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PubMed1994

[Dermatofibrosarcoma protuberans. A clinico-pathologic analysis of 5 cases].

The author study the clinical histories and the histopathologic findings in five patients who were hospitalized in the National Oncological Institute, between 1983 and 1993 in whom the diagnosis of Dermatofibrosarcoma protuberans was established. He note the age, sex, localization of the tumor, the frequency and cause of recurrence; he discuss the differential diagnosis with benign fibrous histiocytoma and neurofibroma and, since recurrence may exhibit mixoid changes (as occurred in two of the cases), also discuss the differential diagnosis with mixoid liposarcoma and mixoid malignant fibrous histiocytoma and pseudo sarcomatous nodular fascitis.

Adolescent↗
PubMed1993

[Ultrastructural study of 14 cases of dermatofibrosarcoma protuberans].

14 cases of dermatofibrosarcoma protuberans (DFSP) were investigated with electron microscopy. Perineural cells, fibroblasts and primitive mesenchymal cells were found in all cases with perineural cells as the most prominent component. The findings suggest that DFSP is a tumor with heterogeneity, most probably arising from primitive dermal mesenchymal cells which have the potential for differentiation toward different cell lines, especially toward perineural cells.

Adolescent↗
PubMed1996

A comparison between Mohs micrographic surgery and wide surgical excision for the treatment of dermatofibrosarcoma protuberans.

BACKGROUND: There is growing evidence that Mohs micrographic surgery (MMS) is the treatment of choice for dermatofibrosarcoma protuberans (DFSP). OBJECTIVE: This study retrospectively compared the recurrence rates of DFSP after MMS with those after wide surgical excision; results at the Mayo Clinic and in the world literature were evaluated. In addition, preoperative tumor sizes and postoperative defect sizes after MMS were compared to determine whether MMS conserved more normal tissue than wide surgical excision. METHODS: The medical records of 84 patients with DFSP who had been treated at the Mayo Clinic were reviewed. They were categorized into two treatment groups: MMS and surgical excision. RESULTS: Fifteen patients with DFSP who underwent MMS had follow-up data available; one of these patients had local recurrence (recurrence rate, 6.6%; average duration of follow-up, 40 months). Thirty-nine patients had wide excision; four of these patients had local recurrences and one had pulmonary metastases (recurrence rate, 10%; average duration of follow-up, 36 months). A review of the world literature revealed neither local recurrences nor metastases in the 11 studies in which DFSP was treated with MMS. Overall, including our results, the average recurrence rate of DFSP after MMS was 0.6% (range, 0% to 6.6%) and the total recurrence rate was 1.6% (1 of 64). Including our series, DFSP was treated with wide excision in 15 studies; the average recurrence rate was 18% (range, 0% to 60%) and the total recurrence rate was 20% (100 of 489). In eight published studies, DFSP was surgically resected with undefined or conservative excisional margins; the average recurrence rate was 43% (range, 26% to 60%) and the total recurrence rate was 44% (140 of 317). A surgical margin of 2.5 cm to deep fascia was required for complete clearance of all tumors treated with MMS. Twenty-two percent of tumors were removed with a 0.5 cm margin, 50% with a 1.0 cm margin, 67% with a 1.5 cm margin, and 89% with a 2.0 cm margin. CONCLUSION: On the basis of our results and data compiled from the literature, MMS may be the treatment of choice for DFSP because of its high cure rate and maximal conservation of tissue.

Adolescent↗
PubMed1996

Dermatofibrosarcoma protuberans of the vulva. A case report.

BACKGROUND: Dermatofibrosarcoma protuberans (DFSP) is a cutaneous malignancy that is uncommon anywhere on the body but is especially uncommon on the vulva. This cancer rarely metastasizes but has a high incidence of local recurrence because of its tendency to grow in fingerlike projections away from the primary tumor. CASE: A 36-year-old, black woman was the 10th and youngest patient with DFSP of the vulva to be reported. Treatment of the 5-cm, mobile, well-circumscribed lesion consisted of local excision. Several adjacent microscopic lesions showing DFSP were found in the pathologic specimen, however, so an even wider excision was performed. Twenty-seven months following removal, there was no evidence of recurrence. CONCLUSION: Survival rates for DFSP of the skin range from 91% to 100% in reported series, but local recurrence rates of 20-49% have also been noted. The reported deaths from the disease have resulted from extensive local spread due to inadequate excision and, only rarely, metastases. Because DFSP tends to spread to microscopic projections away from the visible lesions, very wide local excision is required for tumor control.

