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PubMed1993

Fine-needle aspiration biopsy: dermatofibrosarcoma protuberans.

Dermatofibrosarcoma protuberans (DFSP) represents a spectrum of mesenchymal spindle cell tumors that typically involve both dermis and subcutis. Presented herein are six cases of DFSP, four of which were initially diagnosed by FNAB. The cytologic features useful in the identification of this lesion on Papanicolaou- and Diff-Quik-stained smears are discussed. Chief among these are the storiform stromal fragments, presence of entrapped adipose tissue and the recognition of fibrohistiocytic spindle cells. The potential pitfalls and the differential diagnostic possibilities of spindle-cell lesions, particularly those of fibrohistiocytic origin are discussed.

Adult↗
PubMedDate not supplied

Dermatofibrosarcoma protuberans with regional lymph node metastasis: a case report.

The case of a 21-year-old man with dermatofibrosarcoma protuberans of the scalp that spread to deep cervical lymph nodes is presented. Available literature is reviewed, and the infrequency of metastasis is discussed. Since cases of metastases almost always involve recurrent lesions, the importance of wide and deep resection in the initial operation cannot be overemphasized.

Adult↗
PubMedDate not supplied

Dermatofibrosarcoma protuberans.

The consultants agreed that dermatofibrosarcoma protuberans is characterized by its persistent growth and locally aggressive behavior. However, they did not all agree on the mode of treatment. Drs. Schuller and Snyderman favored surgical resection of the involved areas including the right cheek, lower eyelid, and any necessary portions of the nasal ala and upper lip. They encouraged the use of Moh's chemosurgery to detect microscopic disease. They were also in agreement regarding reconstruction, suggesting a cervical-facial or bi-lobed flap for the cheek. A full-thickness skin graft was recommended for the lower eyelid with primary closure planned for the lip defect. They disagreed on the use of postoperative radiation therapy. Dr. Schuller elected not to irradiate; Dr. Snyderman favored 60 to 65 Gy post-operatively. Dr. Quivey expressed concern regarding the morbidity from such a large resection and extensive reconstruction and suggested primary radiation therapy with external beam. All three consultants felt that good follow-up would include thorough head and neck examinations with MRI and/or CT scans if there were any concern about tumor recurrence.

Adult↗
PubMed1981

The fibroblastic nature of dermatofibrosarcoma protuberans. A tissue culture and ultrastructural study.

Dermatofibrosarcoma protuberans has been considered to be of fibrohistiocytic or fibroblastic origin. The purpose of this paper is to identify the original cell strain from which this neoplasm derives, using tissue culture and electron microscopic methods. Thirteen cases of DFSP characterised by clinical, topographical, histological and behavioral criteria were explanted. The emigrating cells were bipolar with two opposed processed and showed a radial arrangement in respect to the explants. After the second week the distal processes tended to curve back towards the cell body forming flame-like structures. This cell morphology and cellular orientation persisted during the whole life of the culture. Electron microscopy was performed in three cases; the newly grown cells maintained an electron microscopic picture similar to that found in the original tumors. This pattern of behaviour is characteristic of fibroblastic tumors and has been found in explants of normal fibroblasts, of fibromatosis and of fibrosarcomas used as controls. On this basis, we believe that DFSP is a fibrosarcoma of the skin of low grade malignancy.

Adult↗
PubMed1986

Dermatofibrosarcoma protuberans--a clinicopathological study of ten cases.

Dermatofibrosarcoma protuberans is a low grade soft tissue tumour of skin and frequently recurs. It is a locally invasive tumour and hence wide excision is required to minimize recurrences. Tumours of low mitotic rate, less than 5 per 10 high power fields with a lesser degree of cellularity and anaplasia tend to follow a more indolent course than those with moderate to high grades with higher mitotic rates.

Adolescent↗
PubMed1998

A Case of Dermatofibrosarcoma Protuberans in the Skin over the Breast of a Young Woman.

A 19-year-old woman with dermatofibrosarcoma protuberans (DFSP) of the breastis described. She presented with a tumor of the left breast with redness and swelling. She had noticed a small tumor five years previously, but it had begun togrow rapidly three months previously. Mammography, ultrasonography and aspiration biopsy cytology demonstrated an apparently benign tumor of the breast preoperatively. Wide excision was performed to confirm the diagnosis. Histologically, the tumor was composed of a uniform population of fibroblasts in a distinct storiform pattern. DFSP was diagnosed in the skin over the breast. This lesion ordinarily originates from the dermis in the trunk and proximal extremities. It is important to diagnose a soft tissue lesion accurately when it occurs in the breast.

