACKGROUND: Dermatofibrosarcoma protuberans (DFSP) is a rare sarcoma with a propensity for local recurrence. Treatments with wide excision, Mohs surgery, and other approaches have been reported with widely variable local control rates. The objective of this study was to review the experience with a multidisciplinary approach employing wide excision and Mohs surgery selectively in the treatment of patients with DFSP at a single academic institution over the past 10 years. METHODS: The records of 62 patients with 63 DFSP tumors who underwent wide excision, Mohs surgery, or a multidisciplinary combination approach from January 1991 to December 2000 were reviewed retrospectively. Primary endpoints included the ability to extirpate the DFSP lesion completely, the tumor recurrence rate, and the need for skin grafts or local tissue flaps. Additional objectives included defining surgical practice patterns at the authors' institution. RESULTS: Sixty-three DFSP lesions were removed from 62 patients. At a median follow-up of 4.4 years, no local or distant recurrences were detected in any patient. Forty-three lesions were treated with wide local excision, 11 lesions were treated with Mohs surgery, and 9 lesions were treated with a combination approach. Ninety-five percent of lesions that were approached initially with wide local excision were cleared histologically. Two patients (5%) received postoperative radiation for positive margins after undergoing maximal excision. Eighty-five percent of lesions that were approached initially with Mohs surgery were cleared histologically. The remaining 15% of lesions subsequently were cleared surgically with a wide excision. DFSP lesions that were approached initially with Mohs surgery tended to be smaller. Patients with head and neck lesions most often underwent Mohs surgery or were treated with a multidisciplinary combination approach (87%). CONCLUSIONS: Wide local excision with careful pathologic analysis of margins was found to have a very low recurrence rate and was used for the majority of patients with DFSP lesions at the authors' institution. Wide local excision, Mohs surgery, and a multidisciplinary combination approach, selected based on both tumor and patient factors, were capable of achieving very high local control rates in the treatment of DFSP. The evolution of a multidisciplinary approach has provided a level of expertise that no single individual could achieve for the treatment of the full spectrum of DFSP lesions at the authors' institution.

Adolescent↗

PubMed2004

Dermatofibrosarcoma protuberans: a report on 29 patients treated by Mohs micrographic surgery with long-term follow-up and review of the literature.

BACKGROUND: Dermatofibrosarcoma protuberans (DFSP) is an uncommon tumor of the skin. Clinically, it often masquerades as a benign, indolent tumor on the trunk and extremities. Microscopically, it extends far beyond assessed clinical margins, spreading locally in the dermis, subcutaneous tissue, and muscle. The local recurrence rate in patients with DFSP who undergo wide local excision ranges from 0% to 21%. Recent preliminary reports indicate more consistently favorable cure rates with Mohs micrographic surgery (MMS). However, to date only a few scattered reports have documented long-term 5-year follow-up. The authors present data on 29 patients with DFSP who underwent MMS. In addition, they reviewed the medical literature to summarize the accumulated experience of MMS treatment in the management of DFSP. METHODS: The authors conducted a retrospective review of a series of 40 consecutive patients with DFSP who underwent MMS over the last 20 years. Of these, there were 29 patients with > 5 years of follow-up who formed the basis of the current review. The literature also was searched for patients with DFSP who underwent MMS with > 5 years of follow-up RESULTS: At the University of Wisconsin Mohs Surgery Clinic, 29 patients with > 5 years of follow-up were treated. There were 16 women and 13 men. Eight patients developed recurrent disease after previous non-Mohs treatment. Site distribution was 45% head and neck and 55% trunk and extremities. In the current series, there were no local recurrences, with a local 5-year cure rate of 100%. In the literature review, which included the current series, there were 136 patients with DFSP who underwent Mohs surgery with > 5 years of follow-up. Nine patients in the current series developed local recurrences, including five patients who underwent a second Mohs procedure. The local cure rates after the first and second Mohs surgeries were 93.4% and 98.5%, respectively. The rate (percent) and time to local recurrence was 50% at 3 years and 75% at 5 years. However, 25% of local recurrences appeared late, after the usual 5-year recommendation. CONCLUSIONS: In a series of 29 patients with of DFSP and in an accompanying update of the medical literature, 136 patients with DFSP underwent MMS with > 5 years of follow-up. There were no regional and/or distant metastases. However, late recurrences beyond the usual recommended 5-year follow-up may occur. Therefore, all patients with DFSP, especially those with recurrent tumors, should be followed for an extended period. The accumulated data continue to confirm that, when DFSP is discovered early and is accessible readily to excision by MMS, a favorable outcome can be expected with minimal trauma or sacrifice of adjacent normal structures and with a low recurrence rate.

Adolescent↗

PubMed2004

Dermatofibrosarcoma protuberans.

In the current review, the authors set out to discuss the natural history and treatment of dermatofibrosarcoma protuberans (DFSP), a rare indolent cutaneous tumor. Approximately 10-15% of all DFSPs contain areas of fibrosarcoma (DFSP-FS), and such cases tend to exhibit more aggressive behavior. The optimal treatment for DFSP is resection with wide margins; the likelihood of local control associated with this procedure exceeds 90%. The probability of regional or distant metastases is </= 5%. Patients with positive or close surgical margins have an elevated risk of local recurrence after resection alone; however, postoperative radiotherapy results in local control rates of >/= 85% in such patients. Postoperative radiotherapy also is indicated in the rare event that a patient has unresectable macroscopic disease. Experience with the use of radiotherapy alone to treat macroscopic disease is limited.

Dermatofibrosarcoma↗