Adult↗
PubMed1996

Dermatofibrosarcoma protuberans: a clinicopathological study in an African population.

Dermatofibrosarcoma protuberans (DFSP) is a slow-growing, locally aggressive fibrous tumour which rarely metastasises to regional lymphnode or distant sites. The rarity, age pattern of occurrence and the male to female ratio of the tumour were found to correspond to reports elsewhere. But contrary to other reports, the lower limb and not the trunk was found to be the site of predilection of the tumour in Tanzania. This and the tendency of occurrence of the tumour on the scalp in females rather than at other sites in the head and neck region seem to implicate trauma as a predisposing factor. It is difficult to differentiate between DFSP, keloids and hypertrophi scars on the basis of clinical presentation without biopsy. The treatment of choice of this lesion is wide excision followed by skin grafting. It is emphasised that all hypertrophi scars and keloids should be histologically examined in order to rule out DFSP so that it is correctly treated in order to avoid recurrence.

Adolescent↗
PubMed1997

Dermatofibrosarcoma protuberans (DFSP): growth characteristics based on tumor modeling and a review of cases treated with Mohs micrographic surgery.

Dermatofibrosarcoma protuberans (DFSP) is a relatively rare cutaneous tumor that is clinically challenging since it is locally highly invasive and aggressive, although it rarely metastasizes. Traditionally, wide and deep local surgical excision has been regarded as the treatment of choice for DFSP. However, even 3-cm-wide local excision margins have resulted in a local recurrence rate of 11%. In recent years, reports of DFSPs successfully treated with Mohs micrographic surgery have been appearing in the literature. The DFSP literature is reviewed here, including worldwide experience reported to date in the use of Mohs surgery to excise DFSPs. Using this margin control technique, the recurrence rate is shown to be 2.4%, much lower than the best previously reported recurrence rate of 11% when wide local excision was used. Three-dimensional reconstructions of DFSPs, based on Mohs micrographic surgical technique, are also presented. These illustrations provide new insight into the growth characteristics of these tumors and why Mohs micrographic surgery is emerging as the treatment of choice for DFSP.

Adult↗
PubMed1997

Encapsulated uninodular dermatofibrosarcoma protuberans of the dermis: a case report.

We report an unusual case of dermatofibrosarcoma protuberans presenting as a small encapsulated uninodular mass, located exclusively in the dermis. Differential diagnostic problems with nodular spindle-shaped cell lesions of the dermis are discussed. A distinct histologic finding was the presence of a myofibroblast-rich fibrous pseudocapsule surrounding the tumor. The possible origin and role of this pseudocapsule are proposed.

Actins↗
PubMed1997

[Pigmented fibrosarcomatous dermatofibrosarcoma protuberans (Bednar tumor). 3 case reports, analogy with the "conventional" type and review of the literature].

BACKGROUND: The so-called Bednar tumor represents a pigmented variant of dermatofibrosarcoma protuberans (DFSP) and is characterized by a usually scant (1-5% of cells) population of dendritic melanocytes within an otherwise typical DFSP. This pigmented variant accounts for up to 5% of all DFSPs. Other variants of DFSP include cases showing features (in the primary or recurrence) of either giant cell fibroblastoma or fibrosarcoma. Less than 5% of DFSPs are associated with metastases and many of these show either a fibrosarcomatous component or, much more rarely, an "MFH"-like appearance. Only one previous case has been reported which showed combined features of the pigmented and fibrosarcomatous variants. MATERIALS: We present herein 3 cases of fibrosarcomatous Bednar tumor, all occurring in males, 2 aged 75 and 1 aged 23; two patients were white and one black. The tumors were located on the trunk or shoulder and two had been present for many years with recent rapid growth. One patient developed local recurrence and metastases to bone and lung and died within 1 year. The other two patients are disease free at 3 and 5 years follow-up respectively. All three cases showed typical histological features and in two tumors the pigment was evident macroscopically. CONCLUSIONS: A through literature review, including all cases of fibrosarcomatous DFSP and metastasizing fibrosarcomatous DFSP (whether or not pigmented), confirms that the fibrosarcomatous variant of DFSP (including its pigmented counterpart) is significantly more aggressive than usual DFSP, thus underlining the importance of its accurate recognition.

Adult↗
PubMed1998

Giant cell fibroblastoma associated with dermatofibrosarcoma protuberans: a case report.