Journal Article↗
PubMed1986

Dermatofibrosarcoma protuberans: the problems of surgical management.

This paper reports the findings of a study of five patients each of whom had a dermatofibrosarcoma protuberans. The difficulties encountered in making an accurate clinical diagnosis and ensuring adequate surgical excision of this uncommon skin tumour are discussed. Recommendations are made on methods designed to reduce the high recurrence rate normally associated with this locally aggressive tumour.

Adult↗
PubMed1983

Dermatofibrosarcoma protuberans of the vulva.

An unusual case of vulvar sarcoma, histologically identified as dermatofibrosarcoma protuberans, is presented together with a summary of current information regarding this lesion.

Female↗
PubMed1985

Dermatofibrosarcoma protuberans of the vulva.

A woman aged 52 years had a tumor of the mons pubis. The morphologic picture, featuring a whirling of spindle cells with long slender, interdigitating cell processes and so-called labyrinth nuclei, conformed to a dermatofibrosarcoma protuberans. Wide local excision is sufficient therapy, but more experience with this rare sarcoma of the vulva is necessary.

Cell Nucleus↗
PubMed1990

Cytogenetic analysis of dermatofibrosarcoma protuberans.

A case of dermatofibrosarcoma protuberans that occurred in an old burn scar over the anterior chest wall of a 46-year-old man is reported. Cytogenetic analysis of the tumor cells showed the presence of two abnormal clones: 47,XY, +8 and 48,XY, +8, +r.

Chromosome Aberrations↗
PubMed1992

Ring chromosome in a dermatofibrosarcoma protuberans.

We report the cytogenetic findings in a case of dermatofibrosarcoma protuberans in a 40-year-old male. Chromosome analysis revealed one clone consisting of +7, +11, +13, +14, +15, and a ring chromosome. This is consistent with two previously reported cases, each of which also had a single ring chromosome.

Adult↗
PubMed1993

Expression of the human progenitor cell antigen CD34 (HPCA-1) distinguishes dermatofibrosarcoma protuberans from fibrous histiocytoma in formalin-fixed, paraffin-embedded tissue.

BACKGROUND: Histologic distinction of dermatofibrosarcoma protuberans (DFSP) from fibrous histiocytoma (FH) may be difficult. In addition, differential diagnosis is hampered by the lack of appropriate immunohistochemical markers that reliably distinguish between these two entities. OBJECTIVE: This study is aimed at the introduction of a monoclonal antibody (anti-human progenitor cell antigen-1; anti-CD34) that distinguishes between DFSP and FH in formalin-fixed, paraffin-embedded tissue. METHODS: Paraffin-embedded specimens of DFSP, FH, and other soft-tissue tumors were investigated for CD34 expression by anti-human progenitor cell antigen-1/alkaline phosphatase-antialkaline phosphatase immunostaining. RESULTS: Strong CD34 reactivity was present in each DFSP (n = 19) but was consistently absent from FH (n = 45) and other soft-tissue tumors (n = 47). CONCLUSION: CD34 immunostaining of paraffin-embedded specimens may be useful in differentiating between DFSP and FH.

Antigens, CD↗
PubMed1989

Dermatofibrosarcoma protuberans treated with radiation therapy.

Dermatofibrosarcoma Protuberans is an uncommon soft tissue tumor that is locally aggressive, prone to recur locally following excision, and has a low propensity for metastasis. Ten patients have received radiation (both external beam and interstitial implant) as part of, or as their only treatment, at Massachusetts General Hospital from 1979-1987. Three patients received radiation alone (66.7-75 Gy) for gross palpable disease. One patient has slowly regressing nodules at 33 months; the other two had complete regression and are free of disease at 24 and 26 months posttreatment. Seven patients received radiation in conjunction with surgery. One received both preoperative and postoperative radiation and six received postoperative radiation (60-67 Gy) for microscopic residual disease following resection. Six of these seven patients are free of disease 16 to 105 months following treatment. One patient, with diffuse microscopic tumor infiltration of the scalp, had a local recurrence within the radiation field 16 months following 64.4 Gy. There has been no significant treatment related morbidity. Even though the present experience is limited and the follow-up brief, it does suggest that radiation can be considered a viable therapeutic option in the patient who has a lesion that is not resectable.

Adult↗
PubMed1996

Dermatofibroma and dermatofibrosarcoma protuberans: an immunohistochemical study reveals distinctive antigenic profiles.