A case of congenital giant cell fibroblastoma (GCF) associated with dermatofibrosarcoma protuberans (DFSP) in a 1.5-year-old girl is reported. The GCF component was characterized by a mixed proliferation of fibroblastic cells and multinucleated giant cells and sinusoid-like spaces. The DFSP component, which was spatially distinct from the GCF region, was composed of short spindle-shaped cells arranged in a storiform pattern. Mitotic figures were frequently identified in the DFSP area but not in the GCF region. Immunohistochemically, tumor cells in both components were positive for CD34. The Ki-67 labeling index of the DFSP area (4.9%) was markedly higher than that of the GCF (0.5%). Ultrastructurally, spindle-shaped tumor cells in both components, as well as giant cells, were fibroblastic cells that frequently exhibited multilobulated nuclei. This case illustrates the close histogenetic relationship between GCF and DFSP, although the degree of proliferative activity is much higher in the DFSP area.

Dermatofibrosarcoma↗
PubMed1998

Deep dermatofibrosarcoma protuberans: a subcutaneous variant.

AIMS: Dermatofibrosarcoma protuberans (DFSP) is a distinctive cutaneous spindle cell neoplasm that invariably infiltrates the subcutaneous tissue. Other reports have suggested that exceptional cases of DFSP may be confined to the subcutaneous tissue and lack dermal involvement. We wish to confirm this observation by describing cases of a rare variant of DFSP confined to the subcutaneous tissue, and analyse possible histogenetic implications. METHODS AND RESULTS: Three cases of DFSP located in the subcutaneous tissue are reported. Multiple step sections demonstrated the lack of dermal involvement in two of them, whereas the third case infiltrated the dermis at the junction with the subcutis minimally in one of five blocks. Immunohistochemical studies using a battery of monoclonal antibodies were performed. All the tumours stained strongly for vimentin and CD34. CONCLUSIONS: Because of the lack of dermal involvement in two cases and only minimal dermal involvement in one case, we called this variant deep DFSP. Except for deep setting of the tumour, deep DFSP is indistinguishable from typical DFSP clinically, histologically and immunohistochemically. The existence of deep DFSP provides evidence that specific structures of the skin may be not involved in this tumour's histogenesis.

Adult↗
PubMedDate not supplied

Metastatic dermatofibrosarcoma protuberans mimicking meningioma.

Dermatofibrosarcoma protuberans (DFSP) is a locally aggressive fibrohistiocytic tumor of intermediate malignancy with potential for local recurrences and with a small, but well recognized risk of distant metastases. We report a case of a 43-year-old Filipino male with an otherwise typical cutaneous DFSP followed 4 years later by a local recurrence of tumor with subsequent pulmonary metastasis at year 5, and 3 sequential central nervous system (CNS) metastases at years 5, 6, and 12 following the original diagnosis of DFSP. Recurrent and metastatic tumors showed areas of fibrosarcomatous change, high cellularity, high mitotic rate (10 - 15 per 10 high power fields), loss of CD34 expression, and aneuploidy. DFSP has not been previously reported to metastasize to the CNS. In addition, we report complex karyotypic aberrations within the metastasis, which have not been previously associated with DFSP. The dural-based location of the intracranial metastasis, together with the light- and electron-microscopic appearance, could cause diagnostic confusion with an anaplastic syncytial meningioma.

Adult↗
PubMed1998

Various regions within the alpha-helical domain of the COL1A1 gene are fused to the second exon of the PDGFB gene in dermatofibrosarcomas and giant-cell fibroblastomas.

Dermatofibrosarcoma protuberans (DFSP) and its juvenile form, giant-cell fibroblastoma (GCF), are uncommon infiltrative tumors of the dermis, which present unique cytogenetic features, such as the reciprocal translocation t(17;22) or, more commonly, supernumerary ring chromosomes containing sequences from chromosomes 17 and 22. We have recently shown that these aberrations are cytogenetic manifestations of gene fusions between the platelet-derived growth factor B-chain gene (PDGFB), the cellular equivalent of the v-sis oncogene, and the collagen type 1 alpha 1 gene (COL1A1), the major protein constituent of the extracellular matrix in connective tissue of skin. We now report characterization of COL1A1/PDGFB chimeric genes at the RNA and DNA sequence levels in a series of DFSPs and GCFs. All 16 tumors studied contained the COL1A1/PDGFB gene. The location of breakpoints within COL1A1 varied greatly, but was always limited to the region encoding the alpha-helical domain. The PDGFB segment of the chimeric transcript always starts with exon 2, placing PDGFB under the control of the COL1A1 promoter and removing all known elements negatively controlling PDGFB transcription and translation. Production of these aberrant transcripts in fibroblasts, the suspected cell of origin of DFSP/GCF, likely causes autocrine stimulation and cell proliferation. No specific function has yet been assigned to exon 2 of PDGFB, and this exon does not encode for the mature growth factor. Its retention in all chimeric COL1A1/PDGFB genes suggests that it is important for the normal processing of the PDGFB polypeptide.

Adult↗
PubMed1998

COL1A1-PDGFB fusion in a ring chromosome 4 found in a dermatofibrosarcoma protuberans.

Dermatofibrosarcoma protuberans (DP), an infiltrative skin tumor of intermediate malignancy, presents specific cytogenetic features such as reciprocal translocations t(17;22)(q22;q13.1) or, more often, supernumerary ring chromosomes derived from t(17;22). Different translocations, including t(2;17) and t(X;7), have also been described. We have shown previously that both r(17;22) and t(17;22) present the same molecular rearrangement fusing the COL1A1 gene on chromosome 17 and the PDGFB gene on chromosome 22. Out of our series of 16 DPs, we detected an extra ring chromosome in tumor T96-1175, which juxtaposed sequences from chromosomes 4 and 17. As shown by fluorescence in situ hybridization (FISH) using chromosome painting and alpha-satellite probes, T96-1175 apparently lacked chromosome 22 material in the ring. However, involvement of chromosome 22 through a rearrangement of PDGFB was shown by Southern blotting, reverse transcriptase-polymerase chain reaction (RT-PCR), and FISH. This study demonstrates that a cryptic molecular rearrangement between chromosomes 17 and 22 occurred in addition to the recombination of chromosomes 4 and 17 initially identified by FISH. Assessment for cryptic molecular events should be performed in other variant DP rearrangements.

Adult↗
PubMed1984

Dermatofibrosarcoma protuberans of 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. It is more common in men and in persons aged between 30 and 40 years. The scalp and neck-supraclavicular fossa are the most common sites, accounting for half of the cases. Approximately 50% to 75% of the patients experience local recurrence after initial surgery and, of these, half to three fourths manifest within three years of treatment. Metastases, seen in no more than 6% of all cases, are almost invariably preceded by two or more local recurrences and occur primarily through the bloodstream to the lungs and bones and less often to regional lymph nodes. Prognostic factors, differential diagnosis, and histogenesis are included.

Adult↗
PubMed1989

Giant cell fibroblastoma. A juvenile form of dermatofibrosarcoma protuberans.

A clinicopathologic analysis of 28 cases of giant cell fibroblastoma (GCF), a rare mesenchymal tumor occurring predominantly in the first decade of life, is presented. This disease presented as a painless, slowly enlarging, subcutaneous mass. The tumor recurred locally in 47% of the patients; however, metastasis was not detected. On microscopic examination, GCF showed an unique combination of spindle cell patterns, pleomorphic and multinucleated giant cells, myxoid areas, and distinctive sinusoid-like spaces. This unrecognized histomorphologic picture led to a misdiagnosis of sarcoma in 40% of the cases. The histogenesis of this lesion remains uncertain; however, based on both clinical and morphologic similarities, it is proposed that GCF is a juvenile form of dermatofibrosarcoma protuberans (DFSP).

Adolescent↗
PubMed1993

Metastatic dermatofibrosarcoma protuberans with fibrosarcomatous change in the absence of local recurrence. A case report of simultaneous occurrence with a malignant giant cell tumor of soft parts.

An unusual case of a metastasizing dermatofibrosarcoma protuberans with fibrosarcomatous change (DFSP-FS) is reported. The tumor metastasized to unusual sites, including soft tissues of the cheek, forearm, and retroperitoneum 5 years after surgical resection without local recurrence. In addition, the patient had a second, histologically distinct fibrohistiocytic tumor: a malignant giant cell tumor of soft parts. The occurrence of these two tumor types in the same patient highlights the relationship of DFSP to the family of fibrohistiocytic tumors. The late onset of metastases in the absence of local relapse, although a rare event, reflects the potential difficulty in predicting its biologic behavior. DFSP-FS may be a more aggressive tumor than ordinary DFSP.

Cheek↗