Recent studies of mesenchymal cells of the dermis using antibodies to factor XIIIa (FXIIIa) and CD34 have demonstrated immunophenotypic heterogeneity amongst the normal resident spindle/dendritic cells of the dermis. These immunohistochemical markers also have been reported to be useful in the distinction between two dermal mesenchymal tumors of uncertain histogenetic origin - the dermatofibroma (DF) and dermatofibrosarcoma protuberans (DFSP). DFs are FXIIIa positive, CD34 negative while DFSPs are FXIIIa negative and CD34 positive. Expression of CD34 may also have histogenetic implications for these cutaneous neoplasms. In order to further study these tumors we studied 13 DFs and 12 DFSPs immunohistochemically using a microwave antigen retrieval technique in formalin fixed, paraffin embedded tissue with antibodies to FXIIIa, CD34, CD45, factor VIII related antigen (FVIII-RA), the Ki-67 antigen (MIB-1 antibody) and the lectin Ulex europaeus. Of the DFs, all 13 were FXIIIa positive; 12/13 were CD34 negative and 1 was strongly CD34 positive. All DFSPs were FXIIIa negative and CD34 positive. One DFSP also contained an area of fibrosarcoma which was negative for both markers. All tumors were negative with anti-FVIII-RA Ulex europaeus, and anti-CD45. MIB-1 staining demonstrated nuclear staining of the tumor cells in both DFs and DFSPs. Image analysis of MIB-1 stained sections revealed a significant difference in mean percent positive nuclear area between DFs (1.16% +/- 0.405) and DFSPs (2.265% +/- 0.963). In summary, FXIIIa reliably distinguished between DFs and DFSPs; however, CD34 immunoreactivity can be seen in DFs. No evidence for vascular or hematopoietic origin of these tumors was found using microwave antigen retrieval and anti-FVIII-RA, Ulex europaeus, or CD45 staining. With microwave enhancement trypsin was not necessary for FXIIIa staining; however, it did not significantly enhance detection of FVIII-RA, CD45, or Ulex antigens. DF and DFSP tumor cells are in the cell cycle as demonstrated by MIB-1 staining and there are significant differences in percent positive nuclear area between these neoplasms, being higher in DFSP compared to DF.

Animals↗
PubMed1977

Congenital dermatofibrosarcoma protuberans of the hand.

A case of congenital dermatofibrosarcoma protuberans is presented. It was treated by local radical surgical excision, the function of the hand remaining normal. Six months post operatively the child is in excellent health, and there are no signs of recurrence.

Fibrosarcoma↗
PubMed2002

Fusion of COL1A1 exon 29 with PDGFB exon 2 in a der(22)t(17;22) in a pediatric giant cell fibroblastoma with a pigmented Bednar tumor component. Evidence for age-related chromosomal pattern in dermatofibrosarcoma protuberans and related tumors.

In contrast with classic dermatofibrosarcoma protuberans (DP), genetic information about the juvenile or pigmented variant forms of DP, so-called giant cell fibroblastoma (GCF) and Bednar tumor (BT), is limited. In the sole karyotyped case of BT a supernumerary ring containing chromosomes 17 and 22 sequences, similar to DP rings, was reported, whereas in three GCF cases, t(17;22) or der(22)t(17;22) with COL1A1-PDGFB fusion involving exons 11, 40, and 47, respectively, have been described. Here, we report the first cytogenetic and molecular analysis of a tumor from a 5-year-old child that contained both GCF and BT components. The karyotype and molecular analyses confirmed the common histogenetic origin between DP, GCF, and BT in showing the presence of a der(22)t(17;22) fusing the COL1A1 exon 29 to PDGFB exon 2. Because COL1A1 exon 29 has been involved previously in gene fusion with PDGFB exon 2 in several cases of adult or infantile DP presenting either t(17;22) or ring chromosomes, our results support the concept that DP, GCF, and BT are morphologic variants of a same entity, rather than distinct tumors. Of interest, our findings give prominence to the relation between patient age and the chromosomal rearrangement pattern in DP and related tumors. Whereas only a few adult DP cases presented with translocations, all the infantile cases, either DP, GCF, or mixed BT-GCF, as shown here, contained translocation derivatives but not ring chromosomes. All the ring chromosomes were observed in adult cases. With respect to cytogenetic studies, DP, GCF, and BT appear to be a unique model for age-related chromosomal rearrangement progression.

Adolescent↗